Pleurisy, coughing and/or dyspnea are often the first clues of lung involvement in SLE.
We present a 42 year old woman with a history of pelvic vein thrombosis and left sided pneumonia in the preceeding two years who was referred to our department for evaluation of progressive exertional dyspnea, unproductive cough, fatigue and migratory pleuritic chest pain. She also described having recurrent episodes of tender and swollen metacarpophalangeal and proximal joints. She was a smoker with a history of 50 packyears.The physical examination was unremarkable. Chest x-ray and high resolution computer tomography (HRCT) showed normal lung structure. Pulmonary function testing revealed a restrictive pattern (total lung capacity 75% pred), a significant reduction of the diffusing capacity for carbon monoxide (43% pred) and exertional hypoxemia with normal oxygen partial pressure at rest. Bronchoscopy was performed. Bronchioalveolar lavage showed a normal cell distribution and transbronchial biopsy showed changes compatible with chronic bronchitis, discrete alveolar septal thickening and interstitial fibrosis. Immunologic testing was positive for antinuclear antibodies (1:640) including those to double stranded DNA, suggesting the diagnosis of SLE. For further confirmation an open lung biopsy was performed. The histopathologic findings were similar to those of the transbronchial biopsy. Therapy with 75mg prednisone and 100mg azathioprine was started and tapered to 25mg prednisone and 50mg azathioprine after 12 weeks. Under therapy the patient reported a marked improvement of her exertional dyspnea and coughing. Lung function testing after 12 weeks showed normal lung volumes, the exertional hypoxemia has resolved.
Our patient presented with a significant pulmonary functional impairment. HRCT is considered a sensitive technique for detecting pulmonary changes in SLE but was negative in the present case. The invasive diagnostic procedures did little to confirm our diagnosis as the histopathological findings were only discrete. Her history of fatigue, arthralgias and pleurisy has led us to suspect a systemic disease, but the major diagnostic clue were the immunologic abnormalities. In applying the criteria of the American Rheumatism Association the patient could be classified having SLE.
In patients with SLE and lung involvement with significant impairment of their lung function, correlating changes in HRCT may be absent.
D. Kiss, None.