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Flashcards in Sarcoidosis Deck (19)
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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


epidemiology of sarcoidosis

African Americans more at risk

age of diagnosis 20-40

slight female predominance

polygenic, complex, non-medelian inheritance


pathogenesis of sarcoidosis

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


pathology of sarcoidosis

discreet, compact, non-caseating granuloma

highly differentiated macrophages - epithelioid histiocytes and multinucleated giant cells

lymphocytes also present


organ distribution of sarcoid granulomas

lung, lymph nodes, liver, spleen, skin


lung distribution of non-caseating granulomas

peribronchovascular, perilobular, subpleural

end-stage pulmonary sarcoidosis - parenchymal fibrosis and honeycombing


clinical manifestations of sarcoidosis

pulmonary involvement in 90-95%

asymptomatic hilar lymphadenopathy

cough, dyspnea, chest pain

chest physical exam usually normal - clubbing is rare


constitutional symptoms of sarcoidosis

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

mostly in African Americans


skin manifestations of sarcoidosis

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


Stage I sarcoidosis CXR

bilateral hilar lymphadenopathy without interstitial pulmonary infiltrates


Stage II sarcoidosis CXR

bilateral hilar lymphadenopatyh with intersitial infiltrates


Stage III sarcoidosis CXR

parynchymal infiltrate without bilateral hilar lymphadenopathy


Stage IV CXR and CT

end-stage fibrosis


HRCT patterns suggestive of sarcoidosis

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


pulmonary function tests for sarcoidosis

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


diagnostic methods for sarcoidosis

biopsy via flexible bronchoscopy

biopsy of other sites



What is a favorable predictor of prognosis for sarcoidosis?

Lofgren's syndrome


treatment of sarcoidosis

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


alternative therapies for sarcoidosis

methotrexate - immunosuppresive agent used if steroids are ineffective

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