EULAR/PReS standards and recommendations for the transitional care of young people with juvenile-onset rheumatic diseases.

To develop standards and recommendations for transitional care for young people (YP) with juvenile-onset rheumatic and musculoskeletal diseases (jRMD). The consensus process involved the following: (1) establishing an international expert panel to include patients and representatives from multidisciplinary teams in adult and paediatric rheumatology; (2) a systematic review of published models of transitional care in jRMDs, potential standards and recommendations, strategies for implementation and tools to evaluate services and outcomes; (3) setting the framework, developing the process map and generating a first draft of standards and recommendations; (4) further iteration of recommendations; (5) establishing consensus recommendations with Delphi methodology and (6) establishing standards and quality indicators. The final consensus derived 12 specific recommendations for YP with jRMD focused on transitional care. These included: high-quality, multidisciplinary care starting in early adolescence; the integral role of a transition co-ordinator; transition policies and protocols; efficient communications; transfer documentation; an open electronic-based platform to access resources; appropriate training for paediatric and adult healthcare teams; secure funding to continue treatments and services into adult rheumatology and the need for increased evidence to inform best practice. These consensus-based recommendations inform strategies to reach optimal outcomes in transitional care for YP with jRMD based on available evidence and expert opinion. They need to be implemented in the context of individual countries, healthcare systems and regulatory frameworks.

Remarkable damage along with poor quality of life in Takayasu arteritis: cross-sectional results of a long-term followed-up multicentre cohort.

OBJECTIVES: We aimed to assess the outcome of a large Takayasu arteritis (TAK) cohort using the vasculitis damage index (VDI) and quality of life (QoL) scale, tools which have been validated for vasculitis. METHODS: Disease activity, damage and QoL were cross-sectionally evaluated in 165 TAK patients from 6 centres. SF-36 were applied to 51 age-matched healthy controls (HC). Persistent activity for ≥6 months was considered as treatment resistance (r-TAK). The correlation between VDI, clinical characteristics and mental (MCS)/physical (PCS) component scores of SF-36 were analysed. SF-36 and VDI scores were compared between TAK subgroups and HC. RESULTS: The median age, follow-up time and disease duration were 40 (17-68), 60 (6-384), and 72 (6-396) months, respectively. 35% of them were r-TAK. VDI scores (VDIs) in TAK 4 (1-12) were mainly due to the disease itself [4 (1-10)]. VDIs in r-TAK were significantly higher than nr-TAK [5 (2-12) vs. 3 (2-10), p<0.001)]. In the TAK patients, MCS and PCS were found as 43±10 and 38±11, respectively. A high proportion of poor MCS (70%) and PCS (80%) were demonstrated in TAK. A significantly negative but weak correlation was observed between VDI and MCS (p=0.003, r=-0.23), PCS (p<0.001, r=-0.34). Higher VDIs were detected in patients with PCS <50 [5 (1-12) vs. 2 (1-6) p<0.001)]. SF-36 score was significantly lower in TAK than HC. CONCLUSIONS: Disease-related damage mainly caused by peripheral vascular involvement was more predominant than treatment-related damage without reaching the level of severe damage scores, but contributing to poor QoL, in the TAK cohort.

A single endoplasmic reticulum aminopeptidase-1 protein allotype is a strong risk factor for Behçet's disease in HLA-B*51 carriers.

INTRODUCTION: Endoplasmic reticulum aminopeptidase-1 (ERAP1) protein is highly polymorphic with numerous missense amino acid variants. We sought to determine the naturally occurring ERAP1 protein allotypes and their contribution to Behçet's disease. METHODS: Genotypes of all reported missense ERAP1 gene variants with 1000 Genomes Project EUR superpopulation frequency >1% were determined in 1900 Behçet's disease cases and 1779 controls from Turkey. ERAP1 protein allotypes and their contributions to Behçet's disease risk were determined by haplotype identification and disease association analyses. RESULTS: One ERAP1 protein allotype with five non-ancestral amino acids was recessively associated with disease (p=3.13×10-6, OR 2.55, 95% CI 1.70 to 3.82). The ERAP1 association was absent in individuals who lacked HLA-B*51. Individuals who carry HLA-B*51 and who are also homozygous for the haplotype had an increased disease odds compared with those with neither risk factor (p=4.80×10-20, OR 10.96, 95% CI 5.91 to 20.32). DISCUSSION: The Behçet's disease-associated ERAP1 protein allotype was previously shown to have poor peptide trimming activity. Combined with its requirement for HLA-B*51, these data suggest that a hypoactive ERAP1 allotype contributes to Behçet's disease risk by altering the peptides available for binding to HLA-B*51.

EULAR recommendations for the management of familial Mediterranean fever.

Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease, but many rheumatologists are not well acquainted with its management. The objective of this report is to produce evidence-based recommendations to guide rheumatologists and other health professionals in the treatment and follow-up of patients with FMF. A multidisciplinary panel, including rheumatologists, internists, paediatricians, a nurse, a methodologist and a patient representative, was assembled. Panellists came from the Eastern Mediterranean area, Europe and North America. A preliminary systematic literature search on the pharmacological treatment of FMF was performed following which the expert group convened to define aims, scope and users of the guidelines and established the need for additional reviews on controversial topics. In a second meeting, recommendations were discussed and refined in light of available evidence. Finally, agreement with the recommendations was obtained from a larger group of experts through a Delphi survey. The level of evidence (LoE) and grade of recommendation (GR) were then incorporated. The final document comprises 18 recommendations, each presented with its degree of agreement (0-10), LoE, GR and rationale. The degree of agreement was greater than 7/10 in all instances. The more controversial statements were those related to follow-up and dose change, for which supporting evidence is limited. A set of widely accepted recommendations for the treatment and monitoring of FMF is presented, supported by the best available evidence and expert opinion. It is believed that these recommendations will be useful in guiding physicians in the care of patients with FMF.

What is the best acute phase reactant for familial Mediterranean fever follow-up and its role in the prediction of complications? A systematic review.

The most dreaded complication of familial Mediterranean fever (FMF) is amyloidosis; controversy exists as to what acute phase reactant (APR) should be monitored in these patients. To analyze the best acute phase reactant for FMF follow-up to help guide physicians to decide on what APR parameter to use, we also attempted to define the best APR in predicting the complications of FMF, specifically the development of amyloidosis. Systematic review based on a sensitive search to capture studies that: (1) included FMF patients; (2) measured serum amyloid A (SAA), CRP (C-reactive protein), proteinuria, or ESR (erythrocyte sedimentation rate); (3) amyloidosis were the outcome measure; (4) sensitivity, specificity, predictive value, and other performance parameters could be calculated; and (5) had a longitudinal design. Of 1905 captured items, 26 were selected for detailed review, of which only two finally met the criteria, and the quality was only moderate; the articles did not analyzed the performance by means of sensitivity and specificity to predict, or even detect, amyloidosis, and thus had to be calculated based on text. The 26 screened studies were very heterogeneous in designs, parameters measured, and results, despite being set from research questions similar to ours. They were mainly descriptive, and it was very difficult to interpret the true performance of the tests. The correlation between the various APR is low. The evidence supporting the monitoring of FMF with any APR over the others is limited. Well designed longitudinal studies with a mixture of outcomes should be undertaken. Until them, recommending an APR over other would be based on expert opinion and indirect evidence.

Efficacy and safety of treatments in Familial Mediterranean fever: a systematic review.

Familial Mediterranean fever (FMF) is an autoinflammatory disease, which can be well controlled with lifelong use of colchicine. Since studies dealing with the efficacy and safety of colchicine were conducted mainly in the sixties and seventies of the previous century, it seems that this topic needs to be updated. Recently, an international expert panel was undertaken for the establishment of recommendations on how to manage FMF. We aimed to summarize the efficacy and safety of the current treatments available to prevent FMF attacks and to avert the appearance of amyloidosis secondary to FMF. A systematic review was performed. Two reviewers and methodologist established the protocol of the review and the epidemiological questions in PICO terms. MEDLINE through PubMed, Embase, and Cochrane Central Trials Register all up to May 31, 2014, were searched, and only randomized controlled trials or quasicontrolled trials were accepted. For each study, a judgment on risk of bias was then rated as high, moderate, or low. Of 1222 initially captured publications, 153 articles were studied in detail. Finally, only seven studies met all criteria and were included. Among these seven studies, four were randomized crossover clinical trials of colchicine including a total of 57 patients, one RCT of Andrographis paniculata Herba Nees extract employed in 24 patients, one randomized crossover clinical trial of Rilonacept used in 12 patients, and one RCT of interferon treating 34 acute abdominal attacks in 22 patients. The quality of the colchicine trials was low compared with the other drugs trials. Safety was not clearly mentioned in the trials. Colchicine is an effective treatment in FMF.

Targeted resequencing implicates the familial Mediterranean fever gene MEFV and the toll-like receptor 4 gene TLR4 in Behçet disease.

Genome-wide association studies (GWAS) are a powerful means of identifying genes with disease-associated common variants, but they are not well-suited to detecting genes with disease-associated rare and low-frequency variants. In the current study of Behçet disease (BD), nonsynonymous variants (NSVs) identified by deep exonic resequencing of 10 genes found by GWAS (IL10, IL23R, CCR1, STAT4, KLRK1, KLRC1, KLRC2, KLRC3, KLRC4, and ERAP1) and 11 genes selected for their role in innate immunity (IL1B, IL1R1, IL1RN, NLRP3, MEFV, TNFRSF1A, PSTPIP1, CASP1, PYCARD, NOD2, and TLR4) were evaluated for BD association. A differential distribution of the rare and low-frequency NSVs of a gene in 2,461 BD cases compared with 2,458 controls indicated their collective association with disease. By stringent criteria requiring at least a single burden test with study-wide significance and a corroborating test with at least nominal significance, rare and low-frequency NSVs in one GWAS-identified gene, IL23R (P = 6.9 × 10(-5)), and one gene involved in innate immunity, TLR4 (P = 8.0 × 10(-4)), were associated with BD. In addition, damaging or rare damaging NOD2 variants were nominally significant across all three burden tests applied (P = 0.0063-0.045). Furthermore, carriage of the familial Mediterranean fever gene (MEFV) mutation Met694Val, which is known to cause recessively inherited familial Mediterranean fever, conferred BD risk in the Turkish population (OR, 2.65; P = 1.8 × 10(-12)). The disease-associated NSVs in MEFV and TLR4 implicate innate immune and bacterial sensing mechanisms in BD pathogenesis.

The relationship between nailfold capillaroscopic assessment and telangiectasia score with severity of peripheral vascular involvement in systemic sclerosis.

OBJECTIVES: To determine the association of nailfold video-capillaroscopy (NVC) findings and telangiectasia score with digital ulcer (DU) history and severity of peripheral vascular involvement (PVI) in systemic sclerosis (SSc). METHODS: Fifty-nine SSc patients fulfilling Leroy & Medsger criteria were evaluated including telangiectasia score, disease activity and severity scores. NVC was performed according to qualitative (early, active and late patterns) and semi-quantitative assessments. RESULTS: When DU+ and DU- groups were compared; the mean score of capillary number (CN) was 2.0±0.5 vs. 1.4±0.7 (p<0.001), irregularly enlarged capillaries (IEC) was 1.8±0.6 vs. 1.4±0.7 (p<0.05), microangiopathy evolution score (MES) was 2.5±1.5 vs. 1.8±1.0 (p<0.05) and 'early' pattern was significantly less frequent in DU+ patients (1 vs. 9, p=0.016). The frequency of severe-PVI (Medsger severity score of 2-4) was 22% in females (12/54) and 80% in males (4/5). When severe and non-severe groups were compared; the mean score of CN was 2.1±0.4 vs. 1.5±0.7 (p<0.001), MES was 2.8±1.6 vs. 1.8±1.1 (p<0.05) and 'early' pattern was significantly less frequent in patients with severe PVI (0 vs. 9, p=0.049). The mean values of telangiectasia score were similar between groups. CONCLUSIONS: DU history and severe PVI in SSc were associated with capillary loss and microangiopathy. 'Early' NVC pattern was very rare in patients with DU history and was not found in severe PVI. Severe PVI in males was more frequent than females. Telangiectasia scores were not found to be related to PVI. NVC may be a helpful method in the assessment of SSc patients for PVI prognosis, warranting prospective studies.

Osteoporosis and osteopathy markers in patients with mastocytosis.

OBJECTIVE: Osteoporosis, osteosclerosis, and lytic bone lesions have been observed in patients with systemic mastocytosis (SM). We examined bone mineral density (BMD) biochemical turnover markers and serum tryptase levels in SM, which is considered a rare disease. MATERIALS AND METHODS: Seventeen adult patients (5 females, 12 males; median age: 33 years, range: 20-64) with mastocytosis were included in this study. We investigated the value of quantitative ultrasound (QUS) of the calcaneus in the assessment of BMD in SM patients, as well as BMD of the lumbar spine (L1-L4), femoral neck, and distal radius using dual energy x-ray absorptiometry (DXA) and plasma tryptase levels, biochemical markers of bone turnover. RESULTS: At lumbar spine L1-L4, the femoral neck, and the distal radius or as calcaneus stiffness, 12 of 17 patients had T-scores of less than -1 at least at 1 site, reflecting osteopenia. Three of 17 patients had T-scores showing osteoporosis (T-score <-2.5). There was no relationship between DXA and bone lesion severity. We also found a significant positive correlation between tryptase levels and disease severity, as well as between disease severity and pyridinoline (p<0.01 by Spearman's test). CONCLUSION: DXA and calcaneal QUS may not be appropriate techniques to assess bone involvement in SM patients because of the effects of osteosclerosis. This study further shows that the osteoclastic marker pyridinoline is helpful in patients with severe disease activity and sclerotic bone lesions to show bone demineralization.

Small bowel mucosal damage in familial Mediterranean fever: results of capsule endoscopy screening.

OBJECTIVE: Familial Mediterranean fever (FMF) is the most common form of autoinflammatory diseases. We aimed to evaluate the small bowel mucosa by capsule endoscopy (CE) in FMF patients for investigation of other possible causes of abdominal pain. MATERIAL AND METHODS: The study group consisted of 41 patients with FMF. A standard questionnaire was used to record the gastrointestinal symptoms, other clinical findings, Mediterranean fever gene (MEFV) mutations, and history of medications including non-steroidal anti-inflammatory drugs (NSAIDs). Gastroscopy, colonoscopy and small bowel CE were performed in all patients, and biopsies were taken from terminal ileum and duodenum. RESULTS: The mean age of the patients was 34 ± 11 years, 63% of them were female, and 76.5% of them were carrying MEFV exon 10 mutations. Only one patient used NSAIDs in addition to colchicine. In endoscopic investigations, gastric erosion was detected in only one patient, and no significant findings were detected in colonoscopy. CE showed small bowel mucosal defects in 44% (erosions in 26.8%, ulcer in 17.1%) and edema in 29.3% of the patients. Most (64%) of the ulcer and erosions were localized to jejunum, and only 24% were in ileum. Mitotic changes as an indirect finding of colchicine toxicity were not different from the changes observed in samples of independent group of patients with irritable bowel syndrome. CONCLUSION: Mucosal defect was observed in half of the FMF patients, which may be associated with underlying inflammation or chronic colchicine exposure. Detection of nonspecific chronic inflammation without mitotic changes supports that mucosal defects may be associated with the autoinflammatory process.

Corticosteroid treatment normalizes QTc prolongation and improves heart block in an elderly patient with anti-Ro-positive systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a multisystemic disease which potentially involves various organs including the skin, joints, kidneys, liver, hematopoetic system, and serous membranes. It is rarely seen in elderly males. The most common cardiovascular involvement type is pericarditis. Anti-Ro antibodies may be associated with neonatal lupus which causes heart blocks. Recent literature indicates that anti-Ro antibodies may be associated with various rhythm and conduction disturbances in the adulthood. The most common finding associated with anti-Ro antibodies is prolonged corrected QT (QTc) interval. Herein, we present an elderly male patient with anti-Ro-positive SLE associated with prolonged QTc interval and AV blocks that significantly improved after corticosteroid treatment.

MEFV gene variations in patients with systemic lupus erythematosus.

OBJECTIVE: The aim of this study was to investigate the frequency of familial Mediterranean fever (FMF)-associated MEFV gene variations in patients with systemic lupus erythematosus (SLE). METHODS: The study group comprised 190 SLE patients and 101 healthy controls of Turkish origin with no clinical features of FMF. All individuals were genotyped for the four most common MEFV gene variations (M694V, M680I, V726A and E148Q) by PCR-restriction fragment length polymorphism analysis. RESULTS: The frequency of carrying any of the four MEFV gene variations under study was 15 % in patients with SLE and 10 % in the healthy controls (p = 0.23). After the exclusion of the less penetrant E148Q variation, re-analysis for the three penetrant mutations revealed a significant association between exon 10 variations and pericarditis [p = 0.038, odds ratio (OR) 3.5, 95 % confidence interval (CI) 1.0-12.1], and pleural effusion (p = 0.043, OR 5.2, 95 % CI 0.8-30.9). No significant association was detected between the MEFV gene variations and a higher acute phase response. CONCLUSIONS: The MEFV gene variations analyzed in our study do not seem to increase the overall susceptibility to SLE and do not have any strong association with its clinical manifestations. The possibility of a modest effect of penetrant exon 10 MEFV variants on the development of serosal effusions needs to be explored in a larger series of patients.

The Relationship between P-Selectin Polymorphisms and Thrombosis in Antiphospholipid Syndrome: A Pilot Case-Control Study.

OBJECTIVE: The selectins are cell adhesion molecules that mediate the interactions among leukocytes, activated platelets, and endothelial cells. We aimed to investigate whether P-selectin polymorphisms are associated with thrombosis in patients with antiphospholipid syndrome (APS). MATERIALS AND METHODS: The diagnosis and classification of APS were based on the report of an international workshop. Genomic DNA was extracted from citrated blood samples of all subjects. Three single nucleotide polymorphisms associated with the P-selectin coding region (S290N, c.1087G>A; N562D, c.1902G>A; T715P, c.2363A>C) were assessed. RESULTS: There were 26 APS (65%) patients with thrombosis. The number of patients without thrombosis was 14 (35%). The frequency of the N562D-DN genotype was significantly higher in patients with APS than in healthy controls (p=0.003). The frequency of this genotype was significantly higher in patients with APS with thrombosis compared with patients with no thrombosis (p=0.03). The N562D-NN genotype was found at a higher frequency in patients with APS than in healthy controls (p=0.004). CONCLUSION: Our results suggest that the N562D polymorphism of the DN genotype of P-selectin is associated with an increased risk of thrombosis in patients with APS.

Bone density in proton pump inhibitors users: a prospective study.

Patients with gastroesophageal reflux disease (GERD) receive long-term therapy with proton pump inhibitor (PPI) agents. Several studies have recently been published suggesting that treatment with PPI may cause bone fractures, although the number of prospective studies in this regard is limited. The aim of this study is to prospectively investigate the effect of PPIs on bone density. Between March 2009 and January 2011, 114 GERD patients (18-56 years) and 110 healthy controls were included in the present study. Bone mineral densitometry (BMD) by using dual-energy X-ray absorptiometry was assessed at lumbar spine and femur neck. BMD measurements were performed on all subjects at the beginning of the study. The patients were divided according to three drugs by their treatment with esomeprazole, lansoprazole, or pantoprazole. The study group was followed for at least 6 months on PPI therapy, and then BMD measurements were repeated. The mean duration of treatment with PPIs was 8.5 ± 2.3 months. In patients receiving PPIs, the mean reduction in total vertebra T score following treatment compared to pre-treatment values was 00.23 ± 0.42 units (95 % CI 0.15-0.30) (p < 0.01), while the mean reduction in the femur T score was 0.10 ± 0.40 units (95 % CI 0.03-0.18) (p = 0.03). Reduction following treatment in L4 and total vertebra T scores of lansoprazole group was significantly higher than of pantoprazole group (p = 0.04). Reduction in femur T score of esomeprazole group was higher than of lansoprazole group and pantroprazole group, but it is not statistically significant. Treatment with a PPI results in a significant reduction in bone density. Close monitoring is beneficial for patients who are to receive long-term treatment with PPI.

MEFV gene variations in patients with systemic lupus erythematosus.

OBJECTIVE: The aim of this study was to investigate the frequency of familial Mediterranean fever (FMF)-associated MEFV gene variations in patients with systemic lupus erythematosus (SLE). METHODS: The study group comprised 190 SLE patients and 101 healthy controls of Turkish origin with no clinical features of FMF. All individuals were genotyped for the four most common MEFV gene variations (M694V, M680I, V726A and E148Q) by PCR-restriction fragment length polymorphism analysis. RESULTS: The frequency of carrying any of the four MEFV gene variations under study was 15 % in patients with SLE and 10 % in the healthy controls (p = 0.23). After the exclusion of the less penetrant E148Q variation, re-analysis for the three penetrant mutations revealed a significant association between exon 10 variations and pericarditis [p = 0.038, odds ratio (OR) 3.5, 95 % confidence interval (CI) 1.0-12.1], and pleural effusion (p = 0.043, OR 5.2, 95 % CI 0.8-30.9). No significant association was detected between the MEFV gene variations and a higher acute phase response. CONCLUSIONS: The MEFV gene variations analyzed in our study do not seem to increase the overall susceptibility to SLE and do not have any strong association with its clinical manifestations. The possibility of a modest effect of penetrant exon 10 MEFV variants on the development of serosal effusions needs to be explored in a larger series of patients.

Diaphragmatic movements in ankylosing spondylitis patients and their association with clinical factors: an ultrasonographic study.

We compared diaphragmatic motion between ankylosing spondylitis (AS) patients and controls, as assessed by the ultrasonographic method. We included 33 consecutive AS patients (19 males, 14 females) followed up at our center and 14 apparently healthy controls (8 males, 6 females) into our study. AS patients fulfilled the modified New York classification criteria for AS. Patients' demographic and clinical data, functional parameters, and radiographic findings were recorded down. By evaluating the motion of right and left diaphragm during deep expirium and inspirium, the mean diaphragmatic motion was determined by ultrasonography. Diaphragmatic motion in AS patients was less than in controls, but the difference was not significant (68.9 ± 17 mm vs. 77.8 ± 22.4 mm, P = 0.14). Diaphragmatic motion in AS patients who were active according to BASDAI score (>4) was not different from inactive patients (70.4 ± 20.5 vs. 67.5 ± 13.5, P > 0.05). The mean diaphragmatic motion had a positive correlation with occiput-to-wall distance (r = 0.35, P = 0.048); and negative correlations with cervical rotation (r = -0.45, P = 0.01) and modified Schober test (r = -0.34, P = 0.05) in AS patients. We did not detect any association of mean diaphragmatic motion with thoracic expansion on deep expiration. Diaphragmatic motion in AS does not differ significantly from the control group. Factors like disease activation, chest expansion, and the severity of radiographic findings do not affect diaphragmatic motion. There is no compensatory increase in diaphragmatic motion in AS.

Magnetic resonance imaging of rheumatoid meningitis: a case report and literature review.

Rheumatoid meningitis is a rare and serious complication of rheumatoid arthritis (RA) with high mortality rate. Clinical importance of the disease is high because diagnosis is difficult, and the disease is treatable if diagnosed successfully. We present the clinical and cranial magnetic resonance imaging findings of 62-year-old female patient with RA who has been followed up for 4 years.

A case of primary Sjögren's syndrome presenting primarily with central nervous system vasculitic involvement.

Sjögren's syndrome is primarily a chronic systemic autoimmune disease that affects exocrine organs. Neurologic symptoms frequently present as peripheral neuropathy due to small vessel vasculitis. Type and prevalence of central nervous system involvement are still controversial. In this report, we present a 35-year-old woman with primary Sjögren's syndrome with central nervous system vasculitic involvement.

Budd-Chiari syndrome in Behçet's disease: a retrospective multicenter study.

OBJECTIVE: To compare the clinical features, laboratory findings, and prognosis of Behçet's disease (BD) patients with and without Budd-Chiari syndrome (BCS). METHODS: This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. RESULTS: Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14-47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). CONCLUSION: To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points • Mortality rate is higher in BD-associated BCS patients with IVC involvement. • Chronic and silent form of BD-associated BCS has a better prognosis. • The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial.