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.

Demographic, clinical, and laboratory features of Turkish patients with gouty arthritis: A prospective cohort study.

OBJECTIVE: We have conducted this study to evaluate clinical and laboratory findings and to gather and evaluate information that would be useful in clinical practice, such as demographics, joint involvement patterns, laboratory anomalies, treatments applied, and responses obtained in patients diagnosed with gout. METHODS: In our study, the demographic, clinical, and laboratory characteristics of 94 patients diagnosed with gout were evaluated. The patients were re-evaluated with regard to their clinical and laboratory findings at the end of the 1st and 3rd months, their responses to the treatment were observed, and where necessary, new treatment adjustments were made. RESULTS: Seventy-nine (84%) of the patients were men, and 15 (16%) were women. The ages of the patients ranged between 22 and 86 years with the average age being 57.5 years. The joint involvement during a gouty arthritis attack was most frequently observed in the 1st metatarsophalangeal joint (87.2%), followed by the ankle joint (26.9%), and the knee joint (23.7%). The joint involvement pattern was evaluated to reveal that the acute monoarticular arthritis was observed in 87 patients (93.5%), followed by acute oligoarticular arthritis coming in second in terms of frequency (26.9%). An average attack was determined to last 10.7 days, and the average number of attacks in a year was 2.69. In our study, the colchicine treatment in 24 of our patients, in whom it was determined during the admission and follow-up process that they have not had gouty arthritis attack in the past 3 months, was stopped. Five (20.8%) patients whose colchicine treatment was stopped later developed gouty arthritis attacks, and the colchicine treatment was restarted. On the other hand, 10 out of 38 patients (26.3%), who have either been continuing or had never been on colchicine treatment, were observed to experience a gouty arthritis attack during their 1st and 3rd month check-ups. CONCLUSION: In patients with gout, it is recommended that the related diseases, as well as the triggering factors (alcohol, drug use, and dietary effect), be determined, the necessary lifestyle changes be made, and the treatments started.

Exon 2: Is it the good police in familial mediterranean fever?

OBJECTIVE: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease. Most of the identified disease-causing mutations are located on exon 10. As the number of studies about the effect of the exonal location of the mutation and its phenotypic expression is limited, we aimed to investigate whether the exonic location of the Mediterranean fever (MEFV) mutation has an effect on the clinical manifestation in patients with FMF. METHODS: Study population was derived from the main FMF registry that included 2246 patients from 15 different rheumatology clinics. We categorized the mutations according to their exon locations and retrieved the clinical and demographic information from the database. RESULTS: Patients having the MEFV mutations on exon 2 or 10 (n:1526) were divided into three subgroups according to the location of the MEFV mutations: Group 1 (exon 2 mutations), Group 2 (exon 10 mutations), and Group 3 (both exon 2 and exon 10 mutations). Group 2 patients were of a significantly younger age at onset, and erysipel-like erythema, arthritis, amyloidosis, and a family history of FMF were more common in this group. CONCLUSION: Patients with FMF and exon 10 mutations show more severe clinical symptoms and outcome. Exon 2 mutations tend to have a better outcome.

Cavitary pulmonary nodules in rheumatoid arthritis; case reports and review of the literature.

Rheumatoid nodules are the most common pulmonary manifestations of rheumatoid arthritis (RA) and are usually asymptomatic. In rare cases, they progress cavitary formation and cause severe clinical symptoms because of disease activity, infectious diseases, and other etiologies. The determination of clinical and histopathological features may be helpful for differential diagnosis in patients with RA with cavitary pulmonary nodules. Herein, we report two female patients with RA with cavitary pulmonary nodules and aim to obtain more detail data by means of reviewing previously reported cases.

Short report: correlates of functional disability with disease activity in elderly patients with rheumatoid arthritis.

Rheumatoid arthritis (RA) prevalence increases with age and old people are special patient population. The recognition of functional disability related to RA could be challenging in elderly patients because aging itself and potential co-morbid disease may also cause functional disability. In this study, we aimed to look at the correlation between disease activity and functional disability in elderly RA patients. Elderly RA patients, ≥65 years old at their routine visits were included in the study. The composite 'disease activity score' in 28 joints (DAS-28) was used to determine disease activity groups. Health assessment questionnaire (HAQ) scores were calculated to describe the functional disability and compared across the disease activity groups. Two hundred and fifty-eight RA patients with the mean age of 71 ± 5 (65-90) and a total disease duration of 8.4 ± 8.5 (.5-50) years were recruited. The proportion of patients with high and moderate disease activity was 70%. HAQ scores were significantly correlated with disease activity (p < .05). Functional disability estimated by HAQ was correlated with disease activity in elderly patients with RA.

Nationwide Experience With Off-Label Use of Interleukin-1 Targeting Treatment in Familial Mediterranean Fever Patients.

OBJECTIVE: Approximately 30-45% of patients with familial Mediterranean fever (FMF) have been reported to have attacks despite colchicine treatment. Currently, data on the treatment of colchicine-unresponsive or colchicine-intolerant FMF patients are limited; the most promising alternatives seem to be anti-interleukin-1 (anti-IL-1) agents. Here we report our experience with the off-label use of anti-IL-1 agents in a large group of FMF patients. METHODS: In all, 21 centers from different geographical regions of Turkey were included in the current study. The medical records of all FMF patients who had used anti-IL-1 treatment for at least 6 months were reviewed. RESULTS: In total, 172 FMF patients (83 [48%] female, mean age 36.2 years [range 18-68]) were included in the analysis; mean age at symptom onset was 12.6 years (range 1-48), and the mean colchicine dose was 1.7 mg/day (range 0.5-4.0). Of these patients, 151 were treated with anakinra and 21 with canakinumab. Anti-IL-1 treatment was used because of colchicine-resistant disease in 84% and amyloidosis in 12% of subjects. During the mean 19.6 months of treatment (range 6-98), the yearly attack frequency was significantly reduced (from 16.8 to 2.4; P < 0.001), and 42.1% of colchicine-resistant FMF patients were attack free. Serum levels of C-reactive protein, erythrocyte sedimentation rate, and 24-hour urinary protein excretion (5,458.7 mg/24 hours before and 3,557.3 mg/24 hours after) were significantly reduced. CONCLUSION: Anti-IL-1 treatment is an effective alternative for controlling attacks and decreasing proteinuria in colchicine-resistant FMF patients.

Oral ulcer activity in Behcet's disease: Poor medication adherence is an underestimated risk factor.

OBJECTIVE: The aim of this study was to evaluate the relationship between oral ulcer activity and medication adherence according to gender in Behçet's disease (BD) patients. MATERIAL AND METHODS: The study group included 330 BD patients (F/M: 167/163, mean age: 38.5±10.5 years). Oral ulcer activity and medication adherence were evaluated in the previous month. Medication adherence was evaluated using the 8-item Morisky Medication Adherence Scale (MMAS-8) having a score range of "0" to "8" with high scores indicating better adherence. Low adherence was defined as <6 points on MMAS-8. RESULTS: Over half of the group had active oral ulcers (n=219, 66.4%) within the month preceding the visit. The number of oral ulcers was significantly higher in female patients with low medication adherence (2.39±3.24) than in the rest of the female group (1.28±2.05; p=0.023). Although a similar trend was also observed in male patients (2.14±3.3 vs. 1.81±2.31), a significant relationship was not observed (p=0.89). The frequency of medication intake per day was lower in patients with high medication adherence than in the rest of the study group (p=0.04). CONCLUSION: Low medication adherence is a hidden risk factor in the management of BD. Poor adherence was associated with oral ulcer activity in female BD patients.

Serum leptin levels do not correlate with disease activity in rheumatoid arthritis.

OBJECTIVES: Leptin, is a fat tissue hormone which effects energy expenditure, food intake , hematopoiesis, osteogenesis, angiogenesis, reproductive and immune systems. We aimed to determine serum leptin levels and investigate the association between disease activity and other parameters in RA patients. METHODS: Patients with RA (n=106) as the study group, healthy controls (n=52) and osteoarthritis (OA) patients (n=37) as a control group were enrolled to the study. RA patients were categorized in four different groups according to DAS28 scores: remission ,low (LDA), moderate (MDA) or high (HDA) disease activity . RESULTS: No differences were present between the body mass indices of the three groups. Mean leptin levels in RA patients, OA group and healthy individuals were 25,60 ± 13,41, 23,03 ± 11,51 and 23,81 ± 12,85 ng/ml, respectively and no significant difference was present between the groups. Nine of (8,5%) RA patients were in remission, 16 (15,1%) were in LDA, 40 (37,7%) in MDA and 41 (38,7%) were in HDA. Leptin levels did not correlate with DAS28 scores of RA patients (r=-0,12, p=0,11). Mean leptin levels in RA patients with remission was 32,65 ± 7, 28 in LDA 23,94 ± 10,94 in MDA 26,73 ± 14,92 and in HDA 23,59 ± 13,50 ng/ml (p=NS). No associations were observed between leptin levels and CRP, ESR, RF positivity and disease duration. CONCLUSIONS: Our study revealed no correlation of disease activity and serum leptin levels. Therefore leptin does not seem to be an appropriate biomarker to monitorize inflammation in RA.

Unmet need in Behcet's disease: most patients in routine follow-up continue to have oral ulcers.

The clinical course of Behcet's disease (BD) as a multisystemic disorder with a remitting-relapsing nature is insufficiently explored. As complete remission should be aimed in all inflammatory diseases, we investigated the frequency of complete remission in patients with BD followed in long-term, routine practice. In this retrospective study, 258 patients with BD who were regularly followed in outpatient clinics were assessed. The demographic and clinical data for active organ manifestations and treatment protocols were evaluated, and "complete remission" for this study was defined as no sign of any disease manifestation in the current visit and the preceding month. Two hundred fifty-eight patients with BD (F/M 130/128, mean age 41.1 ± 11.5 years) were included to the study. Mucocutaneous disease was present in 48.4 % (n = 125). Mean visit number was 6.8 ± 2.7, and mean follow-up duration was 45.8 ± 36.5 months. Patients were clinically active in 67.2 % (n = 1,182) of the total visits (n = 1,757), which increased to 75.6 % (68.1-90.3) when the month preceding the visit was also included. The most common active manifestation was oral ulcers (39.4-63.2 %) followed by other mucocutaneous manifestations and musculoskeletal involvement. When multivariate analysis was performed, oral ulcers, which are the main cause of the clinical activity, negatively correlated with immunosuppressive treatments (ß = -0.356, p < 0.000) and age (ß = -0.183, p = 0.04). It is fairly difficult to achieve complete remission in BD with current therapeutic regimens. The reluctance of the clinician to be aggressive for some BD manifestations with low morbidity, such as mucocutaneous lesions and arthritis, might be influencing the continuous, low-disease activity state, especially due to oral ulcers, in BD patients.

Amyloidosis and its related factors in Turkish patients with familial Mediterranean fever: a multicentre study.

OBJECTIVE: The primary aim of this study was to investigate the prevalence of amyloidosis and its related factors in a large number of FMF patients. METHODS: Fifteen centres from the different geographical regions of Turkey were included in the study. Detailed demographic and medical data based on a structured questionnaire and medical records were collected. The diagnosis of amyloidosis was based on histological proof of congophilic fibrillar deposits in tissue biopsy specimens. RESULTS: There were 2246 FMF patients. The male/female ratio was 0.87 (1049/1197). The mean age of the patients was 34.5 years (S.D. 11.9). Peritonitis was the most frequent clinical finding and it was present in 94.6% of patients. Genetic testing was available in 1719 patients (76.5%). The most frequently observed genotype was homozygous M694V mutation, which was present in 413 (24%) patients. Amyloidosis was present in 193 patients (8.6%). Male sex, arthritis, delay in diagnosis, M694V genotype, patients with end-stage renal disease (ESRD) and family history of amyloidosis and ESRD were significantly more prevalent in patients with amyloidosis compared with the amyloidosis-negative subjects. Patients with homozygous M694V mutations had a 6-fold higher risk of amyloidosis compared with the other genotypes (95% CI 4.29, 8.7, P < 0.001). CONCLUSION: In this nationwide study we found that 8.6% of our FMF patients had amyloidosis and homozygosity for M694V was the most common mutation in these patients. The latter finding confirms the association of homozygous M694V mutation with amyloidosis in Turkish FMF patients.