Abstract
Amyloidosis is a rare cause of pulmonary infiltrates in primary Sjögren's syndrome(SS), affecting 0–2% of symptomatic patients. Diffuse multi nodular amyloidosis(DMNPA) is the most common radiological pattern, occurring alone or in association with cysts of varying size. Amyloid can be easily overlooked or mistaken for other entities with similar staining qualities. We describe the case of a 61-year-old woman with DMNPA (AL (λ)) related to SS, in the presence of primary systemic amyloidosis(PSA). She had suffering from cough, hemoptysis and dyspnoea of two -month duration. She had been diagnosed with SS 10 years previously. She had macroglossia and multiple skin nodules. Chest excursions were reduced with crackles in both lungs.Her chest radiograph and CT showed multiple nodules and cysts in both lung fields. Calcification in the nodules and lymphadenopathy were apparent in the mediastinal windows. Spirometry indicated moderate restriction, and BAL fluid was negative for acid-fast bacilli (AFB), fungi. Bronchoscopy with transbronchial lung biopsy revealed hyaline eosinophilic material associated with a giant cell granulomatous reaction, a patchy lymphocytic infiltrate. The congo red stain was positive in this hyaline material and showed apple green birefringence on polarising microscopy. Electrophoresis of serum proteins revealed hypergammaglobulinaemia. Serum immunofixation showed a monoclonal IgGλ. In the bone marrow (BM) plasma cells were increased to 10-15%. PSA (AL) associated with SS was diagnosed. Amyloid deposits were found not only in the lung but also in skin and BM biopsy specimens. Staining for amyloidosis should be performed in patient with SS and pulmonary infiltration.
- © 2012 ERS
