Primary Sjögren Syndrome With Pleural Effusion (original) (raw)

2017, Archivos De Bronconeumologia

Síndrome de Sjögren primario con derrame pleural To the Editor, Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disease characterized by lymphocytic infiltration of the exocrine glands, particularly the lacrimal and salivary glands, causing the characteristic symptoms of xerophthalmia and xerostomia. The lung, thyroid, kidney and the hepatobiliary tract can also be affected. This disease can be present in an isolated form (primary SS), or it can be associated with other connective tissue diseases (secondary SS). Pleural effusion (PE) in primary SS is rarely described in the literature. 1 We report the case of a 40-year-old woman with insulindependent diabetes mellitus, consulting due to xerostomia and xerophthalmia for some months, and intermittent diffuse arthromyalgia. Vital signs were normal and the only notable finding on physical examination was limited mobility of the right shoulder. Routine clinical laboratory tests (including angiotensin-converting enzyme levels) and urine tests were normal. Of particular interest were rheumatoid factor 103 IU/ml (upper level of normal 14 IU/ml), positive antinuclear antibodies (ANA) (1/640), and positive anti-Ro/SS-A and anti-La/SS-B antibodies and immunoglobulin G 4050 mg/dl. The Schirmer test was positive in both eyes, a minor salivary gland biopsy showed Chisholm grade 2 lymphocytic infiltration, and scintigraphy of the salivary glands was consistent with SS. A diagnosis of primary SS was established on the basis of these results. Four years later, the patient developed right pleuritic pain, without dyspnea, cough or expectoration, but with low-grade fever in the evenings. She reported no previous chest injury and no contact with tuberculosis patients. Physical examination was unremarkable, except for signs of right PE. The chest computed tomography revealed thick-walled cystic and cavitary lesions in the right lung and a small ipsilateral PE. Fiberoptic bronchoscopy was performed that was macroscopically normal, and smear tests and PCR for Mycobacterium tuberculosis in bronchoalveolar lavage were negative. PE was an exudate (total pleural fluid protein/serum protein ratio 0.7; lactate dehydrogenase 597 IU/l and pleural fluid LDH/serum LDH 1.8), leukocytes 5830/l (neutrophils 14%, lymphocytes 24%, eosinophils 16%, macrophages 46%), with normal pH, glucose, amylase, adenosine deaminase, tumor markers and N-terminal pro-brain natriuretic peptide levels, rheumatoid factor 58.6 IU/ml, positive ANA (1/320), and positive anti-Ro/SS-A