Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a chronic autoimmune, inflammatory condition that causes immune-complex deposition in organs, resulting in systemic manifestations. Women, particularly those of African American descent, are more commonly affected. The clinical presentation can vary greatly. Notable clinical features include malar rash, nondestructive arthritis, lupus nephritis, serositis, cytopenia, thromboembolic disease, seizures Seizures A seizure is abnormal electrical activity of the neurons in the cerebral cortex that can manifest in numerous ways depending on the region of the brain affected. Seizures consist of a sudden imbalance that occurs between the excitatory and inhibitory signals in cortical neurons, creating a net excitation. The 2 major classes of seizures are focal and generalized. Seizures, and/or psychosis. Diagnosis is based on clinical criteria, and includes tests to determine ANAs, SLE-specific antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins, and specific clinical findings. The goal of management is to control symptoms and prevent organ damage, using corticosteroids, hydroxychloroquine, and immunosuppressants Immunosuppressants Immunosuppressants are a class of drugs widely used in the management of autoimmune conditions and organ transplant rejection. The general effect is dampening of the immune response. Immunosuppressants.

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Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

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Epidemiology and Etiology

Epidemiology

  • Prevalence in the United States:
    • Overall: 20–150 per 100,000
    • Women: 100–400 per 100,000
    • Pediatric systemic lupus erythematosus (SLE): approximately 2.5 per 100,000
  • More prevalent in people of African American or Asian descent
  • More common in urban areas
  • 10 times more common in women than men (no gender preference in drug-induced lupus)
  • Peak age at onset: 
    • Adults: 18–55 years
    • Children: 9–15 years

Etiology

  • The exact etiology is unknown.
  • Predisposing factors:
    • Genetic:
      • HLA-DR2
      • HLA-DR3
      • Congenital deficiencies of complement factors C1q, C2, and C4
    • Hormonal:
      • Increased estrogen (e.g., oral contraceptives, hormonal replacement)
      • Decreased testosterone
      • Hyperprolactinemia Hyperprolactinemia Hyperprolactinemia is defined as a condition of elevated levels of prolactin (PRL) hormone in the blood. The PRL hormone is secreted by the anterior pituitary gland and is responsible for breast development and lactation. The most common cause is PRL-secreting pituitary adenomas (prolactinomas). Hyperprolactinemia
      • Thyroid abnormalities
    • Environmental:
      • Cigarette smoking
      • EBV exposure
      • Ultraviolet (UV) light exposure
      • Silica exposure
    • Drug induced

Pathophysiology

The exact mechanism is unknown. Patients likely have a genetic predisposition to autoreactive immune cells (B and T cells T cells T cells, also called T lymphocytes, are important components of the adaptive immune system. Production starts from the hematopoietic stem cells in the bone marrow, from which T-cell progenitor cells arise. These cells migrate to the thymus for further maturation. T Cells).

  • Cell damage due to an environmental trigger → defective apoptosis and clearance of cellular debris → ↑ exposure to nuclear antigens ( DNA DNA The molecule DNA is the repository of heritable genetic information. In humans, DNA is contained in 23 chromosome pairs within the nucleus. The molecule provides the basic template for replication of genetic information, RNA transcription, and protein biosynthesis to promote cellular function and survival. DNA Types and Structure and RNA RNA Ribonucleic acid (RNA), like deoxyribonucleic acid (DNA), is a polymer of nucleotides that is essential to cellular protein synthesis. Unlike DNA, RNA is a single-stranded structure containing the sugar moiety ribose (instead of deoxyribose) and the base uracil (instead of thymine). RNA generally carries out the instructions encoded in the DNA but also executes diverse non-coding functions. RNA Types and Structure from damaged cells)
  • Autoreactive T and B cells B cells B lymphocytes, also known as B cells, are important components of the adaptive immune system. In the bone marrow, the hematopoietic stem cells go through a series of steps to become mature naive B cells. The cells migrate to secondary lymphoid organs for activation and further maturation. B Cells are activated → production of autoantibodies:
    • ANA
    • Anti-double-stranded DNA DNA The molecule DNA is the repository of heritable genetic information. In humans, DNA is contained in 23 chromosome pairs within the nucleus. The molecule provides the basic template for replication of genetic information, RNA transcription, and protein biosynthesis to promote cellular function and survival. DNA Types and Structure (anti-dsDNA) antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins
    • Anti-Smith (anti-Sm) antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins
    • Anti-histone antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins
    • Anti-Ro and anti-La antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins
    • Anti-ribonucleoprotein antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins
    • Antiphospholipid antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins
  • Antibody-antigen immune complexes form → endocytosis by B cells B cells B lymphocytes, also known as B cells, are important components of the adaptive immune system. In the bone marrow, the hematopoietic stem cells go through a series of steps to become mature naive B cells. The cells migrate to secondary lymphoid organs for activation and further maturation. B Cells and dendritic cells 
    • B cells B cells B lymphocytes, also known as B cells, are important components of the adaptive immune system. In the bone marrow, the hematopoietic stem cells go through a series of steps to become mature naive B cells. The cells migrate to secondary lymphoid organs for activation and further maturation. B Cells: activation of Toll-like receptors in endosomes → immune complexes are broken down by proteases → resulting peptides presented to T cells T cells T cells, also called T lymphocytes, are important components of the adaptive immune system. Production starts from the hematopoietic stem cells in the bone marrow, from which T-cell progenitor cells arise. These cells migrate to the thymus for further maturation. T Cells → T cell activation → further B cell activation
    • Dendritic cells: release of interferon → further B cell activation
  • Perpetual T and B cell activation results in persistent ↑ in antibody levels
  • Antibodies cause organ damage through: 
    • Immune (antigen-antibody)-complex deposition in microvasculature → excessive complement activation → inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body's defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation
    • Immune-complex deposition in the basement membrane of organs (e.g., skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin, kidneys Kidneys The kidneys are a pair of bean-shaped organs located retroperitoneally against the posterior wall of the abdomen on either side of the spine. As part of the urinary tract, the kidneys are responsible for blood filtration and excretion of water-soluble waste in the urine. Kidneys)
    • Direct interaction with nuclear antigens on cell surfaces → complement activation → cell injury Cell injury The cell undergoes a variety of changes in response to injury, which may or may not lead to cell death. Injurious stimuli trigger the process of cellular adaptation, whereby cells respond to withstand the harmful changes in their environment. Overwhelmed adaptive mechanisms lead to cell injury. Mild stimuli produce reversible injury. If the stimulus is severe or persistent, injury becomes irreversible. Cell Injury and Death and apoptosis
Pathophys of sle

Pathogenesis of systemic lupus erythematosus:
Genetic predisposition results in autoreactive B and T cells T cells T cells, also called T lymphocytes, are important components of the adaptive immune system. Production starts from the hematopoietic stem cells in the bone marrow, from which T-cell progenitor cells arise. These cells migrate to the thymus for further maturation. T Cells. When an environmental trigger causes cell apoptosis, defective clearance of cellular debris results in increased exposure to nuclear antigens. These antigens and autoantibodies lead to immune-complex formation. Endocytosis of the immune complexes results in interferon release from dendritic cells, leading to continuous activation of B cells B cells B lymphocytes, also known as B cells, are important components of the adaptive immune system. In the bone marrow, the hematopoietic stem cells go through a series of steps to become mature naive B cells. The cells migrate to secondary lymphoid organs for activation and further maturation. B Cells and persistent antibody production.

Image by Lecturio.

Clinical Presentation

The clinical presentation of SLE is highly variable Variable Variables represent information about something that can change. The design of the measurement scales, or of the methods for obtaining information, will determine the data gathered and the characteristics of that data. As a result, a variable can be qualitative or quantitative, and may be further classified into subgroups. Types of Variables and can include several organ systems.

General

  • Fatigue (most common symptom)
  • Fever Fever Fever is defined as a measured body temperature of at least 38°C (100.4°F). Fever is caused by circulating endogenous and/or exogenous pyrogens that increase levels of prostaglandin E2 in the hypothalamus. Fever is commonly associated with chills, rigors, sweating, and flushing of the skin. Fever
  • Weakness
  • Weight loss
  • Lymphadenopathy Lymphadenopathy Lymphadenopathy is lymph node enlargement (> 1 cm) and is benign and self-limited in most patients. Etiologies include malignancy, infection, and autoimmune disorders, as well as iatrogenic causes such as the use of certain medications. Generalized lymphadenopathy often indicates underlying systemic disease. Lymphadenopathy

Musculoskeletal

  • 80%‒90% of cases
  • Arthritis: 
    • Migratory
    • Symmetrical
    • Polyarticular
    • Nondestructive
    • Common locations: 
      • Knees
      • Hands
      • Fingers
  • Joint deformities (rare):
    • Swan-neck deformity
      • Proximal interphalangeal joint hyperextension 
      • Distal interphalangeal flexion
    • Ulnar drift
    • Due to joint laxity 
  • Arthralgia
  • Myalgia

Mucocutaneous

  • > 50% of cases 
  • Photosensitivity
  • Acute cutaneous lupus erythematosus:
    • A rash may precede systemic symptoms from months to years.
    • Localized:
      • Malar rash (“butterfly rash”)
      • Red or purple erythema on the cheeks and nasal bridge
      • Spares the nasolabial folds
      • Appears after sun exposure
    • Generalized:
      • Erythematous maculopapular lesions
      • Seen on sun-exposed skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin
      • Common sites: extensor surfaces of arms, hands
      • Sparing of the knuckles may be noted.
  • Discoid rash: 
    • Vibrant, scaling papules
    • Tends to cause scarring
  • Non-scarring alopecia Alopecia Alopecia is the loss of hair in areas anywhere on the body where hair normally grows. Alopecia may be defined as scarring or non-scarring, localized or diffuse, congenital or acquired, reversible or permanent, or confined to the scalp or universal; however, alopecia is usually classified using the 1st 3 factors. Alopecia 
  • Painless oral or nasal ulcers

Systemic manifestations

  • Renal (> 50% of cases):
    • Lupus nephritis (of varying severity)
      • Asymptomatic proteinuria or hematuria
      • Nephrotic syndrome Nephrotic syndrome Nephrotic syndrome is characterized by severe proteinuria, hypoalbuminemia, and peripheral edema. In contrast, the nephritic syndromes present with hematuria, variable loss of renal function, and hypertension, although there is sometimes overlap of > 1 glomerular disease in the same individual. Nephrotic Syndrome
      • Rapidly progressive glomerulonephritis Rapidly Progressive Glomerulonephritis Rapidly progressive glomerulonephritis (RPGN) is a syndrome of severe glomerular disease with progressive loss of kidney function within weeks to months. Histologically, crescents (the proliferation of epithelial cells and the infiltration of monocytes/macrophages in the Bowman space) are found in the glomeruli and arise from immunologic injury. Rapidly Progressive Glomerulonephritis
    • Hypertension
  • Cardiac:
    • Pericardial effusion Pericardial effusion Pericardial effusion is the accumulation of excess fluid in the pericardial space around the heart. The pericardium does not easily expand; thus, rapid fluid accumulation leads to increased pressure around the heart. The increase in pressure restricts cardiac filling, resulting in decreased cardiac output and cardiac tamponade. Pericardial Effusion and Cardiac Tamponade
    • Pericarditis Pericarditis Pericarditis is an inflammation of the pericardium, often with fluid accumulation. It can be caused by infection (often viral), myocardial infarction, drugs, malignancies, metabolic disorders, autoimmune disorders, or trauma. Acute, subacute, and chronic forms exist. Pericarditis
    • Libman-Sacks endocarditis Endocarditis Endocarditis is an inflammatory disease involving the inner lining (endometrium) of the heart, most commonly affecting the cardiac valves. Both infectious and noninfectious etiologies lead to vegetations on the valve leaflets. Patients may present with nonspecific symptoms such as fever and fatigue. Endocarditis
    • Severe myocarditis Myocarditis Myocarditis is an inflammatory disease of the myocardium, which may occur alone or in association with a systemic process. There are numerous etiologies of myocarditis, but all lead to inflammation and myocyte injury, most often leading to signs and symptoms of heart failure. Myocarditis
    • Coronary artery disease
  • Vascular:
    • Raynaud’s phenomenon
    • Thromboembolism
    • Vasculitis 
  • Neuropsychiatric:
    • Headaches
    • Seizures
    • Neuropathies
      • Peripheral neuropathy
      • Cranial neuropathy
      • Autonomic neuropathy
      • Mononeuritis multiplex
    • Ischemic stroke Ischemic Stroke An ischemic stroke (also known as cerebrovascular accident) is an acute neurologic injury that occurs as a result of brain ischemia; this condition may be due to cerebral blood vessel occlusion by thrombosis or embolism, or rarely due to systemic hypoperfusion. Ischemic Stroke
    • Aseptic meningitis Meningitis Meningitis is inflammation of the meninges, the protective membranes of the brain, and spinal cord. The causes of meningitis are varied, with the most common being bacterial or viral infection. The classic presentation of meningitis is a triad of fever, altered mental status, and nuchal rigidity. Meningitis
    • Demyelinating syndromes
      • Optic neuritis
      • Myelitis
    • Psychosis 
    • Anxiety
    • Depression 
    • Cognitive dysfunction
  • Pulmonary:
    • Pleuritis Pleuritis Pleuritis, also known as pleurisy, is an inflammation of the visceral and parietal layers of the pleural membranes of the lungs. The condition can be primary or secondary and results in sudden, sharp, and intense chest pain on inhalation and exhalation. Pleuritis
    • Pleural effusion Pleural Effusion Pleural effusion refers to the accumulation of fluid between the layers of the parietal and visceral pleura. Common causes of this condition include infection, malignancy, autoimmune disorders, or volume overload. Clinical manifestations include chest pain, cough, and dyspnea. Pleural Effusion
    • Chest pain Pain Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of Pain
    • Pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressure, which can lead to chronic progressive right heart failure. Pulmonary hypertension is grouped into 5 categories based on etiology, which include primary PAH, and PH due to cardiac disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear etiologies. Pulmonary Hypertension
    • Interstitial lung disease
      • Nonspecific interstitial pneumonia Pneumonia Pneumonia or pulmonary inflammation is an acute or chronic inflammation of lung tissue. Causes include infection with bacteria, viruses, or fungi. In more rare cases, pneumonia can also be caused through toxic triggers through inhalation of toxic substances, immunological processes, or in the course of radiotherapy. Pneumonia
      • Usual interstitial pneumonia Pneumonia Pneumonia or pulmonary inflammation is an acute or chronic inflammation of lung tissue. Causes include infection with bacteria, viruses, or fungi. In more rare cases, pneumonia can also be caused through toxic triggers through inhalation of toxic substances, immunological processes, or in the course of radiotherapy. Pneumonia
    • Diffuse alveolar hemorrhage
    • Shrinking lung syndrome (rare)
      • Progressive dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea
      • Diaphragm Diaphragm The diaphragm is a large, dome-shaped muscle that separates the thoracic cavity from the abdominal cavity. The diaphragm consists of muscle fibers and a large central tendon, which is divided into right and left parts. As the primary muscle of inspiration, the diaphragm contributes 75% of the total inspiratory muscle force. Diaphragm elevation
      • Decreased lung volumes
      • Restrictive pattern on pulmonary function test
  • GI (40% of cases):
    • Esophagitis Esophagitis Esophagitis is the inflammation or irritation of the esophagus. The major types of esophagitis are medication-induced, infectious, eosinophilic, corrosive, and acid reflux. Patients typically present with odynophagia, dysphagia, and retrosternal chest pain. Esophagitis
    • Protein-losing enteropathy
    • Hepatitis
    • Splenomegaly Splenomegaly Splenomegaly is pathologic enlargement of the spleen that is attributable to numerous causes, including infections, hemoglobinopathies, infiltrative processes, and outflow obstruction of the portal vein. Splenomegaly
    • Acute pancreatitis Acute pancreatitis Acute pancreatitis is an inflammatory disease of the pancreas due to autodigestion. Common etiologies include gallstones and excessive alcohol use. Patients typically present with epigastric pain radiating to the back. Acute Pancreatitis
    • Mesenteric vasculitis
  • Hematological:
    • Leukopenia (> 50% of cases)
    • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview
      • Iron-deficiency anemia
      • Autoimmune hemolytic anemia Autoimmune Hemolytic Anemia Autoimmune hemolytic anemia (AIHA) is a rare type of hemolytic anemia characterized by antibody production against self RBCs, leading to destruction of these cells in the spleen and other reticuloendothelial tissues. The disease is generally categorized as warm or cold, depending on the thermal reactivity of the autoantibodies. Autoimmune Hemolytic Anemia
    • Thrombocytopenia Thrombocytopenia Thrombocytopenia occurs when the platelet count is < 150,000 per microliter. The normal range for platelets is usually 150,000-450,000/µL of whole blood. Thrombocytopenia can be a result of decreased production, increased destruction, or splenic sequestration of platelets. Patients are often asymptomatic until platelet counts are < 50,000/µL. Thrombocytopenia

Subtypes

Drug-induced lupus

  • Etiology: 
    • Procainamide
    • Hydralazine
    • Isoniazid
    • Quinidine
    • Methyldopa
    • Diltiazem
    • Sulfasalazine
    • Tumor necrosis factor Tumor necrosis factor Tumor necrosis factor (TNF) is a major cytokine, released primarily by macrophages in response to stimuli. The presence of microbial products and dead cells and injury are among the stimulating factors. This protein belongs to the TNF superfamily, a group of ligands and receptors performing functions in inflammatory response, morphogenesis, and cell proliferation. Tumor Necrosis Factor (TNF) ( TNF TNF Tumor necrosis factor (TNF) is a major cytokine, released primarily by macrophages in response to stimuli. The presence of microbial products and dead cells and injury are among the stimulating factors. This protein belongs to the TNF superfamily, a group of ligands and receptors performing functions in inflammatory response, morphogenesis, and cell proliferation. Tumor Necrosis Factor (TNF)) inhibitors (e.g., etanercept, infliximab)
  • Pathogenesis theories:
    • Altered drug metabolism
    • Altered response to drug metabolites
    • Abnormal activation of lymphocytes Lymphocytes Lymphocytes are heterogeneous WBCs involved in immune response. Lymphocytes develop from the bone marrow, starting from hematopoietic stem cells (HSCs) and progressing to common lymphoid progenitors (CLPs). B and T lymphocytes and natural killer (NK) cells arise from the lineage. Lymphocytes
  • Clinical presentation
    • Similar to idiopathic SLE
    • Cutaneous manifestations are generally less common.
    • Severe systemic manifestations are less common, but may vary depending on the offending drug.
    • Duration of medication exposure before onset can vary.
  • Diagnosis: associated with positive anti-histone antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins
  • Management: improves with removal of the offending medication

Discoid lupus erythematosus

  • Subset of chronic cutaneous lupus
  • Clinical presentation:
    • Photodistributed cutaneous lesions
    • May be localized or diffuse
    • Characteristics:
      • Erythematous, violaceous scaly plaques
      • Follicular plugging
    • Results in scarring and atrophy
    • Systemic disease is usually not present.
Discoid lupus erythematosus

Cutaneous presentation of discoid lupus:
Image shows scaling plaque and scarring.

Image: “Discoid lupus erythematosus” by Department of Dermatology, Uniformed Services University. License: Public Domain

Neonatal lupus

  • Etiology:
    • Passive transfer of autoantibodies from the mother to the fetus
    • Particularly involves anti-Ro and anti-La antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins
  • Clinical presentation:
    • Cutaneous:
      • Often noted at delivery, but may not show until exposure to UV light
      • Reversible
      • Erythematous annular lesions
      • Slight central atrophy
      • Raised margins
      • Scaly
      • Locations: periorbital, scalp, palms, soles, diaper region
    • Cardiac:
      • Occurs between 18 and 25 weeks of gestation
      • Varying levels of atrioventricular heart block and fetal bradycardia
      • Congestive heart failure Congestive heart failure Congestive heart failure refers to the inability of the heart to supply the body with normal cardiac output to meet metabolic needs. Echocardiography can confirm the diagnosis and give information about the ejection fraction. Congestive Heart Failure
      • Myocarditis
      • Aortic dilation
    • Other:
      • Transaminitis
      • Hepatomegaly or hepatitis
      • Cytopenias 
      • Hydrops fetalis
  • Diagnosis:
    • Anti-Ro and anti-La antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins in the mother or child
    • Clinical manifestations (listed above)
  • Management:
    • Prenatal:
      • Controversial
      • Fluorinated steroids: dexamethasone, betamethasone
      • Beta-agonists for heart rate < 50/minute
      • Early delivery if in poor overall health
    • Postnatal:
      • Depends on severity of cardiac involvement
      • Observation
      • Cardiac pacemaker for some with complete heart block
      • Rash will typically resolve without scarring.

Diagnosis

Antibody testing

When SLE is initially suspected: ANA

  • Nonspecific, but the most sensitive screening test
  • Seen in > 95% of cases
  • Immunofluorescence pattern may be diffuse or speckled.

If ANA is positive, the following should be evaluated:

  • Anti-dsDNA antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins:
    • Specific for SLE
    • Seen in 70% of cases
    • Correlates with disease activity
  • Anti-Sm antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins:
    • Highly specific, but less sensitive than anti-dsDNA
    • Only seen in 30% of cases
    • Not present in drug-induced lupus
  • Anti-histone antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins: positive in drug-induced lupus
  • Antiphospholipid antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins:
    • Lupus anticoagulant, anti-cardiolipin antibody, and anti-beta2-glycoprotein antibody
    • Associated with thrombotic events and spontaneous abortion Spontaneous abortion Spontaneous abortion, also known as miscarriage, is the loss of a pregnancy before 20 weeks' gestation. However, the layperson use of the term "abortion" is often intended to refer to induced termination of a pregnancy, whereas "miscarriage" is preferred for spontaneous loss. Spontaneous Abortion
  • Anti-Ro or anti-La: 
    • Seen in 20%‒30% of cases
    • Frequently present in those with Sjögren’s syndrome
    • Anti-Ro is associated with neonatal lupus.
  • Anti-ribonucleoprotein antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins:
    • Seen in 25% of cases
    • Associated with mixed connective-tissue disease

Supporting workup

  • CBC:
    • Thrombocytopenia Thrombocytopenia Thrombocytopenia occurs when the platelet count is < 150,000 per microliter. The normal range for platelets is usually 150,000-450,000/µL of whole blood. Thrombocytopenia can be a result of decreased production, increased destruction, or splenic sequestration of platelets. Patients are often asymptomatic until platelet counts are < 50,000/µL. Thrombocytopenia 
    • Leukopenia
    • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview → should be evaluated for autoimmune hemolysis:
      • ↑ Lactate dehydrogenase
      • ↑ Indirect bilirubin
      • ↓ Haptoglobin
      • Positive direct Coombs test
  • ↑ Erythrocyte sedimentation rate (ESR) and CRP
  • ↓ Complement component (C3 and C4) levels 
    • Will be normal in drug-induced lupus
    • Correlates with disease activity
  • Rule out the following:
    • Hepatitis B Hepatitis B Hepatitis B virus (HBV) is a partially double-stranded DNA virus, which belongs to the Orthohepadnavirus genus and the Hepadnaviridae family. Most individuals with acute HBV infection are asymptomatic or have mild, self-limiting symptoms. Chronic infection can be asymptomatic or create hepatic inflammation, leading to liver cirrhosis and hepatocellular carcinoma (HCC). Hepatitis B Virus and C
    • Borrelia Borrelia Borrelia are gram-negative microaerophilic spirochetes. Owing to their small size, they are not easily seen on Gram stain but can be visualized using dark-field microscopy, Giemsa, or Wright stain. Spirochetes are motile and move in a characteristic spinning fashion due to axial filaments in the periplasmic space. Borrelia serology (in endemic areas)
    • EBV
  • Evaluation for renal involvement:
    • ↑ BUN and creatinine 
    • Urinalysis: 
      • Proteinuria
      • Hematuria
      • RBC casts
    • ↑ Urine protein:creatinine ratio
    • Renal biopsy may be considered to classify the disease and guide management:
      • Class I: minimal mesangial lupus nephritis
      • Class II: mesangial proliferative lupus nephritis
      • Class III: focal proliferative lupus nephritis
      • Class IV: diffuse segmental or diffuse global lupus nephritis
      • Class V: membranous lupus nephritis
      • Class VI: advance sclerosing lupus nephritis

Diagnostic criteria

  • Based on the European League Against Rheumatisms/American College of Rheumatology (EULAR/ACR) 2019 classification criteria
  • All patients must have an ANA titer > 1:80.
  • A score of ≥ 10 with ≥ 1 clinical finding confirms a diagnosis of SLE.
  • Previous ACR criteria (from 1997) required 4 of 11 findings.
Table: Diagnostic criteria for systemic lupus erythematosus
Domain Criteria Score
Clinical findings
Constitutional Fever Fever Fever is defined as a measured body temperature of at least 38°C (100.4°F). Fever is caused by circulating endogenous and/or exogenous pyrogens that increase levels of prostaglandin E2 in the hypothalamus. Fever is commonly associated with chills, rigors, sweating, and flushing of the skin. Fever 2
Mucocutaneous Non-scarring alopecia Alopecia Alopecia is the loss of hair in areas anywhere on the body where hair normally grows. Alopecia may be defined as scarring or non-scarring, localized or diffuse, congenital or acquired, reversible or permanent, or confined to the scalp or universal; however, alopecia is usually classified using the 1st 3 factors. Alopecia 2
Oral ulcers 2
Subacute cutaneous or discoid lupus 4
Acute cutaneous lupus 6
Musculoskeletal > 2 joints involved 6
Neuropsychiatric Delirium Delirium Delirium is a medical condition characterized by acute disturbances in attention and awareness. Symptoms may fluctuate during the course of a day and involve memory deficits and disorientation. Delirium 2
Psychosis 3
Seizure 5
Serositis Pleural or pericardial effusion 5
Acute pericarditis 6
Hematological Leukopenia 3
Thrombocytopenia Thrombocytopenia Thrombocytopenia occurs when the platelet count is < 150,000 per microliter. The normal range for platelets is usually 150,000-450,000/µL of whole blood. Thrombocytopenia can be a result of decreased production, increased destruction, or splenic sequestration of platelets. Patients are often asymptomatic until platelet counts are < 50,000/µL. Thrombocytopenia 4
Autoimmune hemolysis 4
Renal Proteinuria 4
Renal biopsy class II or V lupus nephritis 8
Renal biopsy class III or IV lupus nephritis 10
Immunological findings
SLE-specific antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins Anti-dsDNA antibody or anti-Smith antibody 6
Complement levels Low C3 or C4 3
Low C3 and C4 4
Antiphospholipid antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins Anti-cardiolipin antibody or lupus anticoagulant or anti-beta2-glycoprotein antibody 2
Anti-dsDNA: anti-double-stranded DNA DNA The molecule DNA is the repository of heritable genetic information. In humans, DNA is contained in 23 chromosome pairs within the nucleus. The molecule provides the basic template for replication of genetic information, RNA transcription, and protein biosynthesis to promote cellular function and survival. DNA Types and Structure
C3: complement component 3
C4: complement component 4
SLE: systemic lupus erythematosus

Mnemonic

To help recall some criteria, remember “SOAP BRAIN MD:”

  • Serositis (e.g., pleuritis, pericarditis)
  • Oral or nasopharyngeal ulcers (painless)
  • Arthritis 
  • Photosensitivity
  • Blood disorders (e.g., cytopenias)
  • Renal disease
  • ANAs 
  • Immunological disorders (e.g., anti-dsDNA, anti-Sm, antiphospholipid)
  • Neurological disorders (e.g., seizures Seizures A seizure is abnormal electrical activity of the neurons in the cerebral cortex that can manifest in numerous ways depending on the region of the brain affected. Seizures consist of a sudden imbalance that occurs between the excitatory and inhibitory signals in cortical neurons, creating a net excitation. The 2 major classes of seizures are focal and generalized. Seizures, psychosis)
  • Malar rash 
  • Discoid rash

Management

As there is no definitive treatment available, management is aimed at controlling acute flares, suppressing symptoms, and preventing organ damage.

General

  • Educate the patient about the disease.
  • Smoking cessation
  • UV protection:
    • Sunscreen
    • Avoid medications that cause photosensitivity.
    • Avoid tanning.
  • Isometric exercise
  • Relaxation techniques to curb stress
  • Acetaminophen Acetaminophen Acetaminophen is an over-the-counter nonopioid analgesic and antipyretic medication and the most commonly used analgesic worldwide. Despite the widespread use of acetaminophen, its mechanism of action is not entirely understood. Acetaminophen and NSAIDs to manage pain Pain Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of Pain

Cutaneous treatment

  • Topical corticosteroids
  • Topical calcineurin inhibitors
  • Hydroxychloroquine

Systemic management

  • All patients must be on hydroxychloroquine.
  • Mild-to-moderate symptoms:
    • Low dose of glucocorticoids Glucocorticoids Glucocorticoids are a class within the corticosteroid family. Glucocorticoids are chemically and functionally similar to endogenous cortisol. There are a wide array of indications, which primarily benefit from the antiinflammatory and immunosuppressive effects of this class of drugs. Glucocorticoids 
    • Steroid-sparing immunosuppressants Immunosuppressants Immunosuppressants are a class of drugs widely used in the management of autoimmune conditions and organ transplant rejection. The general effect is dampening of the immune response. Immunosuppressants may be added:
      • Methotrexate
      • Azathioprine
  • Severe symptoms with organ damage:
    • High dose of glucocorticoids Glucocorticoids Glucocorticoids are a class within the corticosteroid family. Glucocorticoids are chemically and functionally similar to endogenous cortisol. There are a wide array of indications, which primarily benefit from the antiinflammatory and immunosuppressive effects of this class of drugs. Glucocorticoids
    • Immunosuppressive or biological agents:
      • Cyclophosphamide
      • Mycophenolate
      • Rituximab
      • Belimumab (used if disease is resistant to the above therapies)

Prevention of complications

  • Prophylaxis for osteoporosis Osteoporosis Osteoporosis refers to a decrease in bone mass and density leading to an increased number of fractures. There are 2 forms of osteoporosis: primary, which is commonly postmenopausal or senile; and secondary, which is a manifestation of immobilization, underlying medical disorders, or long-term use of certain medications. Osteoporosis
  • Influenza Influenza Influenza viruses are members of the Orthomyxoviridae family and the causative organisms of influenza, a highly contagious febrile respiratory disease. There are 3 primary influenza viruses (A, B, and C) and various subtypes, which are classified based on their virulent surface antigens, hemagglutinin (HA) and neuraminidase (NA). Influenza typically presents with a fever, myalgia, headache, and symptoms of an upper respiratory infection. Influenza Viruses/Influenza, human papillomavirus Human papillomavirus Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomaviridae: HPV, and pneumococcal vaccines
  • Suppression of recurrent urinary tract infections Urinary tract infections Urinary tract infections (UTIs) represent a wide spectrum of diseases, from self-limiting simple cystitis to severe pyelonephritis that can result in sepsis and death. Urinary tract infections are most commonly caused by Escherichia coli, but may also be caused by other bacteria and fungi. Urinary Tract Infections
  • Blood pressure should be managed to prevent renal damage.

Complications and Prognosis

Lupus nephritis

  • Seen in 30% of patients with SLE
  • Due to subendothelial deposition of immune complexes
  • Patients usually present with nephritic or nephrotic syndromes.
  • Management depends on the severity of the disease and may include:
    • Antihypertensive/antiproteinuric therapy:
      • ACE inhibitors
      • Angiotensin II receptor blockers (ARBs)
    • High dose of corticosteroids
    • Immunosuppressants:
      • Azathioprine
      • Mycophenolate
      • Tacrolimus
      • Cyclophosphamide
    • Dialysis Dialysis Renal replacement therapy refers to dialysis and/or kidney transplantation. Dialysis is a procedure by which toxins and excess water are removed from the circulation. Hemodialysis and peritoneal dialysis (PD) are the two types of dialysis, and their primary difference is the location of the filtration process (external to the body in hemodialysis versus inside the body for PD). Overview and Types of Dialysis

Antiphospholipid syndrome Antiphospholipid syndrome Antiphospholipid syndrome (APLS) is an acquired autoimmune disorder characterized by the persistent presence of antiphospholipid antibodies, which create a hypercoagulable state. These antibodies are most commonly discovered during a workup for a thrombotic event or recurrent pregnancy loss, which are the 2 most common clinical manifestations. Antiphospholipid Syndrome (APS)

  • 40% of patients with SLE are positive for APS antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins.
  • Only 20% of patients exhibit symptoms (thrombosis).
  • Management: 
    • Anticoagulants Anticoagulants Anticoagulants are drugs that retard or interrupt the coagulation cascade. The primary classes of available anticoagulants include heparins, vitamin K-dependent antagonists (e.g., warfarin), direct thrombin inhibitors, and factor Xa inhibitors. Anticoagulants
    • Hydroxychloroquine

Libman-Sacks endocarditis Endocarditis Endocarditis is an inflammatory disease involving the inner lining (endometrium) of the heart, most commonly affecting the cardiac valves. Both infectious and noninfectious etiologies lead to vegetations on the valve leaflets. Patients may present with nonspecific symptoms such as fever and fatigue. Endocarditis

  • One of the most common cardiac presentations of SLE
  • Non-bacterial endocarditis Endocarditis Endocarditis is an inflammatory disease involving the inner lining (endometrium) of the heart, most commonly affecting the cardiac valves. Both infectious and noninfectious etiologies lead to vegetations on the valve leaflets. Patients may present with nonspecific symptoms such as fever and fatigue. Endocarditis:
    • Deposition of sterile platelet thrombi on heart valves
    • Commonly affects mitral-aortic valves
  • Patients usually present with:
    • Valvular insufficiency
    • Heart failure
    • Embolism
    • Secondary infective endocarditis Endocarditis Endocarditis is an inflammatory disease involving the inner lining (endometrium) of the heart, most commonly affecting the cardiac valves. Both infectious and noninfectious etiologies lead to vegetations on the valve leaflets. Patients may present with nonspecific symptoms such as fever and fatigue. Endocarditis
  • Management:
    • SLE management
    • Systemic anticoagulants
    • Surgery in severe disease

Prognosis

  • Rate of survival is very good with optimal therapy.
  • 5-year survival: > 90%
  • Most common causes of death: 
    • Cardiovascular disease
    • Infections
    • End-stage renal disease
  • Poor prognosis has been associated with:
    • ↑ Serum creatinine levels and nephrotic syndrome 
    • Hypertension
    • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview, hypoalbuminemia, or hypocomplementemia 
    • Antiphospholipid antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins
    • Male gender
    • African American ethnicity 
    • Low socioeconomic status

Mnemonic

To recall the 3 most common causes of death in SLE, remember: “Lupus patients die with Redness In their Cheeks.”

  • Renal disease
  • Infections
  • Cardiovascular disease

Differential Diagnosis

  • Rheumatoid arthritis Rheumatoid arthritis Rheumatoid arthritis (RA) is a symmetric, inflammatory polyarthritis and chronic, progressive, autoimmune disorder. Presentation occurs most commonly in middle-aged women with joint swelling, pain, and morning stiffness (often in the hands). Rheumatoid Arthritis: an autoimmune disease resulting in joint inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body's defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation and destruction. Rheumatoid arthritis Rheumatoid arthritis Rheumatoid arthritis (RA) is a symmetric, inflammatory polyarthritis and chronic, progressive, autoimmune disorder. Presentation occurs most commonly in middle-aged women with joint swelling, pain, and morning stiffness (often in the hands). Rheumatoid Arthritis is typically symmetric with tenderness and swelling of the joints of the hands and feet. Systemic manifestations may include Sjögren’s syndrome, interstitial lung disease, pleural effusion, pericarditis, vasculitis, and anemia. Positive rheumatoid factor and anti-cyclic citrullinated peptide antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins may be seen. Treatment is with NSAIDs, disease-modifying antirheumatic drugs Disease-modifying antirheumatic drugs Disease-modifying antirheumatic drugs are antiinflammatory medications used to manage rheumatoid arthritis. The medications slow, but do not cure, the progression of the disease. The medications are classified as either synthetic or biologic agents and each has unique mechanisms of action and side effects. Disease-modifying Antirheumatic Drugs (DMARDs), steroids, and immunosuppressive medications.
  • Adult Still’s disease: a rare, systemic inflammatory disease characterized by daily fevers, arthritis, and an evanescent, salmon-colored rash on the trunk and extremities. Laboratory evaluation will show leukocytosis, transaminitis, negative ANA, and elevated inflammatory markers. Overall, the diagnosis of adult-onset Still’s disease is based on exclusion. Management depends on the severity of the condition. Treatment is with NSAIDs, glucocorticoids Glucocorticoids Glucocorticoids are a class within the corticosteroid family. Glucocorticoids are chemically and functionally similar to endogenous cortisol. There are a wide array of indications, which primarily benefit from the antiinflammatory and immunosuppressive effects of this class of drugs. Glucocorticoids, DMARDs, and biological agents.
  • Behcet’s disease: inflammatory vasculitis of small and large vessels. Symptoms can include arthritis and painful oral and genital ulcers. Ocular, neurologic, GI, and vascular manifestations are also seen. The diagnosis is based on clinical criteria, and the characteristic SLE antibody test will be negative. Management depends on clinical manifestations. Treatment is with corticosteroids, DMARDs, and TNF TNF Tumor necrosis factor (TNF) is a major cytokine, released primarily by macrophages in response to stimuli. The presence of microbial products and dead cells and injury are among the stimulating factors. This protein belongs to the TNF superfamily, a group of ligands and receptors performing functions in inflammatory response, morphogenesis, and cell proliferation. Tumor Necrosis Factor (TNF) inhibitors.
  • Fibromyalgia Fibromyalgia Fibromyalgia is a chronic pain syndrome characterized by widespread body pain, chronic fatigue, mood disturbance, and cognitive disturbance. It also presents with other comorbid symptoms such as migraine headaches, depression, sleep disturbance, and irritable bowel syndrome. Fibromyalgia: a nonarticular disorder of unknown etiology that causes generalized pain Pain Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of Pain in the muscles, points of tendon insertion, and soft tissues. Associated symptoms include fatigue, muscle stiffness, cognitive disturbances, depression, and anxiety. Diagnosis is based on clinical criteria. Imaging and laboratory testing will be unremarkable. Management includes exercise and non- opioid analgesics Opioid analgesics Opiates are drugs that are derived from the sap of the opium poppy. Opiates have been used since antiquity for the relief of acute severe pain. Opioids are synthetic opiates with properties that are substantially similar to those of opiates. Opioid Analgesics, and is aimed at improving sleep Sleep Sleep is a reversible phase of diminished responsiveness, motor activity, and metabolism. This process is a complex and dynamic phenomenon, occurring in 4-5 cycles a night, and generally divided into non-rapid eye movement (NREM) sleep and REM sleep stages. Physiology of Sleep and reducing stress.
  • Sjogren’s syndrome: an autoimmune, inflammatory condition of the glandular tissues, resulting in decreased tear and saliva production. Symptoms include dry eyes and mouth. Systemic manifestations include Raynaud’s phenomenon, neuropathy, and cutaneous vasculitis. Diagnosis is based on symptoms, clinical findings, determination of anti-Ro and anti-La antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins, and salivary-gland biopsy. Management is targeted at achieving symptomatic relief and immunosuppressive therapy is reserved for severe symptoms.
  • Sarcoidosis Sarcoidosis Sarcoidosis is a multisystem inflammatory disease that causes noncaseating granulomas. The exact etiology is unknown. Sarcoidosis usually affects the lungs and thoracic lymph nodes, but it can also affect almost every system in the body, including the skin, heart, and eyes, most commonly. Sarcoidosis: an inflammatory disorder causing non-caseating granulomas in organs and tissues. The etiology is unclear, and symptoms can vary widely. Patients may develop cough, dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea, fatigue, fever, arthritis, erythema nodosum Erythema nodosum Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum, uveitis Uveitis Uveitis is the inflammation of the uvea, the pigmented middle layer of the eye, which comprises the iris, ciliary body, and choroid. The condition is categorized based on the site of disease; anterior uveitis is the most common. Diseases of the Uvea, heart block, and neuropsychiatric manifestations. Diagnosis is based on biopsy findings, as the antibody test tends to be negative. Management is with corticosteroids.
  • Non-Hodgkin’s lymphoma: a diverse group of malignancies of B cell, T cell, and NK cell origin. Most cases involve the lymph nodes. Non-Hodgkin’s lymphoma often presents with fatigue, fevers, night sweats, weight loss, anemia, and lymphadenopathy. Other symptoms can occur in patients with extranodal involvement (e.g., GI, CNS). Lymph node or bone marrow Bone marrow Bone marrow, the primary site of hematopoiesis, is found in the cavities of cancellous bones and the medullary canals of long bones. There are 2 types: red marrow (hematopoietic with abundant blood cells) and yellow marrow (predominantly filled with adipocytes). Composition of Bone Marrow biopsy is helpful in diagnosis. Management is primarily with chemotherapy, immunotherapy, and targeted therapy Targeted Therapy Targeted therapy exerts antineoplastic activity against cancer cells by interfering with unique properties found in tumors or malignancies. The types of drugs can be small molecules, which are able to enter cells, or monoclonal antibodies, which have targets outside of or on the surface of cells. Targeted Therapy and Other Nontraditional Antineoplastic Agents.

References

  1. Schur, P.H., and Hahn, B.H. (2019). Epidemiology and pathogenesis of systemic lupus erythematosus. In Ramirez Curtis, M. (Ed.), UpToDate. Retrieved February 9, 2021, from https://www.uptodate.com/contents/epidemiology-and-pathogenesis-of-systemic-lupus-erythematosus
  2. Wallace, D.J., and Gladman, D.D. (2019). Clinical manifestations and diagnosis of systemic lupus erythematosus in adults. In Ramirez Curtis, M. (Ed.), UpToDate. Retrieved February 9, 2021, from https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-systemic-lupus-erythematosus-in-adults
  3. Wallace, D.J. (2020). Overview of the management and prognosis of systemic lupus erythematosus in adults. In Ramirez Curtis, M. (Ed.), UpToDate. Retrieved February 9, 2021, from https://www.uptodate.com/contents/overview-of-the-management-and-prognosis-of-systemic-lupus-erythematosus-in-adults
  4. Buyon, J.P. (2020). Neonatal lupus: Management and outcomes. In TePas, E. (Ed.), UpToDate. Retrieved February 14, 2021, from https://www.uptodate.com/contents/neonatal-lupus-management-and-outcomes
  5. Buyon, J.P. (2019). Neonatal lupus: Epidemiology, pathogenesis, clinical manifestations, and diagnosis. In TePas, E. (Ed.), UpToDate. Retrieved February 14, 2021, from https://www.uptodate.com/contents/neonatal-lupus-epidemiology-pathogenesis-clinical-manifestations-and-diagnosis
  6. Merola, J.F. (2019). Overview of cutaneous lupus erythematosus. In Ofori, A.O., and Ramirez Curtis, M. (Eds.), UpToDate. Retrieved February 14, 2021, from https://www.uptodate.com/contents/overview-of-cutaneous-lupus-erythematosus
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  8. Bartels, C.M., and Muller, D. (2020). Systemic lupus erythematosus (SLE). In Diamond, H.S. (Ed.), Medscape. Retrieved February 9, 2021, from https://emedicine.medscape.com/article/332244-overview
  9. Justiz Vaillant, A.A., Goyal, A., Bansal, P., and Varacallo, M. (2020). Systemic lupus erythematosus. [Online] StatPearls. Retrieved February 9, 2021, from https://www.ncbi.nlm.nih.gov/books/NBK535405/
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  11. Aringer, M., Leuchten, N., and Johnson, S.R. (2020). New criteria for lupus. Current Rheumatology Reports, 22(6):18. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7220972/

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