Relapsing poly(peri)chondritis associated with fibrosing alveolar disease and antibodies to pneumocytes type II and clara cells

Abstract

A 62-year-old man with histological confirmed relapsing polychondritis showed chondritis of ears and nose, arthritis, keratitis and a hemolytic anemia. The bronchoalveolar lavage, computed tomography of the thorax and pulmonary function tests disclosed findings compatible with fibrosing alveolar disease. IgG antibodies to alveolar pneumocytes type II and bronchiolar Clara cells were detected by indirect immunofluorescence of human lung tissue. To our knowledge this is the first report of fibrosing alveolar disease in relapsing polychondritis and detection of antibodies to human pneumocytes type II and Clara cells.

Rare and Unusual Connective Tissue Diseases in the Lung

“…30 When pulmonary infiltrates are present, other possibilities include a pulmonary vasculitis with fleeting infiltrates 31 and diffuse pulmonary fibrosis. 32 Bronchomalacia has been treated with nasal continuous positive airway pressure (CPAP). 33 The use of the Nd YAG laser with endobronchial silicone stent has been described for airway stenosis in RP.…”