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Johns Hopkins CPC4

Biopsy never diagnostic. Always revisit diagnosis or consider additional pathologic processes when either clinical course or clinical manifestations deviate from the expected or the typical.Consider:Not sarcoidosis Sarcoidosis plus independent process-e.g. infection, PE, CHFSarcoidosis plus associated process- e.g. CVID, CTD, othersTreatment effectsRare manifestations of sarcoidosis do occur.

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Johns Hopkins CPC4

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    1. Johns Hopkins CPC#4 David R. Moller, M.D. Johns Hopkins University Baltimore, USA

    14. Sarcoidosis Associated Pulmonary Hypertension Pulmonary hypertension prevalence 6% unselected pulm sarcoidosis patients 50% patients with dyspnea disproportionate to PFTs 70-80% in advanced lung disease Higher when measuring exercise induced pulm htn Multiple potential mechanisms Bronchovascular distribution of inflammation Advanced fibrocystic lung disease (loss of pulmonary capillary bed) Extrinsic compression of pulmonary arteries by LN, mediastinal fibrosis Cardiac sarcoidosis with systolic, diastolic dysfunction Hypoxic vasoconstriction Primary pulmonary vascular involvement (granulomatous arteritis) Veno-occlusive disease (rare)

    16. Cardiac Sarcoidosis: Clinical Manifestations Common Arrhythmias Heart block/conduction defects Congestive heart failure Sudden death

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