This patient had cirrhosis and severe lung dysfunction in Langerhans-cell histiocytosis, a disease commonly associated with intrinsic lung disease and pulmonary failure. Our patient did have mild residual lung changes attributable to pulmonary Langerhans-cell histiocytosis. However, his evaluation and the course of his condition have shown that most of his lung dysfunction was caused by the hepatopulmonary syndrome. Early histologic findings are characterized by interstitial granulomas with Langerhans-cells. The prevalence of lung dysfunction among those with cirrhosis was not reported.
Patients characteristically have cyanosis, clubbing, and stigmata of cirrhosis.
A prominent reticular pattern may be seen on chest radiographs. Pulmonary failure in Langerhans-cell histiocytosis is usually attributed to advanced lung involvement. Our patient actually had the hepatopulmonary syndrome. The potential reversibility of the latter makes necessary the careful distinction between the syndrome and irreversible pulmonary Langerhans-cell histiocytosis in similar patients. The authors thank Ms. Chris Spillman for assistance in manuscript preparation and Dr. David Stone for his review of this manuscript.