Comparison of clinical features in FMF patients according to severity scores: An analysis with the ISSF scoring system.

OBJECTIVE: Familial Mediterranean fever (FMF), is an auto-inflammatory disease characterized by attacks of fever and serositis. Some scoring systems have been developed to evaluate the severity of the disease, however, predicting the severity of FMF is not possible with current knowledge. Our aim in this study was to evaluate the factors affecting disease severity in FMF. METHODS: This study included 150 FMF patients. The medical data of the patients were collected retrospectively and the International severity scoring system for Familial Mediterranean fever (ISSF) was used to evaluate disease severity. RESULTS: Patients were sorted into 3 groups based on the ISSF scores; ≤2=mild (Group 1; n: 61), 3-5=moderate (Group 2; n: 70), and ≥6=severe (Group 3; n: 19). Age at the onset of disease and age at diagnosis was younger in patients with severe disease (p: 0.009 and p: 0.031, respectively). Fever, peritonitis, and vasculitis were similar in all 3 groups. Pleuritis, erysipelas-like erythema (ELE), arthritis, myalgia, amyloidosis, and chronic kidney disease (CKD) were more common in Group 3. CONCLUSION: FMF patients with early onset and early diagnosis, having more frequent pleuritis, ELE, arthritis, and myalgia tended to manifest a more severe form of the disease. Close monitoring of such patients may prevent the development of amyloidosis and CKD and improve the long-term prognosis of the disease.

A rare but serious manifestation of Behçet's disease: intracardiac thrombus in 22 patients.

OBJECTIVES: Behçet's disease (BD) is a chronic, multisystemic disorder characterised by recurrent oral aphtous ulcers, genital ulcers and ocular inflammation. Vasculitis and thrombotic events are the most important causes of mortality. Vena cava thrombosis, pulmonary artery aneurysms, Budd-Chiari syndrome, peripheral artery aneurysms, dural sinus thrombosis and abdominal aorta aneurysms are the other less common vascular manifestations of BD. Cardiac involvement in BD is a rare and life-threatening complication. The aim of this study was to assess the clinical characteristics and outcome of patients with BD who have intracardiac thrombus. METHODS: The hospital files of BD patients followed by rheumatology clinics of four medical centres (Ankara University, Ege University, Hacettepe University and Eskisehir Osmangazi University Hospitals) were retrospectively evaluated. Data included patients' demographic and clinical features, laboratory findings and outcome. All patients fulfilled three or more of the International Study Group Criteria for BD. RESULTS: Twenty-two patients with intracardiac thrombus were evaluated. The mean age of patients with intracardiac thrombosis was 29.1 yrs (22-44) and there was a male predominance with a ratio of 20:2. Cardiac involvement was the first clinical manifestation of BD in 9 of the 22 patients. Initial symptoms of the patients were fever (n=18, 81%), dyspnea (n=9, 40%) chest pain (n=9, 40%) and haemoptysis (n=7, 31.8%). Sixteen patients (72%) had pulmonary arteritis and 10 (45%) patients had venous system lesions included deep vein, inferior vena cava and hepatic vein. Intra-cardiac thrombus were found only in the right cavities in 17 patients (77%), only in the left cavities in 2 patients (9%), and in both left and right cavities of the heart in 3 patients (13.6%). Once the cardiac lesion was diagnosed as a complication of BD, high dose (1mg/kg/d) prednisone (n=22, 100%) plus cyclophosphamide (n=18, 81%) or azathioprine (n=3, 13.6%) and warfarin (n=8, 36.3% (after the elimination of pulmonary aneurysm) therapy for anticoagulation was initiated. Four patients (18%) had high dose prednisone plus cyclophosphamide plus interferon-α (IFN-α) combination treatment and two patients (n=2, 9%) had high dose prednisone plus cyclophosphamide or plus azathioprine combination treatment. After treatment, the intra-cardiac thrombus disappeared in 13 cases and the size of the thrombus reduced in 7 cases. One patient died because of recurrent intra-cardiac thrombus and massive pulmonary arteritis in the emergency department. CONCLUSIONS: Intracardiac thrombus in BD is more common in young men. The right side of the heart is usually involved and cardiac involvement is often accompanied by pulmonary artery occlusion possibly due to pulmonary arteritis. Early and aggressive immunosuppressive and/or anticoagulation therapy are life-saving.

The role of hemostatic mechanisms in the development of thrombosis in Behcet's disease: an analysis by modified rotation thromboelastogram (ROTEM).

Behcet's disease (BD) is a multisystemic disorder characterised by recurrent oral and genital ulcers. Vasculitis and thrombotic events are the most important causes of mortality. Thrombosis is the major clinical finding in patients with BD, but the cause of the thrombosis is still unclear. Thromboelastography is an alternative method to evaluate almost all steps of the hemostatic system. Today, the modified rotation thromboelastogram (ROTEM) is a newer coagulation test and a more powerful technique. Our aim in this study was to investigate whether hemostatic mechanisms play a role in the development of thrombosis in BD patients by using ROTEM. Thirty BD patients, 20 ankylosing spondylitis patients, and 14 healthy controls who are not taking anti-aggregant or anti-coagulant therapy were included in the study. Whole blood count, protrombin time, activated protrombin time, fibrinogen, D-dimer levels, and ROTEM parameters (clotting time, clot formation time (CFT), and maximum clot formation(MCF)) were determined by INTEM and EXTEM analysis. Of the 30 patients with BD, 19 were women and 11 were men, and mean age was 40.6 ± 11.2. Two of the BD patients had vascular involvement, but none of them were in active phase of the disease during the study. In INTEM assay, MCF (p < 0,001) value was significantly increased, and CFT (p>0.05) value was decreased in BD patients compared with the control group. In the EXTEM assay, there was a similar significant increase in MCF (p=0.002) value and a decrease in CFT (p=0.002) value in BD patients compared with the control group. The results of our study indicated that primary hemostatic mechanisms which can be detected by ROTEM may play a role in the development of thrombosis in patients with BD.