Sarcoidosis

Question 1

sarcoidosis

Answer 1

multi-system disorder of unknown cause

commonly affects young to middle-aged adults

usually characterized by interstitial lung disease and hilar/mediastinal lymphadenopathy

commonly affects eyes, skin, and othe rorgans

noncaseating granulomatous inflammation on biopsy without evidence that granuloma formation was triggered by infection or foreign bodies

decreased cutaneous delayed-type hypersensitivity and increased Th1 inflammatory response at sites of disease activity

Question 2

epidemiology of sarcoidosis

Answer 2

African Americans more at risk

age of diagnosis 20-40

slight female predominance

polygenic, complex, non-medelian inheritance

Question 3

pathogenesis of sarcoidosis

Answer 3

initiation of granuloma formation

deposition of poorly soluble antigenic material

oligoclonal expansion of T cells at sites of disease

Th-1 polarized cytokine response

release of TNF by T cells and macrophages

presence of circulating immune complexes

recent evidence suggests presence of mycobacterial antigens

Question 4

pathology of sarcoidosis

Answer 4

discreet, compact, non-caseating granuloma

highly differentiated macrophages - epithelioid histiocytes and multinucleated giant cells

lymphocytes also present

Question 5

organ distribution of sarcoid granulomas

Answer 5

lung, lymph nodes, liver, spleen, skin

Question 6

lung distribution of non-caseating granulomas

Answer 6

peribronchovascular, perilobular, subpleural

end-stage pulmonary sarcoidosis - parenchymal fibrosis and honeycombing

Question 7

clinical manifestations of sarcoidosis

Answer 7

pulmonary involvement in 90-95%

asymptomatic hilar lymphadenopathy

cough, dyspnea, chest pain

chest physical exam usually normal - clubbing is rare

Question 8

constitutional symptoms of sarcoidosis

Answer 8

fever, fatigue, malaise, wieght loss in 30% of patients

mostly in African Americans

Question 9

skin manifestations of sarcoidosis

Answer 9

erythema nodosum - good prognosis, raise, tender on anterior shin, no granulomas underneath

hyperpigmented plaques - granulomatous lesion underneath

lupus pernio - difficult to treat, associated with poor prognosis

Question 10

Stage I sarcoidosis CXR

Answer 10

bilateral hilar lymphadenopathy without interstitial pulmonary infiltrates

Question 11

Stage II sarcoidosis CXR

Answer 11

bilateral hilar lymphadenopatyh with intersitial infiltrates

Question 12

Stage III sarcoidosis CXR

Answer 12

parynchymal infiltrate without bilateral hilar lymphadenopathy

Question 13

Stage IV CXR and CT

Answer 13

end-stage fibrosis

Question 14

HRCT patterns suggestive of sarcoidosis

Answer 14

nodular opacities typically peribronchovascular in location

often with a “tree-in-bud” appearance, distributed in the upper and middle lung zones

subpleural nodules also seen

Question 15

pulmonary function tests for sarcoidosis

Answer 15

restrictive defect is characteristic

airflow obstruction seen in up to 20%

decreased DLCO is common

correlation of PFTs with pathology and CXR or CT is poor

do not predict response to therapy well

Question 16

diagnostic methods for sarcoidosis

Answer 16

biopsy via flexible bronchoscopy

biopsy of other sites

mediastinoscopy/biopsy

Question 17

What is a favorable predictor of prognosis for sarcoidosis?

Answer 17

Lofgren’s syndrome

Question 18

treatment of sarcoidosis

Answer 18

corticosteroids for stages II and III

treat pulmonary disease when symptoms are severe or progressive

treat neurosarcoidosis, cardiac disease, eye disease

relapse common after tapering of treatment

Question 19

alternative therapies for sarcoidosis

Answer 19

methotrexate - immunosuppresive agent used if steroids are ineffective

infliximab, adalimumab, rituximab - anti-TNF biologic agents, off-label use