Pulmonary Sarcoidosis Concurrent with Lupus Pernio and Peri-aortitis

[摘要] Background: In the literature, the concurrence of pulmonary sarcoidosis in association with lupus pernio and peri-aortitis is extremely rare and has poor prognosis. The time lag between the diagnosis of sarcoidosis and aortitis varies from 6 months to 20 years. However, aortitis was diagnosed before sarcoidosis in a few cases. Case Report: We present a 49-year-male with stage 4 pulmonary sarcoidosis and lupus pernio who presented with frequent exacerbation of asthma. Recent pulmonary function test revealed very severe mixed obstruction and restriction with predicted FEV1 21% and DLCO 52%. Chest X-ray showed diffuse bilateral lung fibrosis (stage 4 pulmonary sarcoidosis). CT scan (chest) showed widespread ground-glass attenuation with reticulo-nodular areas in both lungs and dilatation of thoracic aorta. Magnetic resonance angiography (MRA) revealed aortitis. Conclusion: Despite rarity, lupus pernio (LP) can be preceding sign of intra-thoracic involvement of sarcoidosis. In a few, peri-aortitis can be succeeding complication or concurrence of sarcoidosis. Corticosteroid or immunosuppressant is mainstay of treatment.

[学科分类] 生理学

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