Overview
Definition
Sarcoidosis is a multisystem inflammatory disease characterized by the formation of noncaseating granulomas that are most likely caused by a cell-mediated immune reaction of unknown etiology.
Pulmonary sarcoidosis is a restrictive interstitial lung disease with granuloma formation in the:
- Lungs (90% of patients)
- Thoracic lymph nodes (hilar and mediastinal)
Extrapulmonary sarcoidosis is characterized by granuloma formation in:
- Eyes
- Skin
- Joints
- Heart
- Kidney
- CNS and peripheral nervous system Nervous system The nervous system is a small and complex system that consists of an intricate network of neural cells (or neurons) and even more glial cells (for support and insulation). It is divided according to its anatomical components as well as its functional characteristics. The brain and spinal cord are referred to as the central nervous system, and the branches of nerves from these structures are referred to as the peripheral nervous system. General Structure of the Nervous System
- Exocrine glands
Sarcoidosis may be acute or chronic:
- Löfgren syndrome: an acute presentation of sarcoidosis lasting weeks to months:
- Usually resolves spontaneously without treatment
- Typically in younger adults
- Chronic disease: insidious onset, often progressive, may wax and wane
Categorization of
interstitial lung diseases
Interstitial Lung Diseases
Interstitial lung diseases are a heterogeneous group of disorders characterized by the inflammation and fibrosis of lung parenchyma, especially the pulmonary connective tissue in the alveolar walls. It may be idiopathic (e.g., idiopathic pulmonary fibrosis) or secondary to connective tissue diseases, medications, malignancies, occupational exposure, or allergens.
Interstitial Lung Diseases
ILD
ILD
Interstitial lung diseases are a heterogeneous group of disorders characterized by the inflammation and fibrosis of lung parenchyma, especially the pulmonary connective tissue in the alveolar walls. It may be idiopathic (e.g., idiopathic pulmonary fibrosis) or secondary to connective tissue diseases, medications, malignancies, occupational exposure, or allergens.
Interstitial Lung Diseases: interstitial lung disease
Epidemiology
- Most common interstitial lung disease
- Estimated prevalence:
- 34 per 100,000 in African Americans
- 10 per 100,000 in Caucasians
- Age at onset:
- Incidence peaks at 20–40 years of age.
- Second peak in incidence for women between 45 and 65
- Sex bias Bias Epidemiological studies are designed to evaluate a hypothesized relationship between an exposure and an outcome; however, the existence and/or magnitude of these relationships may be erroneously affected by the design and execution of the study itself or by conscious or unconscious errors perpetrated by the investigators or the subjects. These systematic errors are called biases. Types of Biases: women > men
- Racial/ethnic bias Bias Epidemiological studies are designed to evaluate a hypothesized relationship between an exposure and an outcome; however, the existence and/or magnitude of these relationships may be erroneously affected by the design and execution of the study itself or by conscious or unconscious errors perpetrated by the investigators or the subjects. These systematic errors are called biases. Types of Biases: African American > other races
- Most cases are sporadic, but about 5% are familial.
Etiology
Etiology is undetermined but is most likely multifactorial.
Theory: An infectious or environmental agent triggers a cell-mediated inflammatory immune response in a genetically susceptible host, leading to granuloma formation.
Potential triggering exposures:
- Inhalation of talc, aluminum, beryllium, or zirconium
- Exposure to dust and debris at the collapsed World Trade Center after 9/11
- Infectious agents:
- Mycobacterium Mycobacterium Mycobacterium is a genus of the family Mycobacteriaceae in the phylum Actinobacteria. Mycobacteria comprise more than 150 species of facultative intracellular bacilli that are mostly obligate aerobes. Mycobacteria are responsible for multiple human infections including serious diseases, such as tuberculosis (M. tuberculosis), leprosy (M. leprae), and M. avium complex infections. Mycobacterium tuberculosis Tuberculosis Tuberculosis (TB) is an infectious disease caused by Mycobacterium tuberculosis complex bacteria. The bacteria usually attack the lungs but can also damage other parts of the body. Approximately 30% of people around the world are infected with this pathogen, with the majority harboring a latent infection. Tuberculosis spreads through the air when a person with active pulmonary infection coughs or sneezes. Tuberculosis
- Cutibacterium (previously Propionibacterium) acnes
Genetic predispositions:
- Antigens of the major histocompatibility complex (MHC), especially the HLA-DR alleles:
- HLA-DRB1*1101
- HLA-DQB1*0201
- Possible association with ACE variants
- Ongoing studies looking at gene networks
Pathophysiology
Sarcoidosis is the result of a cell-mediated immune reaction.
- Phagocytosis of a new antigen by antigen-presenting cells (e.g., macrophages and dendritic cells)
- Activated macrophages present the antigen to helper 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 via the HLA-CD4 complex.
- Activated
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 macrophages release inflammatory mediators (Th1 response):
- Interleukin 2 (IL-2)
- Interferon gamma
- 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))
- Other cytokines and chemokines
- Inflammatory mediators cause macrophages to fuse into multinucleated giant cells.
- Unable to destroy the antigens, the multinucleated giant cells wall them off → noncaseating granuloma formation
- Fibroblasts are recruited and surround granulomas.
- Granulomas may progress to fibrosis; the mechanism is poorly understood.
Noncaseating granuloma
Image: “Granuloma 20x” by Mutleysmith. License: Public DomainClinical Presentation
Sarcoidosis usually progresses slowly, with few symptoms initially. Symptoms appear as an increasing number of granulomas begin to affect organ function.
| Organ system | Clinical presentation of sarcoidosis |
|---|---|
| Systemic symptoms (typically in acute presentations) |
|
| Pulmonary |
|
| Lymph |
|
| Ocular |
|
| Cutaneous |
|
| Musculoskeletal |
|
| Cardiac |
|
| Nervous system |
|
| Other potential sites of involvement |
|
Löfgren syndrome
An acute presentation of sarcoidosis with a classic triad of symptoms:
- Erythema nodosum
- Hilar adenopathy
- Migratory polyarthralgia
Diagnosis
Diagnosing sarcoidosis typically requires:
- Compatible clinical and radiologic manifestations
- Noncaseating granulomas on pathology
- Exclusion of other conditions
Imaging
- Chest X-ray:
- Best initial test
- Findings:
- Bilateral hilar 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 (classic finding)
- Bilateral reticulonodular infiltrates (often upper lobes)
- Honeycombing (end-stage disease)
- High-resolution CT (HRCT):
- Higher sensitivity than X-ray
- Indications:
- Confirm findings from suspicious X-ray
- Further evaluate lungs Lungs Lungs are the main organs of the respiratory system. Lungs are paired viscera located in the thoracic cavity and are composed of spongy tissue. The primary function of the lungs is to oxygenate blood and eliminate CO2. Lungs in patient with pulmonary symptoms but normal X-ray
- Findings:
- Bilateral hilar and mediastinal 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
- Small centrilobular parenchymal nodules
- Mid–upper zone predominance of lung parenchymal changes
- Fine nodularity
- Ground-glass opacification
- Fibrosis with lung distortion and traction bronchiectasis Bronchiectasis Bronchiectasis is a chronic disease of the airways that results from permanent bronchial distortion. This results from a continuous cycle of inflammation, bronchial damage and dilation, impaired clearance of secretions, and recurrent infections. Bronchiectasis
Chest X-ray in a patient with chronic sarcoidosis:
Image: “Pulmonary infiltrates of sarcoidosis” by Division of Pediatric Rheumatology, Louisiana State University Medical Center and Children’s Hospital of New Orleans, LA, USA. License: CC BY 2.0, edited by Lecturio.
Green circles denote areas of upper lobe interstitial infiltrates. There is also distortion of the hilum.
Non–contrast-enhanced chest CT scan demonstrating pulmonary nodules and calcified mediastinal and hilar 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
Image: “Noncontrast-computed tomography scan” by Penn State/Milton S, Hershey Medical Center Department of Medicine, 500 University Drive, Hershey PA 17033, Pennsylvania, USA. License: CC BY 4.0
Bronchoscopy
- Bronchoalveolar lavage (BAL):
- Obtained primarily to exclude alternative diagnosis (e.g., infections, malignancy)
- Lymphocytosis Lymphocytosis WBCs develop from stem cells in the bone marrow and are called leukocytes when circulating in the bloodstream. Lymphocytes are 1 of the 5 subclasses of WBCs. Lymphocytosis is an increase in the number or proportion of the lymphocyte subclass of WBCs, often as a result of an immune response to infection (known as reactive lymphocytosis). Lymphocytosis ≥ 25% suggests a granulomatous process.
- Findings consistent with pulmonary sarcoidosis:
- ↓ CD8 cells
- ↑ CD4 cells, activated 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 IgG-secreting cells
- ↑ CD4:CD8 ratio > 4:1
- Allows for less invasive pulmonary tissue sampling:
- Endobronchial biopsy
- Transbronchial lung biopsy
- Transbronchial needle aspiration
Biopsy
- Gold standard for diagnosis
- Biopsy the most accessible affected site (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 lesions).
- Lung biopsies can be obtained via bronchoscopy or surgically (more invasive).
- Key finding: well-defined noncaseating granulomas
- Noncaseating: no central necrosis
- Multinucleated giant cells
- Other histopathologic findings:
- Asteroid bodies
- Schaumann bodies
Noncaseating granuloma
Image: “Granuloma 20x” by Mutleysmith. License: Public Domain
Spirometry
Obtained to assess severity of respiratory involvement and monitor disease; less helpful in actually establishing diagnosis
- Sarcoid usually demonstrates a restrictive pattern:
- ↓ Forced vital capacity (FVC; total forced exhaled volume)
- ↓ Total lung capacity (TLC; total volume in lungs Lungs Lungs are the main organs of the respiratory system. Lungs are paired viscera located in the thoracic cavity and are composed of spongy tissue. The primary function of the lungs is to oxygenate blood and eliminate CO2. Lungs at the end of a maximal inspiration)
- ↓ Compliance ( lungs Lungs Lungs are the main organs of the respiratory system. Lungs are paired viscera located in the thoracic cavity and are composed of spongy tissue. The primary function of the lungs is to oxygenate blood and eliminate CO2. Lungs have difficulty expanding)
- Mixed restrictive/obstructive patterns are also possible in severe disease:
- Mixed patterns have both restrictive and obstructive findings.
- Obstructive findings:
- ↓ Forced expiratory volume in 1 second (FEV1):FVC ratio
- ↓ Airflow (airways are partially obstructed by 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 fibrosis)
- Impaired gas diffusion across the alveolar membrane: ↓ diffusing capacity of the lung for carbon monoxide (DLCO)
Other testing
- Electrocardiography: to assess for cardiac involvement
- Ophthalmologic examination: to assess for eye involvement
- Tuberculin 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 test: to rule out tuberculosis Tuberculosis Tuberculosis (TB) is an infectious disease caused by Mycobacterium tuberculosis complex bacteria. The bacteria usually attack the lungs but can also damage other parts of the body. Approximately 30% of people around the world are infected with this pathogen, with the majority harboring a latent infection. Tuberculosis spreads through the air when a person with active pulmonary infection coughs or sneezes. Tuberculosis
- Other testing guided by clinical presentation
Laboratory testing
These tests are important in assessing and monitoring the extent of disease rather than making the diagnosis. The following findings are often seen in sarcoidosis.
- CBC:
- Leukopenia
- Lymphopenia
- 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 (uncommon)
- CMP:
- Hypercalcemia Hypercalcemia Hypercalcemia (serum calcium > 10.5 mg/dL) can result from various conditions, the majority of which are due to hyperparathyroidism and malignancy. Other causes include disorders leading to vitamin D elevation, granulomatous diseases, and the use of certain pharmacological agents. Symptoms vary depending on calcium levels and the onset of hypercalcemia. Hypercalcemia
- ↑ Liver function tests Liver function tests Liver function tests, also known as hepatic function panels, are one of the most commonly performed screening blood tests. Such tests are also used to detect, evaluate, and monitor acute and chronic liver diseases. Liver Function Tests if liver involvement
- ↑ BUN and creatinine if renal involvement
- Urinalysis:
- Hypercalciuria
- Inflammatory markers:
- ↑ ESR
- ↑ CRP
- Others:
- ↑ ACE
- ↑ IgG
Staging Staging Cancer is the 2nd leading cause of death in the US after cardiovascular disease. Many malignancies are treatable or curable, but some may recur. Thus, all malignancies must be assigned a grade and stage in order to guide management and determine prognosis. Grading, Staging, and Metastasis
| Stage | Chest X-ray | Symptoms | Frequency |
|---|---|---|---|
| 0 | Extrapulmonary disease only | None | 10% |
| 1 | BHL only | None or mild cough | 50% |
| 2 | BHL plus parenchymal infiltrates | None, 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, or cough | 25% |
| 3 | Parenchymal infiltrates only | Dyspnea | 10% |
| 4 | Advanced pulmonary fibrosis Pulmonary Fibrosis Idiopathic pulmonary fibrosis is a specific entity of the major idiopathic interstitial pneumonia classification of interstitial lung diseases. As implied by the name, the exact causes are poorly understood. Patients often present in the moderate to advanced stage with progressive dyspnea and nonproductive cough. Pulmonary Fibrosis | Dyspnea | 5% |
Management
Management is based on stage and location of disease and includes:
- Observation:
- Indicated in patients with high rates of spontaneous resolution:
- Acute sarcoidosis
- Stage 1 chronic sarcoidosis
- NSAIDs for treatment of pain if needed
- Indicated in patients with high rates of spontaneous resolution:
- Corticosteroids: 1st-line therapy in patients with:
- ≥ Stage 2 disease with simultaneously restricted pulmonary function
- Renal insufficiency (hypercalcemia and hyperuricemia)
- Extrapulmonary involvement
- Severe systemic symptoms
- Severe arthritis (Löfgren syndrome)
- Localized symptoms including uveitis and 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 lesions → administered locally
- Steroid-sparing agents: 2nd-line therapy:
- Azathioprine
- Methotrexate
- Mycophenolate
- Hydroxychloroquine
- Leflunomide
- Infliximab (anti- 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) agent)
- Lung transplantation: in cases of advanced pulmonary fibrosis Pulmonary Fibrosis Idiopathic pulmonary fibrosis is a specific entity of the major idiopathic interstitial pneumonia classification of interstitial lung diseases. As implied by the name, the exact causes are poorly understood. Patients often present in the moderate to advanced stage with progressive dyspnea and nonproductive cough. Pulmonary Fibrosis
Prognosis
| Stage | Remission rate |
|---|---|
| 0 | – |
| 1 | 90% |
| 2 | 70% |
| 3 | 20% |
| 4 | < 5% |
Differential Diagnosis
- Pneumoconiosis Pneumoconiosis Pneumoconiosis is an occupational disease that results from the inhalation and deposition of mineral dusts and other inorganic particles in the lung. It can be categorized according to the type of causative particle involved or by the type of response provoked. Pneumoconiosis: occupational disease that consists of a group of restrictive interstitial lung diseases Interstitial Lung Diseases Interstitial lung diseases are a heterogeneous group of disorders characterized by the inflammation and fibrosis of lung parenchyma, especially the pulmonary connective tissue in the alveolar walls. It may be idiopathic (e.g., idiopathic pulmonary fibrosis) or secondary to connective tissue diseases, medications, malignancies, occupational exposure, or allergens. Interstitial Lung Diseases caused by inhalation of toxic dust, including silica, asbestos, beryllium, and others: Pneumoconiosis Pneumoconiosis Pneumoconiosis is an occupational disease that results from the inhalation and deposition of mineral dusts and other inorganic particles in the lung. It can be categorized according to the type of causative particle involved or by the type of response provoked. Pneumoconiosis presents with cough and 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. Diagnosis is by occupational history, imaging, and histology. Management is largely supportive.
- Histoplasmosis Histoplasmosis Histoplasmosis is an infection caused by Histoplasma capsulatum, a dimorphic fungus. Transmission is through inhalation, and exposure to soils containing bird or bat droppings increases the risk of infection. Most infections are asymptomatic; however, immunocompromised individuals generally develop acute pulmonary infection, chronic infection, or even disseminated disease. Histoplasma/Histoplasmosis: infection caused by the fungus Histoplasma Histoplasma Histoplasmosis is an infection caused by Histoplasma capsulatum, a dimorphic fungus. The fungus exists as a mold at low temperatures and as yeast at high temperatures. H. capsulatum is the most common endemic fungal infection in the US and is most prevalent in the midwestern and central states along the Ohio and Mississippi River valleys. Histoplasma/Histoplasmosis capsulatum: Histoplasmosis Histoplasmosis Histoplasmosis is an infection caused by Histoplasma capsulatum, a dimorphic fungus. Transmission is through inhalation, and exposure to soils containing bird or bat droppings increases the risk of infection. Most infections are asymptomatic; however, immunocompromised individuals generally develop acute pulmonary infection, chronic infection, or even disseminated disease. Histoplasma/Histoplasmosis presents with symptoms of 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, including 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, headache, myalgias, cough, and chest pain Chest Pain Chest pain is one of the most common and challenging complaints that may present in an inpatient and outpatient setting. The differential diagnosis of chest pain is large and includes cardiac, gastrointestinal, pulmonary, musculoskeletal, and psychiatric etiologies. Chest Pain that develop 2–4 weeks after exposure. In immunocompromised individuals, the infection may be fatal. Both sarcoidosis and histoplasmosis can cause pulmonary infiltrates, mediastinal 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, and 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. Histoplasmosis Histoplasmosis Histoplasmosis is an infection caused by Histoplasma capsulatum, a dimorphic fungus. Transmission is through inhalation, and exposure to soils containing bird or bat droppings increases the risk of infection. Most infections are asymptomatic; however, immunocompromised individuals generally develop acute pulmonary infection, chronic infection, or even disseminated disease. Histoplasma/Histoplasmosis tends to form caseating granulomas, while sarcoidosis causes noncaseating granulomas. Treatment involves antifungal medications.
- Tuberculosis: disease caused by Mycobacterium Mycobacterium Mycobacterium is a genus of the family Mycobacteriaceae in the phylum Actinobacteria. Mycobacteria comprise more than 150 species of facultative intracellular bacilli that are mostly obligate aerobes. Mycobacteria are responsible for multiple human infections including serious diseases, such as tuberculosis (M. tuberculosis), leprosy (M. leprae), and M. avium complex infections. Mycobacterium tuberculosis Tuberculosis Tuberculosis (TB) is an infectious disease caused by Mycobacterium tuberculosis complex bacteria. The bacteria usually attack the lungs but can also damage other parts of the body. Approximately 30% of people around the world are infected with this pathogen, with the majority harboring a latent infection. Tuberculosis spreads through the air when a person with active pulmonary infection coughs or sneezes. Tuberculosis: The bacteria Bacteria Bacteria are prokaryotic single-celled microorganisms that are metabolically active and divide by binary fission. Some of these organisms play a significant role in the pathogenesis of diseases. Bacteriology: Overview usually attacks the lungs Lungs Lungs are the main organs of the respiratory system. Lungs are paired viscera located in the thoracic cavity and are composed of spongy tissue. The primary function of the lungs is to oxygenate blood and eliminate CO2. Lungs, but it can also damage other parts of the body. Tuberculosis presents with a chronic cough with hemoptysis Hemoptysis Hemoptysis is defined as the expectoration of blood originating in the lower respiratory tract. Hemoptysis is a consequence of another disease process and can be classified as either life threatening or non-life threatening. Hemoptysis can result in significant morbidity and mortality due to both drowning (reduced gas exchange as the lungs fill with blood) and hemorrhagic shock. Hemoptysis. The diagnosis is made with a tuberculin 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 test, sputum cultures, and lung imaging. Biopsies show caseating granulomas (central necrosis), whereas sarcoid granulomas are noncaseating. Management is with antimycobacterial drugs.
- Idiopathic pulmonary fibrosis Pulmonary Fibrosis Idiopathic pulmonary fibrosis is a specific entity of the major idiopathic interstitial pneumonia classification of interstitial lung diseases. As implied by the name, the exact causes are poorly understood. Patients often present in the moderate to advanced stage with progressive dyspnea and nonproductive cough. Pulmonary Fibrosis: interstitial lung disease, characterized by irreversible pulmonary fibrosis Pulmonary Fibrosis Idiopathic pulmonary fibrosis is a specific entity of the major idiopathic interstitial pneumonia classification of interstitial lung diseases. As implied by the name, the exact causes are poorly understood. Patients often present in the moderate to advanced stage with progressive dyspnea and nonproductive cough. Pulmonary Fibrosis and impaired pulmonary function: Idiopathic pulmonary fibrosis Pulmonary Fibrosis Idiopathic pulmonary fibrosis is a specific entity of the major idiopathic interstitial pneumonia classification of interstitial lung diseases. As implied by the name, the exact causes are poorly understood. Patients often present in the moderate to advanced stage with progressive dyspnea and nonproductive cough. Pulmonary Fibrosis presents with exertional 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, persistent dry cough, and fatigue. Diagnosis is by chest X-ray or CT, along with pulmonary function tests. Management is supportive, along with therapy targeting the underlying cause.
- Lymphoma: cancers originating from lymphocytes, including 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, 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 natural killer (NK) cells: Lymphomas often present with constitutional signs and/or painless 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. Similar to sarcoidosis, imaging findings may include thoracic 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. Management is based on the specific stage and type of cancer and is usually chemotherapy.
References
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