Pulmonary alveolar proteinosis in two siblings with decreased immunoglobulin A

A brother and sister with classic, biopsy proved pulmonary alveolar proteinosis are described. Both had low serum and low normal secretory immunoglobulin A (IgA) levels. A tendency for familial occurrence is possible and it is recommended that patients with pulmonary alveolar proteinosis, and their families, be evaluated for immunologic deficiencies.