Sjögren’s Syndrome

Sjögren syndrome (SS) is an autoimmune, inflammatory condition where glandular tissues, such as the salivary and lacrimal glands, are infiltrated by 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, resulting in decreased tear and saliva production. The disease mainly affects middle-aged women, and is associated with other autoimmune conditions. Patients may experience symptoms and complications related to dry eyes and mouth. There is a wide range of extraglandular manifestations, including Raynaud’s phenomenon, neuropathy, and cutaneous vasculitis. Patients are also at an increased risk of developing non-Hodgkin’s lymphoma. Diagnosis is based on the presence of symptoms, and is validated by the clinical examination, serologic studies, or salivary gland biopsy. A multidisciplinary approach is needed to manage patients and targets symptomatic relief, with immunosuppressive therapy reserved for severe symptoms.

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Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

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Overview

Definition

Sjögren’s syndrome (SS), also known as sicca syndrome, is a chronic autoimmune and inflammatory condition leading to decreased lacrimal and salivary gland function, resulting in dry eyes (xerophthalmia) and mouth (xerostomia).

Epidemiology

  • The 2nd most common rheumatologic disorder
  • Prevalence: 0.1%–4% of the population 
  • Predominantly seen in women (female : male ratio of 9:1)
  • Age of onset: middle age (40–55 years of age)
  • ½ of patients have another autoimmune disease (e.g., 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, systemic lupus erythematosus 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. Systemic Lupus Erythematosus).

Etiology

Primary SS:

  • Etiology is unknown.
  • Possible genetic predisposition: 
    • HLA-DR3
    • HLA-DQ2
    • HLA-DR5
  • Possible viral connection: 
    • Epstein-Barr virus Epstein-Barr Virus Epstein-Barr virus (EBV) is a linear, double-stranded DNA virus belonging to the Herpesviridae family. This highly prevalent virus is mostly transmitted through contact with oropharyngeal secretions from an infected individual. The virus can infect epithelial cells and B lymphocytes, where it can undergo lytic replication or latency. Epstein-Barr Virus (EBV)
    • Chronic hepatitis C Hepatitis C Hepatitis C is an infection of the liver caused by the hepatitis C virus (HCV). The infection can be transmitted through infectious blood or body fluids and may be transmitted during childbirth or through IV drug use or sexual intercourse. Hepatitis C virus can cause both acute and chronic hepatitis, ranging from a mild to a serious, lifelong illness including liver cirrhosis and hepatocellular carcinoma (HCC). Hepatitis C Virus
    • Human T cell leukemia virus 1
    • Human herpesvirus 6
    • Human immunodeficiency virus (HIV)

Secondary SS:

Presents in association with another autoimmune disease:

  • Rheumatoid arthritis
  • Systemic lupus erythematosus
  • Polymyositis Polymyositis Polymyositis (PM) is an autoimmune inflammatory myopathy caused by T cell-mediated muscle injury. The etiology of PM is unclear, but there are several genetic and environmental associations. Polymyositis is most common in middle-aged women and rarely affects children. Polymyositis
  • Scleroderma Scleroderma Scleroderma (systemic sclerosis) is an autoimmune condition characterized by diffuse collagen deposition and fibrosis. The clinical presentation varies from limited skin involvement to diffuse involvement of internal organs. Scleroderma

Pathophysiology

The overall mechanism for SS is unclear:

  • Process may be set off by an environmental trigger in a patient with a genetic predisposition.
  • Dendritic cells migrate into salivary and lacrimal glands → release interferons Interferons Interferon (IFN) is a cytokine with antiviral properties (it interferes with viral infections) and various roles in immunoregulation. The different types are type I IFN (IFN-ɑ and IFN-β), type II IFN (IFN-ɣ), and type III IFN (IFN-ƛ). Interferons
  • Lymphocytes (usually CD4 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) 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 infiltrate the glands.
  • Interferons stimulate B cell activating factor (BAFF) production → promotes irregular B cell maturation → 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 release autoantibodies
  • 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 and duct epithelial cells produce more cytokines.
  • Cytokines and autoantibodies cause damage and atrophy of the glandular and ductal epithelium Epithelium The epithelium is a complex of specialized cellular organizations arranged into sheets and lining cavities and covering the surfaces of the body. The cells exhibit polarity, having an apical and a basal pole. Structures important for the epithelial integrity and function involve the basement membrane, the semipermeable sheet on which the cells rest, and interdigitations, as well as cellular junctions. Surface Epithelium → ↓ exocrine production (↓ tear and saliva production).
  • Other exocrine glands throughout the body can be involved, as well.
Lymphocytic infiltration of the parotid gland in a patient with sjögren syndrome

Lymphocytic infiltration of the parotid gland in a patient with SS

Image: “Anti-salivary gland protein 1 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 two patients with Sjogren’s syndrome: Two case reports” by Vishwanath S, Shen L, Suresh L, Ambrus JL. License: CC BY 2.0.

Clinical Presentation

Glandular manifestations

  • Keratoconjunctivitis sicca:
    • Symptoms:
      • Xerophthalmia
      • Gritty or sandy feeling
      • Redness
      • Blurry vision
      • Entropion/ectropion
    • Findings:
      • ↓ Tear production
      • Injured corneal tissues 
    • Corneal complications:
      • Ulceration
      • Perforation
  • Salivary hypofunction:
    • Symptoms:
      • Xerostomia
      • Dysphagia Dysphagia Dysphagia is the subjective sensation of difficulty swallowing. Symptoms can range from a complete inability to swallow, to the sensation of solids or liquids becoming "stuck." Dysphagia is classified as either oropharyngeal or esophageal, with esophageal dysphagia having 2 sub-types: functional and mechanical. Dysphagia
      • Food sticking in the mouth
      • Change in taste
      • Difficulty speaking for long periods of time
    • Findings:
      • Dry tongue Tongue The tongue, on the other hand, is a complex muscular structure that permits tasting and facilitates the process of mastication and communication. The blood supply of the tongue originates from the external carotid artery, and the innervation is through cranial nerves. Oral Cavity: Lips and Tongue and lips Lips The lips are the soft and movable most external parts of the oral cavity. The blood supply of the lips originates from the external carotid artery, and the innervation is through cranial nerves. Oral Cavity: Lips and Tongue
      • Glassy appearance of the palate Palate The palate is the structure that forms the roof of the mouth and floor of the nasal cavity. This structure is divided into soft and hard palates. Oral Cavity: Palate
      • Absent sublingual salivary pooling
      • Enlarged salivary and parotid glands
    • Complications:
      • Dental caries 
      • Oral candidiasis Candidiasis Candida is a genus of dimorphic, opportunistic fungi. Candida albicans is part of the normal human flora and is the most common cause of candidiasis. The clinical presentation varies and can include localized mucocutaneous infections (e.g., oropharyngeal, esophageal, intertriginous, and vulvovaginal candidiasis) and invasive disease (e.g., candidemia, intraabdominal abscess, pericarditis, and meningitis). Candida/Candidiasis
      • Parotitis
      • 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
      • Weight loss due to difficulty eating
  • Other glands:
    • Nasal dryness
    • Dry cough
    • Vaginal dryness: 
      • Vulvar infections
      • Dyspareunia

Extraglandular manifestations

  • Cutaneous:
    • Xerosis (dry 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)
    • Raynaud’s phenomenon
    • Cutaneous vasculitis: 
      • Palpable purpura
      • Urticarial lesions
      • Ulcerations
    • 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
    • Livedo reticularis
    • Vitiligo Vitiligo Vitiligo is the most common depigmenting disorder and is caused by the destruction of melanocytes. Patients present with hypo- or depigmented macules or patches which often occur on the face, hands, knees, and/or genitalia. Vitiligo
    • Lichen planus Lichen planus Lichen planus (LP) is an idiopathic, cell-mediated inflammatory skin disease. It is characterized by pruritic, flat-topped, papular, purple skin lesions commonly found on the flexural surfaces of the extremities. Other areas affected include genitalia, nails, scalp, and mucous membranes. Lichen Planus
  • Musculoskeletal:
    • Fatigue
    • Proximal muscle weakness (inflammatory myopathy)
    • 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
    • Arthralgia
    • Arthritis
  • Neurologic: 
    • Cognitive dysfunction
    • Peripheral polyneuropathy Polyneuropathy Polyneuropathy is any disease process affecting the function of or causing damage to multiple nerves of the peripheral nervous system. There are numerous etiologies of polyneuropathy, most of which are systemic and the most common of which is diabetic neuropathy. Polyneuropathy
    • Mononeuritis multiplex
    • Demyelinating disorders
  • Psychiatric: depression
  • Respiratory: 
    • Interstitial lung disease
    • Cystic lung disease
  • Cardiac (rare):
    • 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
    • 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
    • Heart block
  • Gastrointestinal: gastritis Gastritis Gastritis refers to inflammation of the gastric mucosa. Gastritis may occur suddenly (acute gastritis) or slowly over time (chronic gastritis). Gastritis may be asymptomatic or with symptoms, including burning abdominal pain (which either worsens or improves with eating), dyspepsia, nausea, and vomiting. Gastritis
  • Hematologic:
    • 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
    • Monoclonal gammopathy
    • Waldenstrom’s macroglobulinemia
    • Non-Hodgkin’s lymphoma
  • Endocrine:
    • Pancreatitis
    • Autoimmune thyroid disease
  • Renal:
    • Interstitial nephritis
    • Glomerulonephritis
    • Renal tubular acidosis Renal Tubular Acidosis Renal tubular acidosis (RTA) is an imbalance in physiologic pH caused by the kidney's inability to acidify urine to maintain blood pH at physiologic levels. Renal tubular acidosis exist in multiple types, including distal RTA (type 1), proximal RTA (type 2), and hyperkalemic RTA (type 4). Renal Tubular Acidosis

Diagnosis

Laboratory evaluation

  • Anti-SSA/Ro and anti-SSB/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 (present in 60%–70% of patients)
  • Antinuclear 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
  • Rheumatoid factor (present in 40%–60% of patients)
  • Complete blood count:
    • 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
    • Leukopenia
    • 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
  • Basic metabolic panel:
    • Creatinine → evaluate for renal involvement
    • ↓ Potassium and bicarbonate → renal tubular acidosis
  • ↑ Erythrocyte sedimentation rate (ESR) 
  • Urinalysis → evaluate for renal involvement:
    • Proteinuria
    • Hematuria

Ophthalmologic evaluation

  • Schirmer’s test: 
    • Measures tear production
    • Filter paper is placed in the lower eyelid.
    • Wetness is measured after 5 minutes.
    • < 5 mm wetness is considered a positive test.
  • Fluorescein slit-lamp examination and red bengal staining: 
    • Evaluates the cornea and conjunctiva for damage
    • Stain highlights areas of devitalized tissue.

Salivary gland evaluation

  • Sialometry:
    • Measures the rate of saliva production
    • Unstimulated saliva is collected and measured over 15 minutes.
    • Can also be done with stimulation of saliva (with citric acid or pilocarpine)
  • Scintigraphy:
    • Measures salivary gland function through radionuclide uptake
    • Very low uptake is highly specific for SS.
  • Imaging:
    • Ultrasound:
      • Multiple hypoechoic areas with convex borders
      • Hyperdense linear bands, cysts, and calcifications
    • Magnetic resonance imaging (MRI): nodular “honeycomb” appearance of the parotid gland
  • Biopsy: 
    • Performed on a lower lip salivary gland 
    • Histologic findings:
      • Periductal collections of tightly aggregated 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 (focal lymphocytic sialadenitis Sialadenitis Sialadenitis results from inflammation or infection of the glands, and sialolithiasis is due to stone formation in the glands or ducts. Diseases of the Salivary Glands)
      • Parenchymal atrophy
      • Fibrosis
      • Scattered lymphoid cells

Diagnostic criteria

Several criteria have been proposed to aid in the diagnosis of SS. The following is from the American-European Consensus Group (requires 4 of 6 criteria, including a positive biopsy or autoantibodies):

  • Ocular symptoms (at least 1):
    • Dry eyes for ≥ 3 months
    • Foreign body sensation in the eyes
    • Use of artificial tears ≥ 3 times per day
  • Oral symptoms (at least 1):
    • Dry mouth for ≥ 3 months
    • Recurrent or persistently swollen salivary glands Salivary glands The salivary glands are exocrine glands positioned in and around the oral cavity. These glands are responsible for secreting saliva into the mouth, which aids in digestion. There are 3 major paired salivary glands: the sublingual, submandibular, and parotid glands. Salivary Glands
    • Need for liquids to swallow dry foods
  • Ocular signs (at least 1):
    • Positive Schirmer’s test
    • Positive dye staining on the surface of the eye
  • Oral signs (at least 1):
    • Positive sialography
    • Positive scintigraphy
  • Positive biopsy
  • Positive anti-Ro or anti-La (or both)

Management and Complications

Management

  • Multidisciplinary approach (rheumatologist, dentist, and ophthalmologist)
  • Primary SS with disease limited to ocular and oral symptoms:
    • Oral hygiene
    • Smoking cessation
    • Use of a humidifier 
    • Eye protection
    • Artificial tears and saliva substitute
    • Muscarinic agonists: 
      • Pilocarpine
      • Cevimeline
  • Extraglandular involvement:
    • 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
    • Hydroxychloroquine
    • 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):
      • Methotrexate
      • Leflunomide
      • Azathioprine
      • Sulfasalazine
    • Rituximab for severe disease that does not respond to the above therapies
  • Secondary SS can be improved by managing the primary autoimmune disease.

Complications

  • Non-Hodgkin’s lymphoma:
    • Occurs in 5%–10% of patients
    • Usually presents around 7 years after onset
  • Pregnancy Pregnancy Pregnancy is the time period between fertilization of an oocyte and delivery of a fetus approximately 9 months later. The 1st sign of pregnancy is typically a missed menstrual period, after which, pregnancy should be confirmed clinically based on a positive β-HCG test (typically a qualitative urine test) and pelvic ultrasound. Pregnancy: Diagnosis, Maternal Physiology, and Routine Care complications:
    • Premature delivery, intrauterine growth retardation, and fetal loss
    • Neonatal lupus and congenital heart block 
  • Blindness due to corneal scarring
  • Renal failure from progressive renal involvement
  • Pulmonary infections
  • Reduced quality of life

Differential Diagnosis

The differential diagnosis of SS include differentials based on the presenting symptoms as well as other autoimmune disorders:

  • Medication-induced xerophthalmia: Medications such as anticholinergic Anticholinergic Anticholinergic drugs block the effect of the neurotransmitter acetylcholine at the muscarinic receptors in the central and peripheral nervous systems. Anticholinergic agents inhibit the parasympathetic nervous system, resulting in effects on the smooth muscle in the respiratory tract, vascular system, urinary tract, GI tract, and pupils of the eyes. Anticholinergic Drugs agents, antidepressants, antihistamines Antihistamines Antihistamines are drugs that target histamine receptors, particularly H1 and H2 receptors. H1 antagonists are competitive and reversible inhibitors of H1 receptors. First-generation antihistamines cross the blood-brain barrier and can cause sedation. Antihistamines, and beta blockers may cause eye dryness by inhibiting lacrimal gland function. Xerostomia may also be present. Patients with medication-induced xerophthalmia will not have the associated systemic symptoms, and a history of medication initiation will provide a clue to the diagnosis. Laboratory abnormalities will not be present. Management includes symptomatic relief or withdrawal of the offending medication.
  • Conjunctivitis Conjunctivitis Conjunctivitis is a common inflammation of the bulbar and/or palpebral conjunctiva. It can be classified into infectious (mostly viral) and noninfectious conjunctivitis, which includes allergic causes. Patients commonly present with red eyes, increased tearing, burning, foreign body sensation, and photophobia. Conjunctivitis: 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 of the conjunctiva, causing redness, itching, and decreased visual acuity. Etiologies include viral or bacterial infections, and allergies. Watery or purulent discharge is usually present, and will differentiate conjunctivitis from SS. The diagnosis is clinical, and objective tests for SS will be negative. Management depends on the etiology, but can include topical antihistamines Antihistamines Antihistamines are drugs that target histamine receptors, particularly H1 and H2 receptors. H1 antagonists are competitive and reversible inhibitors of H1 receptors. First-generation antihistamines cross the blood-brain barrier and can cause sedation. Antihistamines, antibiotics, or corticosteroids.
  • Mumps Mumps Mumps is caused by a single-stranded, linear, negative-sense RNA virus of the family Paramyxoviridae. Mumps is typically a disease of childhood, which manifests initially with fever, muscle pain, headache, poor appetite, and a general feeling of malaise, and is classically followed by parotitis. Mumps Virus/Mumps: an acute, contagious, viral disease that presents with painful, bilateral parotid swelling. The parotid swelling in SS is not painful. Complications include orchitis Orchitis Epididymitis and orchitis are characterized by acute inflammation of the epididymis and the testicle, respectively, due to viral or bacterial infections. Patients typically present with gradually worsening testicular pain and scrotal swelling along with systemic symptoms such as fever, depending on severity. Epididymitis and Orchitis, meningoencephalitis, and pancreatitis. The diagnosis is clinical, and management is supportive. Mumps Mumps Mumps is caused by a single-stranded, linear, negative-sense RNA virus of the family Paramyxoviridae. Mumps is typically a disease of childhood, which manifests initially with fever, muscle pain, headache, poor appetite, and a general feeling of malaise, and is classically followed by parotitis. Mumps Virus/Mumps can be prevented with a vaccine Vaccine A vaccine is usually an antigenic, non-virulent form of a normally virulent microorganism. Vaccinations are a form of primary prevention and are the most effective form due to their safety, efficacy, low cost, and easy access. Vaccination.
  • Salivary gland tumors: benign or malignant tumors of the parotid or submandibular glands. Patients present with a non-tender mass, which is usually unilateral. Xerostomia and xerophthalmia are not typically present. The diagnosis is made with a biopsy. Malignant tumors are managed with excision and radiation.
  • 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. The parotid and lacrimal glands can be affected, causing painless enlargement. Associated xerostomia, xerophthalmia, arthritis, and interstitial nephritis can also be seen. The diagnosis is made with biopsy, and corticosteroids are used in the management.

References

  1. Baer, A.N. (2020). Diagnosis and classification of Sjögren’s syndrome. UpToDate. Retrieved January 13, 2021, from https://www.uptodate.com/contents/diagnosis-and-classification-of-sjogrens-syndrome
  2. Baer, A.N. (2020). Overview of the management and prognosis of Sjögren’s syndrome. UpToDate. Retrieved January 13, 2021, from https://www.uptodate.com/contents/overview-of-the-management-and-prognosis-of-sjogrens-syndrome
  3. Baer, A.N. (2020). Clinical manifestations of Sjogren’s syndrome: Exocrine gland disease. In Romain, P.L. (Ed.), Uptodate. Retrieved January 16, 2021, from https://www.uptodate.com/contents/clinical-manifestations-of-sjogrens-syndrome-exocrine-gland-disease
  4. Baer, A.N. (2020). Clinical manifestations of Sjögren’s syndrome: Extraglandular disease. UpToDate. Retrieved January 13, 2021, from https://www.uptodate.com/contents/clinical-manifestations-of-sjogrens-syndrome-extraglandular-disease
  5. Ranatunga, S.K. (2020). Sjogren syndrome. In Diamond, H.S. (Ed.), Medscape. Retrieved January 16, 2021, from https://emedicine.medscape.com/article/332125-overview
  6. Nevares, A.M. (2020). Sjogren syndrome. [online] MSD Manual Professional Version. Retrieved January 16, 2021, from https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/autoimmune-rheumatic-disorders/sj%C3%B6gren-syndrome
  7. Nair, J.J., & Singh, T.P. (2017). Sjogren’s syndrome: Review of the aetiology, pathophysiology & potential therapeutic interventions. Journal of clinical and experimental dentistry, 9(4): e584‒e589. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5410683/
  8. Carsons, S.E., & Patel, B.C. (2020). Sjogren syndrome. [online] StatPearls. Retrieved January 16, 2021, from https://www.ncbi.nlm.nih.gov/books/NBK431049/

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