PULMONARY DISORDERS ASSOCIATED WITH SJOGRENS SYNDROME

Abstract

Pulmonary disease occurs as a complication of Sjogren''s syndrome. Seventeen patients, the majority being middle-aged women, with this association were studied. Eight had sicca syndrome alone and 9 had Sjogren''s syndrome in association with a connective tissue disease. Various pulmonary disorders were found: 10 had diseases mainly affecting pulmonary parenchyma (recurrent pneumonia, granulomatous infiltration, pseudolymphoma and fibrosing alveolitis) and 6 had disorders of the airways (bronchitis, bronchiectasis and asthma). One patient presented with a mediastinal immunoblastic lymphoma. To determine if physical factors, such as the retention of viscid mucus, may participate in the development of lung disease in Sjogren''s syndrome, mucociliary clearance was measured in patients with Sjogren''s syndrome using 99Tc-labeled polystyrene particles. Mucociliary clearance rate was normal from central airways, and whole lung clearance rate was significantly increased in Sjogren''s syndrome compared with normal controls. The latter was attributed to the failure of penetrance of the radioaerosol into the periphery of the lung consistent with obstruction of small airways in Sjogren''s syndrome. Patients with Sjogren''s syndrome and lung disease were characterized by immunological markers irrespective of the presence or type of associated connective tissue disorder. The commonest found were speckled antinuclear antibody by indirect immunofluorescence, antibodies to the acidic nuclear antigen B by counterimmunoelectrophoresis, increased serum DNA binding, increased levels of circulating Ig, rheumatoid factor in high titer and circulating soluble immune complexes as detected by C1q [complement component 1q] binding assay.