Cryptogenic fibrosing alveolitis in children

Cryptogenic fibrosing alveolitis (CFA) in children is supposedly a rare diffuse lung disease characterized clinically by tachypnoea, cough, poor weight gain, and cyanosis. Histologically, it is characterized by thickening of alveolar walls and infiltration of the alveolar septae with mononuclear cells and fibrous tissue. The condition is more acute in children, with fulminant course. Ten children were diagnosed as having CFA out of three hundred children seen over a period of 3 years. Diagnosis was established by characteristic clinical findings and laboratory evaluation. All the children had tachypnoea at rest and bilateral crepitations in the absence of fever. Diffuse bilateral persistent irregular shadows were noted on the chest X-ray of these children. Pulmonary function studies revealed a restrictive type of abnormality with low transfer factor for carbon monoxide in six children. Open lung biopsy was done in eight children. The biopsy material was graded according to nine morphologic variables. Administration of steroids early in the course of illness resulted in amelioration of symptoms in 60% of the patients.