Granulomatous Diseases (Restrictive)

Question 1

How is sarcoidosis characterized?

Answer 1

Multi-system disorder of unknown ideology characterized by the presence of non-caseating granulomas in organs

bilateral hilar lymphadenopathy

Question 2

Patient is a 22-year-old male, presenting with progressive dry cough, fatigue, and SOB. CXR shows multiple non-caseating granulomas and bilateral hilar lymphadenopathy. What is most likely going on?

Answer 2

sarcoidosis: Multi-system disorder of unknown ideology characterized by the presence of non-caseating granulomas in organs, most often in young adults

bilateral hilar lymphadenopathy

Question 3

Which type of T cell is thought to play a significant role in the development of sarcoidosis?

Answer 3

Th1 CD4+ cells - promote cellular immunity

sarcoidosis: noncaseating granulomas and bilateral hilar lymphadenopathy

Question 4

Describe the non-necrotizing epithelioid granulomas seen in sarcoidosis

Answer 4

aggregates of tightly clustered epithelioid macrophages often with giant cells rimmed by an outer zone rich in CD4 + T cells. Central necrosis is uncommon. Lymph nodes are involved in almost all cases, particularly the hilar and mediastinal lymph nodes.

Question 5

bronchoalveolar lavage fluid from patients with sarcoidosis contains abundant _____

Answer 5

CD4+ T cells

granulomas predominately involve interstitium rather than air spaces and localize in connective tissue around bronchioles and pulmonary vessels

Question 6

How do patients with sarcoidosis typically present?

Answer 6

persistent, dry, cough, fatigue, and shortness of breath.

Can also have painful red lumps on skin, uveitis/blurring of vision, hoarseness of voice, palpable lymph nodes on multiple sites and neurological manifestations

sarcoidosis is also a cause of cardiomyopathy and AV block

[sarcoidosis: noncaseating granulomas]

Question 7

lupus pernio

Answer 7

variants of cutaneous sarcoidosis (noncaseating granulomas) that present with violaceous or erythematous papules/ plaques/ nodules mainly involving central facial skin

Question 8

why might a patient with sarcoidosis present with elevated serum and urine calcium?

Answer 8

abundant macrophages in granulomas, macrophages can convert 25 – vitamin D to it’s active form, which increases calcium absorption

Question 9

how does sarcoidosis effect DLCO?

Answer 9

[sarcoidosis: noncaseating granulomas]

restrictive disease pattern —> decrease in DLCO

Question 10

HRCT

Answer 10

high resolution computed tomography

obtained to evaluate abnormalities seen on chest radiograph, or to evaluate unexplained dyspnea or cough in a patient with a clear chest radiograph

Question 11

what occupational exposure is often misdiagnosed as sarcoidosis?

Answer 11

chronic Beryllium disease – seen in patients involved in manufacture of alloys, ceramics or high technology electronics

also manifests as non-caseating granulomas in lung parenchyma and hilar lymph nodes

distinguished from sarcoidosis by specific cell mediated immune response to beryllium (delayed hypersensitivity)

Question 12

How can chronic beryllium disease be differentiated from sarcoidosis in a patient?

Answer 12

chronic Berylium disease is positive for a specific cell mediated immune response to beryllium (delayed hypersensitivity)

Question 13

How does acute vs subacute vs chronic hypersensitivity pneumonitis a.k.a. extrinsic allergic alveolitis appear radiographically?

Answer 13

acute: diffuse, ground-glass appearance or air space consolidation

subacute: CXR shows fine nodular or reticulonodular pattern

chronic: prominently reticular pattern

*distribution mainly in upper 2/3 of lungs

Question 14

Asbestos exposure usually shows linear opacities in which lobe of the lung on chest x-ray?

Answer 14

asbestosis - lower lobe linear opacities on CXR

Question 15

interstitial lung disease + hypercalcemia =

Answer 15

sarcoidosis

(*step1 tip)