Granulomatous Diseases: Sarcoidosis Overview

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Questions and Answers

In what percentage of sarcoidosis patients do granulomas eventually lead to "honeycomb lung"?

  • 90%
  • 75-90%
  • 5-15% (correct)
  • 25%

Which of the following is NOT considered a characteristic of sarcoidosis?

  • Noncaseating granulomas in multiple tissues and organs
  • Higher prevalence among people of African descent in the United States
  • A predilection for adults younger than 40 years of age
  • A higher prevalence among smokers (correct)
  • High incidence in Danish and Swedish populations

What is the characteristic texture of lymph nodes in patients with sarcoidosis?

  • Soft and spongy
  • Firm and rubbery (correct)
  • Fluid-filled and cystic
  • Hard and calcified

What is the typical distribution of granulomas in the lungs of sarcoidosis patients?

<p>Clustered around blood vessels and bronchioles, often in the pleura (D)</p> Signup and view all the answers

What type of immune response is suggested to be involved in the pathogenesis of sarcoidosis?

<p>Cell-mediated response driven by CD4+ helper T cells (D)</p> Signup and view all the answers

Which of the following is NOT a characteristic of the immunologic abnormalities observed in sarcoidosis?

<p>Oligoclonal expansion of CD8+ Th1 T cells within the lung (B)</p> Signup and view all the answers

Which of the following is NOT a histopathological feature found in sarcoidosis granulomas?

<p>Central necrosis with caseation (C)</p> Signup and view all the answers

What distinguishes sarcoidosis lymph nodes from those affected by tuberculosis?

<p>Sarcoidosis lymph nodes do not undergo necrosis and are not adherent. (A)</p> Signup and view all the answers

Which of the following pairs of cytokines are NOT both directly implicated in the cell-mediated response associated with sarcoidosis?

<p>TNF and IL-10 (D)</p> Signup and view all the answers

What is the most common location for granulomas in sarcoidosis patients?

<p>Lungs (D)</p> Signup and view all the answers

Why is the histologic diagnosis of sarcoidosis often one of exclusion?

<p>Noncaseating granulomas can be found in other diseases, making it difficult to definitively confirm sarcoidosis based on histology alone. (D)</p> Signup and view all the answers

What is the cardinal histopathologic feature of sarcoidosis?

<p>Nonnecrotizing epithelioid granulomas (A)</p> Signup and view all the answers

What is the most common presentation of sarcoidosis, based on chest radiographs?

<p>Bilateral hilar lymphadenopathy and lung involvement (C)</p> Signup and view all the answers

Which of the following cells are abundant in bronchoalveolar lavage fluid of sarcoidosis patients?

<p>CD4+ T cells (D)</p> Signup and view all the answers

Which of the following is NOT a contributing factor to the pathogenesis of sarcoidosis, according to the provided text?

<p>Autoimmune response (E)</p> Signup and view all the answers

Based on the information provided, what is the most likely explanation for the higher prevalence of sarcoidosis among people of African descent in the United States?

<p>Genetic predisposition (C)</p> Signup and view all the answers

What is the primary feature of erythema nodosum associated with acute sarcoidosis?

<p>Bilateral raised, red, tender nodules (C)</p> Signup and view all the answers

Which complication of ocular involvement in sarcoidosis may lead to significant visual impairment?

<p>Glaucoma (B)</p> Signup and view all the answers

Which of the following statements about spleen involvement in sarcoidosis is incorrect?

<p>Hepatomegaly is more common than splenomegaly. (C)</p> Signup and view all the answers

What is Mikulicz syndrome in the context of sarcoidosis?

<p>Combined uveoparotid involvement (A)</p> Signup and view all the answers

What percentage of patients is likely to show bone marrow involvement in sarcoidosis?

<p>Up to 40% (C)</p> Signup and view all the answers

What common respiratory symptom is typically observed in symptomatic cases of sarcoidosis?

<p>Dry cough (A)</p> Signup and view all the answers

Which of the following is least likely to be a presenting manifestation of sarcoidosis?

<p>Cirrhosis of the liver (C)</p> Signup and view all the answers

Which statement about chronic inflammatory cells in erythema nodosum is correct?

<p>They are marked by fibrosis and chronic inflammatory cell infiltrates. (A)</p> Signup and view all the answers

What is the primary type of immune cell that predominates in the histopathologic picture of hypersensitivity pneumonitis?

<p>Lymphocytes (A)</p> Signup and view all the answers

Which of the following clinical features is typical of acute hypersensitivity pneumonitis?

<p>Cough and dyspnea after 4 to 8 hours post-exposure (A)</p> Signup and view all the answers

Which characteristic granulomas are typically seen in hypersensitivity pneumonitis?

<p>Noncohesive granulomas without necrosis (C)</p> Signup and view all the answers

What is a potential outcome if the inciting agent causing hypersensitivity pneumonitis is not eliminated from the environment?

<p>Irreversible chronic interstitial pulmonary disease (A)</p> Signup and view all the answers

What is required for establishing the diagnosis of sarcoidosis?

<p>Identifying noncaseating granulomas in involved tissues. (C)</p> Signup and view all the answers

Pulmonary eosinophilia is primarily characterized by which of the following?

<p>Idiopathic pulmonary infiltrates rich in eosinophils (B)</p> Signup and view all the answers

Which statement about the course of sarcoidosis is true?

<p>Approximately 65% to 70% of affected individuals recover with few manifestations. (A)</p> Signup and view all the answers

Which form of smoking-related interstitial disease is characterized by a collection of macrophages in the alveoli?

<p>Desquamative interstitial pneumonia (B)</p> Signup and view all the answers

What type of lung pathology is commonly associated with smoking besides obstructive lung disease?

<p>Restrictive or interstitial lung diseases (A)</p> Signup and view all the answers

What is a characteristic laboratory finding in hypersensitivity pneumonitis?

<p>Increased numbers of T lymphocytes in bronchoalveolar lavage. (A)</p> Signup and view all the answers

Which mechanisms are primarily responsible for recruiting eosinophils to the lungs in eosinophilic pulmonary disorders?

<p>Local release of chemotactic factors (B)</p> Signup and view all the answers

What defines hypersensitivity pneumonitis as an immunologically mediated disease?

<p>Specific antibodies against the inhaled antigens found in serum. (D)</p> Signup and view all the answers

What are the potential long-term outcomes for individuals with sarcoidosis?

<p>A fraction may suffer from permanent lung dysfunction or cor pulmonale. (A)</p> Signup and view all the answers

Which of the following sources is commonly associated with hypersensitivity pneumonitis?

<p>Moldy hay containing fungi. (C)</p> Signup and view all the answers

Which characteristic is NOT typical of hypersensitivity pneumonitis?

<p>Presence of caseating granulomas in bronchoalveolar lavage. (B)</p> Signup and view all the answers

In the context of sarcoidosis, what must be excluded prior to diagnosis?

<p>Tuberculosis. (D)</p> Signup and view all the answers

Flashcards

Sarcoidosis

A multisystem disease characterized by noncaseating granulomatous inflammation.

Restrictive Lung Disease

A type of lung disease that restricts airflow due to reduced lung volume.

Bilateral Hilar Lymphadenopathy

Enlargement of lymph nodes on both sides of the mediastinum, often seen in sarcoidosis.

Epidemiology of Sarcoidosis

Examines the distribution and trends of sarcoidosis in populations.

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CD4+ T Cells

A type of helper T cell that plays a crucial role in the immune response in sarcoidosis.

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Immune Regulation

The process by which the immune system maintains balance and appropriate response.

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Oligoclonal Expansion

The increase in a limited number of T cells due to antigen exposure in the lung.

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Granuloma

A small area of inflammation in tissue, characteristic of sarcoidosis, formed by immune cells.

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CD4+ Helper T cells

A type of immune cell crucial for immune responses, often low in certain conditions.

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Anergy

A state of unresponsiveness of the immune system to specific antigens.

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Lung Transplant and Sarcoidosis

Sarcoidosis can recur in at least one-third of lung transplant patients.

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Epithelioid Granuloma

The hallmark histopathologic feature of sarcoidosis , composed of macrophages and T cells.

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Schaumann Bodies

Laminated concretions found in granulomas, made of calcium and proteins, not required for diagnosis.

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Honeycomb Lung

Diffuse interstitial fibrosis resulting from replaces granulomas in some sarcoidosis patients.

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Lymph Node Involvement

Enlarged hilar and paratracheal lymph nodes in 75-90% of sarcoidosis patients, typically painless and firm.

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Erythema nodosum

Bilateral raised, red, tender nodules on the legs, a sign of acute sarcoidosis.

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Panniculitis

Inflammation of subcutaneous fat, often seen in erythema nodosum.

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Ocular involvement in sarcoidosis

Involvement can cause iritis or iridocyclitis, affecting vision and leading to complications.

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Mikulicz syndrome

Combined involvement of the eyes and parotid glands in sarcoidosis.

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Granulomas in spleen

Usually present in three-fourths of cases, but the spleen may appear normal.

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Liver involvement in sarcoidosis

Microscopic granulomas found, mainly in portal triads, often without symptoms.

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Bone marrow involvement

Detected in 40% of patients but rarely shows severe symptoms.

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Symptoms of sarcoidosis

May include respiratory issues or constitutional symptoms like fever and fatigue.

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Hypersensitivity Pneumonitis

An inflammatory response in the lungs due to inhaled antigens, causing cough and dyspnea.

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Acute Reaction Symptoms

Fever, cough, dyspnea occurring 4 to 8 hours after antigen exposure.

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Chronic Disease Symptoms

Gradual onset of cough, dyspnea, malaise, and weight loss over time.

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Granulomas in HP

Poorly formed granulomas without necrosis, often located around bronchioles.

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Pulmonary Eosinophilia

Lung diseases characterized by eosinophil-rich infiltrates, often with unknown causes.

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Smoking-Related Diseases

Lung diseases like desquamative interstitial pneumonia linked to tobacco use.

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Interstitital Fibrosis

Scarring of lung tissue that develops in chronic pulmonary disorders.

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Neutrophils in Acute HP

Variable neutrophil presence noted in acute hypersensitivity pneumonitis.

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Hypercalcemia

An abnormally high level of calcium in the blood, often seen in sarcoidosis.

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Diagnosis of Sarcoidosis

Requires clinical and radiologic findings, excluding other disorders, and finding noncaseating granulomas.

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Course of Sarcoidosis

An unpredictable progression, may be chronic or interspersed with remissions, with recovery possible.

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Antigens in Hypersensitivity Pneumonitis

Sources include moldy hay, contaminated wood, and dairy barns.

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Immunological Evidence

Increased CD4+ and CD8+ T lymphocytes, antibodies against antigens, and noncaseating granulomas are observed.

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Noncaseating Granulomas

Clusters of immune cells often found in the lungs of patients with sarcoidosis and hypersensitivity pneumonitis.

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Outcomes of Sarcoidosis

65%-70% recover, while 20% have lung dysfunction, and 10%-15% may progress to severe complications.

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Study Notes

Granulomatous Diseases: Sarcoidosis

  • Sarcoidosis is a multisystem disease, of unknown cause,
  • It's characterized by non-caseating granulomatous inflammation in various organs.

Pathogenesis of Sarcoidosis

  • The cause of sarcoidosis remains unknown.
  • Evidence suggests a disordered immune response in genetically susceptible individuals exposed to unknown environmental factors.
  • The disease involves CD4+ helper T cells and abnormal immune responses.

Morphology of Sarcoidosis

  • The defining feature is noncaseating granulomas, clusters of immune cells.
  • Epithelioid macrophages are central to these granulomas.
  • Often accompanied by multinucleated giant cells.
  • Schaumann bodies (concretions of calcium) and asteroid bodies (stellar inclusions) may be present but aren't essential for diagnosis.
  • Over time, granulomas can scar and be replaced by fibrous tissue.

Clinical Features of Sarcoidosis

  • May be asymptomatic, or have various symptoms, including:
  • Respiratory issues (cough, shortness of breath)
  • Eye and skin involvement (swollen glands, rash).
  • Spleen and liver enlargement.
  • Occasionally, elevated calcium levels, due to vitamin D production.
  • Diagnosis relies on clinical symptoms, radiographic findings.
  • Key is excluding other diseases with similar manifestations; specifically ruling out tuberculosis.

Hypersensitivity Pneumonitis

  • Characterized by an inflammatory lung condition triggered by an immune response to inhaled antigens.
  • An immunological mediated lung disease affecting the alveoli.
  • Often caused by exposure to antigens like molds or other substances in the environment (e.g., in farms or workplaces).
  • May present with acute symptoms (fever, cough, shortness of breath) or chronic disease (progressive lung damage).
  • Bronchoalveolar lavage shows increased numbers of CD4+ and CD8+ T lymphocytes.
  • Smoking is linked to some interstitial lung diseases like Desquamative interstitial pneumonia (DIP).
  • DIP has macrophages filled with brown pigment, indicative of prior smoking.
  • Respiratory bronchiolitis is another smoking-related condition showing pigmented intraluminal macrophages in respiratory bronchioles.
  • Both conditions can lead to fibrosis.

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