Case Studies

 Case 10.3 Fibrosing alveolitis in rheumatoid arthritis

A 61-year-old man, with a 15-year history of seropositive RA, was admitted with increasing shortness of breath, myalgia and weight loss. He had previously smoked 40 cigarettes a day but had never been exposed to coal or silica dust. On examination, he was pale and thin, with generalized muscle tenderness. Small bilateral pleural effusions were present with widespread crepitations over both lung fields. His joints were tender and he had subluxation of the metacarpophalangeal joints of both hands rather than another typical feature - Swan-neck deformalities. There was bilateral cervical and axillary lymph node enlargement but no splenomegaly. Neurological and cardiac examinations were normal.

Investigations showed a raised CRP (81mg/l) and a normochromic anaemia (Hb 95g/l) but a normal white-cell count. His serum IgG was raised at 44g/l (NR 7.2-19.0), although IgA and IgM levels were normal. He had a strongly positive rheumatoid factor titre of 1/1280 (NR <1/32). There were no detectable antibodies to DNA or to extractable nuclear antigens (ENA) (see Chapter 19) and the serum levels of muscle enzymes were normal. X-rays showed erosions of both wrist joints and some subluxation of the atlantoaxial joint. The pleural aspirate showed no malignant cells but occasional polymorphonuclear leucocytes and a few lymphocytes were seen; the protein content was high (25g/l) and rheumatoid factor was present in the fluid. A diagnosis of rheumatoid pleural effusions and fibrosing alveolitis was made.

This man's dyspnoea was rapidly progressive and he continued to deteriorate despite intravenous corticosteroids and cyclophosphamide. At autopsy, both lungs showed fibrosing alveolitis, which is a rare complication of RA with a poor prognosis. The onset of serious complications of RA so long after the initial diagnosis is not unusual.