Coexistence of Sarcoidosis and Sjögren's Syndrome: A Case-Report

Abstract

SESSION TITLE: Diffuse Lung Disease Global Case Reports SESSION TYPE: Global Case Report Poster PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM INTRODUCTION: Sarcoidosis and Sjögren's syndrome (SS) are both multisystemic diseases of unknown etiology. Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. Although, lungs and the tracheobronchial tree are the most common involvement sites in sarcoidosis it can rarely effect the salivary and lacrimal glands. On the other hand, SS mainly affects the exocrine glands, but it may also have systemic manifestations. Occasionally some features of sarcoidosis may mimic SS when parotid and/or lacrimal gland involvement is present. Although they are different clinical entities there are several reports suggestive of sarcoidosis and coexisting Sjögren's syndrome in the literature (1, 2). CASE PRESENTATION: A 58 years-old Caucasian female presented with dry cough, exertional dyspnea, progressive dry mouth and dry eyes. Physical examination was unremarkable except dryness of oral mucosa and bilateral basal crackles. Laboratory examinations revealed an increased erythrocyte sedimentation rate, C-reactive protein. Routine hematological laboratory and blood chemistry values were normal. Chest radiograph showed bilateral hilar lymphadenopathy and lower zone reticular infiltrates. Chest computed tomography revealed multiple mediastinal and bilateral hilar lymphadenopathies in addition to bilateral pulmonary nodules with peribronchovascular involvement. A mild restrictive ventilatoty defect and mild reduction in carbon monoxide diffusion capacity was observed on pulmonary function tests. She underwent bronchoscopy. A transbronchial fine needle aspiration biopsy from the right lower paratracheal station revealed non-caseating granulomatous inflammation. Serum angiotensin converting enzyme level was elevated. Antinuclear antibody and antibodies against SS-A / Ro were positive. Bilateral Shirmer's tear test yielded positive. Histopathological examination of minor salivary glands showed periductal lymphocytic infiltration. The patient was diagnosed with coexisting SS and sarcoidosis. Treatment with prednisolon and hidroxychloroquine were started. During a 2 year follow-up clinical symptoms and laboratory findings normalized. Radiographic findings regressed. Corticosteroids were tapered and stopped. Until now, the patient has been treated with hidroxychloroquine with no major symptoms and findings. DISCUSSION: Our patient was considered as sarcoidosis on the basis of compatible clinical, radiological findings and histopathological verification of noncaseating granulomas in mediastinal lymph node biopsy. Elevated serum angiotensin converting enzyme also supported sarcoidosis diagnosis. On the other hand, diagnosis of SS was based on dry eye and dry mouth sympthoms, positive bilateral Schirmer's test, the presence of characteristic autoantibody (anti-Ro/SS-A) in addition to evident lymphocytic sialoadenitis in salivary gland biopsy (3). Sarcoidosis and SS share similar immunologic and histopathological features. Making a differential diagnosis between these two diseases may be difficult in cases that present with common clinical features. On the other hand, it was shown that the frequency of sarcoidosis is higher in patients with SS than in the general population (2). Immunologic profile with a high ANA, RF and anti-Ro/SS-A was shown to have an important role in differentiating between sarcoidosis mimicry or coexistence with SS in addition to biopsy. CONCLUSIONS: We emphasize that both sarcoidosis and SS may coexist as illustrated in our case. Analysis of autoantibodies may be very helpful in making differential diagnosis or deciding their coexistence. Currently, the acceptance of sarcoidosis as an exclusion criteria for SS diagnosis may lead to under-diagnosis of SS in these cases. A revision for exclusion criteria of SS diagnosis might be necessary with this respect. Reference #1: Mansour MJ, et. al. Coexistence of Sjogren's syndrome and sarcoidosis: a report of five cases. J Oral Pathol Med 2007:36:337-341. Reference #2: Ramos-Casals M, et. al. Sarcoidosis or Sjogren Syndrome? Clues to defining mimicry or coexistence in 59 cases. Medicine 2004: 83: 85-95. Reference #3: Vitali C, et. al. Classification criteria for Sjogren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002;61:554-558. DISCLOSURE: The following authors have nothing to disclose: Asli Gorek Dilektasli, Ezgi Demirdogen Cetinoglu, Eda Durmus, Esra Uzaslan, Ercument Ege, Mehmet Karadag No Product/Research Disclosure Information