Renal failure and pleural effusion; a diagnostic challenge

Abstract

Sjögren’s syndrome is a chronic inflammatory disorder mostly involving the exocrine glands. Extraglandular disease may occur in up to one quarter of patients. Kidney involvement is rare, more often manifested by tubular dysfunction secondary to tubulointerstitial nephritis. Primary glomerular disease is uncommon. The authors present the case of a 73-year-old woman with xerostomia and positivity for anti-Ro and anti-La antibodies admitted for acute kidney injury and exudative pleural effusion. Biopsy of salivary glands was compatible with Sjögren’s syndrome. Extraglandular involvement was also confirmed by renal and pleural deposition of AA-amyloid. The patient was started on prednisolone followed by azathioprine with rapid improvement of lung disease. However, due to progressing renal disease and clinical deterioration, prognosis was guarded and the patient died. We describe a case of secondary amyloidosis with systemic involvement and infrequent clinical manifestations, briefly reviewing the key aspects of Sjögren’s syndrome and AA-amyloidosis.

Keywords

• sjögren’s syndrome
• aa amyloidosis
• renal amyloidosis
• pleural amyloidosis