Selective IgA deficiency and chronic obstructive pulmonary disease--a family study.

Abstract

A 43 year old housewife with far advanced chronic obstructive pulmonary disease (COPD) was referred for evaluation of alpha1 antitrypsin (AAT) type because of the early onset of her disease and her family history. Gradually progressive dyspnea on exertion and productive cough had been present for six years. There had been frequent upper respiratory infections and a mastoidectomy in the past. She had been a 2 pack per day cigaret smoker for years. On physical exam she was a cachectic woman in moderate respiratory distress. Breath sounds were almost absent at the lung bases and scattered wheezes and rhonchi were present. The chest film showed hyperexpansion and a suggestion of cyst formation at the bases. Pulmonary function testing showed severe obstruction which did not respond to broncho-dilators. AAT quantitation and phenotyping by starch gel electrophoresis and subsequent crossed electrophoresis were normal. Quantitative immunoglobulin determinations revealed less than 4 mg./100 cc. of IgA in serum (adult normals 50–250) and slightly elevated IgG and IgM. IgE level was 44 ng./cc. (Normal—less than 500) Concentrated parotid secretions also showed no IgA.