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Idiopathic multicentric Castleman disease was suspected. The lung and mediastinum lymph node biopsies were performed, and pathological findings of the lymph nodes were compatible with multicentric Castleman disease. Pathological findings of the lung showed that the fibrous thickening of interstitium and the collapse of alveoli. We diagnosed this case as idiopathic multicentric Castleman disease preceded by diffuse interstitial lung disease. Treatment with prednisolone improved the dyspnea, and the pulmonary lesions disappeared. The pres