Abstract
A 26 year-old woman was admitted to our hospital in April, 1985 because of cough and dyspnea on exertion. She developed polyarthritis and Raynaud's phenomenon at age 17. One year later, a diagnosis of systemic lupus erythematosus was made on the basis of a malar rash, maculopapular eruption, photosensitivity, positive LE cell preparations and a high titer of anti-nuclear antibodies. Since then she had been treated with prednisone. Her chest X-ray film showed diffuse fine reticulonodular shadows in both lung fields. Her pulmonary function studies revealed only mild restrictive ventilatory disturbance and impaired diffusing capacity.
TBLB showed deposits of amorphous, eosinophilic substance and lesions suggestive of pulmonary fibrosis. The substance was confirmed to be amyloid by observing Congored stained sections under polarized light, which showed green birefringence. In contrast, potassium permanganate-treated specimens stained with Congo red lost its red staining characteristics and the green birefringence. Immunofluorescence microscopy showed positive staining for AA protein but negative staining for immunoglobulins and complements. On electron microcopy characteristic non-branching fibrils were demonstrated. These findings indicate that the fibrils consisted of AA protein and that the lung was not involved by SLE.
Review of the literature disclosed only nine previous case reports on concurrence of amyloidosis and SLE. Their common clinical features were proteinuria and nephrotic syndrome and renal amyloidosis was demonstrated in eight cases. Our patient developed cough and exertional dyspnea without proteinuria or nephrotic syndrome. Renal biopsy was performed eight years ago and revealed no evidence of SLE and amyloidosis. Since then proteinuria and nephrotic syndrome have not been noted. Therefore, this is the first time that pulmonary amyloidosis has been described in a patient with SLE.
