Pulmonary manifestations of hypogammaglobulinaemia

Fifty-five patients with late-onset idiopathic immunoglobulin deficiency were studied and upper or lower respiratory tract infections were encountered in about 90%. Cylindrical bronchiectasis was shown in all of the 21 patients in whom bronchograms were done. A thymoma was found in four patients. Three patients had diffuse interstitial pulmonary disease--two with proved and one with presumed lymphocytic interstitial pneumonitis. Five patients had no evidence of pulmonary disease, including two patients with long-standing late-onset immunoglobulin deficiency who had essentially no serum immunoglobulins. This small subgroup of patients with immunoglobulin dificiency without severe pulmonary infections cannot be explained in the context of current understanding of immunoglobulin deficiency. Thirty-two patients were followed up for long enough for the response to treatment to be assessed.