Pulmonary histiocytosis X.

Abstract

Seventy-eight cases of histologically verified pulmonary histiocytosis X (HX) were analyzed retrospectively. The patients, although predominantly young adults, ranged from one to 69 years of age, and males outnumbered females by 4 to 1. Some of the patients were asymptomatic and were only discovered after routine chest radiography, whereas others presented with pneumothorax, dyspnea, cough, constitutional disturbance, or symptoms attributable to extrapulmonary HX lesions. Physical signs of lung disease were often lacking, but widespread reticular and micronodular radiographic opacities were present, most apparent in the midzones and bases but often sparing the costophrenic angles. Restrictive defects and low diffusion capacity were often found, and changes consistent with airway obstruction were also recorded. In nearly one half of the patients the disease stabilized or improved. The remaining cases deteriorated with the appearance of radiographic “honeycombing” or bullous change, and one half of these pa...