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Background/Aims Neurologic involvement in Behcet’s syndrome (BS) remains an unelucidated area that needs further attention. In this study, we aimed to analyze the patterns of neurologic involvement and evaluate their association with other clinical features. Methods 800 patients with BS diagnosis according to The International Criteria for Behcet’s Disease (ICBD) followed in our center were retrospectively screened and 55 patients with central nervous system involvement and 20 patients with non-vascular BS were enrolled in this study. These patients were classified as sinus vein thrombosis (SVT), parenchymal involvement, and mixed (SVT and parenchymal). These groups were compared in terms of demographic and clinical features. Results Thirty-two (58,2%) of the patients were male. Distribution of neurologic involvement were as follows; 22 (40%) with isolated sinus vein thrombosis (SVT), 25 (%45) with isolated parenchymal, and 8 (%15) with mixed pattern. Central nervous involvement was the diagnostic feature of BS in 25 patients. Headache was the most common symptom followed by neurologic deficits and dizziness (44%, 25%, 16%). The most common sites of involvement were subcortical and periventricular areas followed by brainstem (63% vs 37%). In SVT group, positive family history of BS (p < 0.001) and previous deep vein thrombosis (DVT) (p:0.004) were found higher. Smoking history, erythema nodosum, cardiac and pulmonary involvement were significantly higher in parenchymal group (p < 0.001) (Table 1). Conclusion Although brainstem involvement is considered as more characteristic in BS, white matter and periventricular involvement were also found as common in our study. Notably, these subgroup analyses may suggest the speculation that BS shows clusters even within the neuro-vascular involvement. Disclosure R. Yıldırım: Other; Reşit Yıldırım is a member of Rheumatology journal as an associate editor. M. Dinler: None. A.E. Arslan: None. N.S.Y. Bilge: None. T. Kaşifoğlu: None.

Objective Persistent inflammation is an insidious and less studied feature of FMF. We investigated clinical determinants of persistent inflammation and its associations with individual damage items. Methods This is a cross-sectional analysis of 917 FMF patients, who fulfilled the Tel Hashomer criteria and had at least 6 months’ follow-up. Patients were stratified based on whether they had persistent inflammation. We used logistic regression analysis to investigate independent predictors of persistent inflammation and the associated individual damage items. Results One hundred and forty-two (15%) patients had persistent inflammation. Active FMF (54%) was the most prominent reason for the persistent inflammation. Spondylarthritis (16%), other inflammatory arthritis (8%) and IBD (2%) were other frequent reasons. Male gender, history of exertional leg pain, inflammatory comorbidities, M694V homozygosity, colchicine resistance, lower education levels and musculoskeletal attack dominance were found to be the independent predictors of persistent inflammation. Earlier disease onset led to a tendency towards persistent inflammation. Patients with persistent inflammation were more likely to suffer damage. There is an increased risk of developing proteinuria, amyloidosis and renal insufficiency. Conclusion We identified, for the first time, the predictors of persistent inflammation in adult FMF patients and related individual damage items of the Autoinflammatory Disease Damage Index. Persistent inflammation is insidious and one of the chief causes of damage; therefore, especially patients with these predictors should be followed up more closely. If detected, underlying inflammatory comorbidities should be assessed meticulously as early detection and proper treatment strategies may favourably impact the natural history of the disease.

The temporal relationship between the onset of skin psoriasis (Pso) and psoriatic arthritis (PsA) is a long and well-known feature of psoriatic disease. Psoriatic skin lesions emerge years before the development of musculoskeletal manifestations in nearly 75% of PsA patients. While skin and joint manifestations develop within one year in 15-20% of the patients, joint manifestations precede the skin involvement by a year or more in about 10% of the patients (1).

Objective. The aim of this study was to evaluate whether there are clinical subgroups that may have different prognoses among FMF patients. Methods. The cumulative clinical features of a large group of FMF patients [1168 patients, 593 (50.8%) male, mean age 35.3 years (S.D. 12.4)] were studied. To analyse our data and identify groups of FMF patients with similar clinical characteristics, a two-step cluster analysis using log-likelihood distance measures was performed. For clustering the FMF patients, we evaluated the following variables: gender, current age, age at symptom onset, age at diagnosis, presence of major clinical features, variables related with therapy and family history for FMF, renal failure and carriage of M694V. Results. Three distinct groups of FMF patients were identified. Cluster 1 was characterized by a high prevalence of arthritis, pleuritis, erysipelas-like erythema (ELE) and febrile myalgia. The dosage of colchicine and the frequency of amyloidosis were lower in cluster 1. Patients in cluster 2 had an earlier age of disease onset and diagnosis. M694V carriage and amyloidosis prevalence were the highest in cluster 2. This group of patients was using the highest dose of colchicine. Patients in cluster 3 had the lowest prevalence of arthritis, ELE and febrile myalgia. The frequencies of M694V carriage and amyloidosis were lower in cluster 3 than the overall FMF patients. Non-response to colchicine was also slightly lower in cluster 3. Conclusion. Patients with FMF can be clustered into distinct patterns of clinical and genetic manifestations and these patterns may have different prognostic significance.