Behcet's syndrome (BS) is a vasculitis, seen more commonly around the Mediterranean and the Far East, and manifests with oral and genital ulcerations, skin lesions, uveitis, and vascular, central nervous system and gastrointestinal involvement. Its natural history of getting less severe over time, more severe disease in males and lack of specific diagnostic testing separates it from other commonly seen conditions in rheumatology. Most of the serious manifestations respond well to immunosuppression, and these are the mainstays of treatment for BS. BS is more prevalent in regions along the Silk Road, from the Mediterranean to the Far East. The genetic risk factor most strongly associated with BS is the human leukocyte antigen (HLA)-B51 allele. While genetic factors seem to play a role in the development of certain features of BS, there is general consensus that as yet unidentified environmental stimuli are necessary for initiation of disease. Proposed exogenous triggers include both bacterial and viral infections, which may then lead to dysregulation of the immune system, ultimately leading to the phenotypic expression of disease. The clinical manifestations of BS are protean in nature. While most patients develop mucocutaneous and genital ulcers along with eye disease, other patients may also present with arthritis, frank vasculitis, thrombophlebitis and CNS disease. Interestingly, the manifestations of this illness vary considerably based on gender and ethnicity. As the phenotypic expression among patients with BS is quite heterogeneous, pharmacological therapy is variable and dependent upon the severity of the disease as well as organ involvement. Treatment for BS overlaps considerably with therapies for other autoimmune diseases, including systemic lupus erythematosus, rheumatoid arthritis and the vasculitides. Pharmacological agents utilized for treatment of BS include corticosteroids, colchicine, azathioprine, and tumour necrosis factor (TNF).α inhibitors, among others. In this article, we review the salient clinical studies for each drug class along with important side effects as well as drug toxicity monitoring. Management of the patient with BS is complex and oftentimes requires a multidisciplinary approach. We discuss strategies to assess and stratify patients based on clinical manifestations and disease severity. A summary of drug toxicities as they relate to the aforementioned pharmacological agents, as well as guidelines regarding vaccinations in this patient population, are offered. Finally, we conclude with treatment strategies for the common manifestations of BS along with a discussion of the management of thrombotic disease in these patients.

Pseudo-pseudo Meigs' syndrome (PPMS) is a rare manifestation of patients with systemic lupus erythematosus (SLE), defined by the presence of ascites, pleural effusions and an elevated CA-125 level. We describe a patient with longstanding lupus who presented with localized lymphadenopathy and subsequently developed massive chylous ascites with marked hypoalbuminemia. A brief historical overview of Meigs' syndrome and related entities is presented, along with a discussion of the differential diagnosis of hypoalbuminemia and ascites in an SLE patient. In addition, we speculate on the optimal therapeutic intervention in such a patient.

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Case presentationA 27-year-old male presented with acute-onset dyspnea, pain, and diffuse swelling in both lower extremities. Past medical history was noncontributory except for recurrent oral ulcers. Physical examination revealed tachycardia, papulopustular lesions on the upper back of the body, and positive Homans sign in the lower extremities bilaterally. Lung examination was clear, but mild sinus tachycardia was present on electrocardiogram (ECG). Laboratory investigations showed high acute phase reactants and elevated D-dimer levels. Doppler imaging of the lower extremities exhibited bilateral widespread deep vein thrombosis throughout the femoral veins. No evidence of pulmonary embolism, vasculitis, and pulmonary aneurysm was found by computed tomography (CT) pulmonary angiography. However, a hypoechoic lesion consistent with thrombus formation was detected incidentally in the right ventricle of the heart (Fig. 1). Intracardiac thrombus formation was confirmed by transesophageal echocardiogram (TEE) (Fig. 2). Results of pathergy test and HLAB51 gene mutation were unrevealing. Lupus anticoagulant, anticardiolipin, and anti-β2 glycoprotein I antibodies were negative. Tests for factor V Leiden mutation, homocysteine, antithrombin III, prothrombin G20210A mutation, and proteins C and S were

BackgroundPulmonary involvement (PI) other than pulmonary artery aneurysm (PAA) in Behcet’s syndrome (BS) is still an area of investigation.ObjectivesHerein, we aimed to retrospectively evaluate all types of pulmonary involvement associated with Behcet’s syndrome (BS).MethodsAmong 800 BS patients according to International Study Group for Behcet’s Disease (ISG) criterias, 28 patients were selected based on their radiologic examination consistent with BS-related PI. Demographic features, other clinical manifestations of BS, treatment modalities and types of PI were analyzed.ResultsThe overall prevalence of PI was estimated 3,5% among all BS patients. PI was more common in males (82.1% vs 17.9%). Mean age for BS diagnosis and onset of PI were as following; 32 ± 10,9 and 37 ± 11,4 years. Deep vein thrombosis (DVT) was the most common accompanying vascular involvement (53,6%). PAA, pulmonary vasculitis (PV), and pulmonary thromboembolism (PTE) were seen in 7 (25%), 13 (46,3%), and 18 (64,4%) of patients, respectively. In 5 patients, intracardiac thrombosis was present simultaneously in the right ventricle. Cyclophosphamide (CYC) was the most common preferred agent (78%) followed by azathioprine (AZA) as first line. Warfarin used in 18 patients (Table 1) Mortality was seen in 3 patients during follow up (1 due to PAA bleeding, others with unknown causes).Table 1.Characteristics of pulmonary involvement in BS patientsMale/Female, (n), (%)23/5 (82,1%/17,9%)Age of diagnosis, years (Mean± SD)32 ± 10,9Age of pulmonary involvement, years (Mean ± SD)37 ± 11,4Oral ulcer, (n), (%)28 (100%)Genital ulcer, (n), (%)20 (71,4%)Osteofollicular lesion,(n), (%)20 (71,4%)Erythema nodosum, (n), (%)13 (46,4%)Uveitis, (n), (%)8 (28,6%)DVT, (n), (%)15 (53,6%)Cardiac involvement, (n), (%)5 (17,8%)Pulmonary involvement, (n)-PAA, (n), (%)287 (25%)-PV, (n), (%)13 (46,4%)-PTE, (n), (%)18 (64,3%)Immunosuppression (first line agents)26/28-Steroid, (n), (%)26/28-CYC, (n), (%)22/26 (84,6%)-AZA, (n), (%)4/26 (15%)Anticoagulation (warfarin), (n), (%)18PAA; pulmonary artery aneurysm, PTE; pulmonary thromboembolism, PV; pulmonary vasculitis, CYC; cyclophosphamide, AZA; azathioprine, DVT; deep vein thrombosisConclusionDespite the importance of PAA in BS patients, capillaritis and thromboembolism other than PAA may occur commonly in BS as well. Onset at young age, male gender and previous DVT seem to be significant risk factors for the development of PI.References[1]Yazgan Ç, Ertürk H, Taşkin A. Imaging Features of Thoracic Manifestations of Behçet’s Disease: Beyond Pulmonary Artery Involvement. Curr Med Imaging. 2021;17(8):996-1002.Disclosure of InterestsNone declared