Disorders of the Pleura |

Primary Sjögren's Syndrome Accompanied by Pleural Effusion FREE TO VIEW

D. Ma, PhD; Shanshan Wang, MD; Yangyang Ying, BS; Yiqing Qu, PhD
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Qilu Hospital, Shandong University, Jinan, China

Chest. 2013;144(4_MeetingAbstracts):502A. doi:10.1378/chest.1703364
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SESSION TITLE: Pleural Global Case Reports

SESSION TYPE: Global Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: The incidence of pleural effusion in Sjögren's Syndrome is less than 1%, extremely rare in primary Sjögren's Syndrome. This case concerned with a woman with bilateral pleural effusion for eight years.

CASE PRESENTATION: Eight years ago, the woman had chest tightness after had a cold. The chest CT showed bilateral pleural effusion. After being drained by thoracentesis, the discomfort disappeared. Similar situation occurred repeatedly. Chest CT showed large quantity of bilateral pleural effusion, and abnormal density lesion observed on the left lung. Brush cytology from bronchoscope found suspect malignant cells. PET-CT displayed no malignant lesions. After pleural effusion drainage, anti-infection, and intrapleural administration of IL-2, dexamethasone, symptom relieved. Ten days ago, chest tightness happened again. She also suffered from xerophthalmia and xerostomia with most of the teeth gradually lost for several year. Physical examination revealed vital signs were normal. Breath sound was coarse in bilateral upper lungs, and weak in lower lungs. Laboratory findings were as follows. Blood routine, urine routine tests, liver, renal function, SACE, PPD skin test, tuberculosis DNA test, serologic humoral immunity and serum tumor markers showed normal. Anti-tuberculosis antibody was weak positive. Results of serological test revealed anti SSA antibody (+), ANA 1:100 (+), and rheumatoid factor (RF) 61.60IU/ml. showed normal. ESR was 40 mm/h. Chest X ray and CT revealed bilateral pleural effusion. The pleural effusion was exudative without malignant cells. Anti SSA antibody was positive, and ANA was 1:100 positive, and RF was 75.30IU/ml. Multiple white nodules were found on both parietal and visceral pleura by the video-assisted thoracoscope. Histopathological examination revealed chronic inflammation of pleura, pleural thickening and infiltrating of lymphocytes. Schirmer's test showed right eye 5mm and left eye 4.5mm. Most teeth were decayed and lost gradually. Biopsy of little salivary glands in the lower lip revealed infiltration of lymphocytes among glands. It was considered that she had developed Sjogren's syndrome, so hydroxychloroquine and paeony (TGP) were given.

DISCUSSION: According to the American and European Consensus Group classification criteria (AECG-criteria), primary Sjogren's syndrome was diagnosed. First ,the patient had dry mouth and dry eye for several years. The schirmer's tests of both eyes were no more than 5mm/5min. Biopsy of little salivary glands in the lower lip showed diffused infiltration of lymphocytes among glands. Besides anti SSA antibody in both serum and pleural effusion was positive. Up to now, we have searched only 3 cases of primary Sjogren's syndrome representing pleural effusion.The precise mechanism of Sjögren's Syndrome accompanied by pleural effusion is not sure so far. It is assumed that ctytokines from CD4+ T lymphocytes may activate B lymphoctes. Then the activated B lymphocytes produce autoantibodies which are associated with pleuritis and other systemic tissue damage. Kawamata KThen, et al,considered that T cell receptor beta-chain variable region gene bias and local autoantibody production in the pleural effusion. At last they thought a common biased T cell response might play a critical role in this manifestation. We will expect further study should be carried on the diagnosis and mechanism of similar desease.

CONCLUSIONS: Primary Sjögren's Syndrome accompanied by pleural effusion is rare. Serological test, bronchoscopy, CT, and biopsy are useful in the diagnosis of Primary Sjögren's Syndrome with pleural effusion.

Reference #1: Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, Daniels TE, Fox PC, Fox RI, Kassan SS, Pillemer SR, Talal N, Weisman MH. Classification criteria for Sjogren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002: 61(6): 554-558.

Reference #2: Teshigawara K, Kakizaki S, Horiya M, Kikuchi Y, Hashida T, Tomizawa Y, Sohara N, Sato K, Takagi H, Matsuzaki S, Mori M. Primary Sjogren's syndrome complicated by bilateral pleural effusion. Respirology 2008: 13(1): 155-158.

DISCLOSURE: The following authors have nothing to disclose: D. Ma, Shanshan Wang, Yangyang Ying, Yiqing Qu

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