| Sub-title | |
| Authors | P J Cassar K Cassar F Camilleri J S DeGaetano |
| Abstract | Sjgrens syndrome (SS) is a relatively common, slowly progressive, systemic autoimmune disorder that characteristically affects the exocrine glands and causes dryness of the eyes and mouth. It is a multi-system disorder that may affect the lungs, liver, kidneys, vasculature and blood. The histological hallmark of all organs affected is a potentially progressive lymphocytic infiltration. Primary SS occurs alone while secondary SS occurs in association with other systemic autoimmune rheumatic diseases. Pulmonary involvement is common in SS but is rarely clinically important. Any part of the respiratory tract may be affected by lymphocytic infiltration, although the most commonly encountered pathology is interstitial lung disease and airways disease. We report the case of a 44 year old lady with a ten year history of primary Sjogrens who presented with recurrent parotid swelling, sicca symptoms, symmetrical joint pains, early morning stiffness, Raynauds phenomenon, pleuritic chest pain, dyspnoea, weight loss and fever. Her clinical condition deteriorated despite treatment with corticosteroids. Bronchiolitis obliterans was diagnosed on lung biopsy. She responded well to treatment with 5 pulses of cyclophosphamide and methylprednisolone. This was subsequently changed to azathioprine and she has remained in remission. The literature of pulmonary involvement in SS is reviewed. |
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| Journal | Malta Medical Journal |
| Volume | 15 Issue 1-2/suppl. 2003 |
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| Key words | case report, sjogrens syndrome, broncholitis obliterans |