Sedat Yilmaz

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.

Living with scleroderma: patients’ perspectives, a phenomenological study

In this study, it is aimed to determine the daily life experiences of patients on the basis their own way of statement. Sixteen patients with scleroderma were enrolled to this qualitative study. Data were collected using both a demographic data form and a semi-structured interview form. Study was made on individual patient interview by face-to-face manner. Data were evaluated using Colaizzi’s phenomenological data analysis method. Data analysis revealed four categories and nine topics. These categories were (1) physical impact of disease, (2) emotional impact of disease, (3) social impact of disease and (4) patient behaviours for the cope with the disease. As emotional impact, patients imply that they have experienced disappointment, hope to get well and have fears about the future. In the physical impact category, tight skin, limitations of hand skills, swelling of the hands and feet, fatigue, swallowing difficulties and deformation of their bodies were the prominent features. In patients with recognizable disease, difficulty to join to social activities increases and eventually leads to isolation. There was also some evidence that patients who have been supported by their family and close relatives seem to be more optimistic about their disease. Most patients are not willing to communicate with other patients, particularly in an advanced stage. Scleroderma patients imply that they experience several difficulties regarding emotional, physical and social aspects. Individual abilities to cope with the disease were much more improved among patients who have a sustained social support. For advanced patients with apparent deformations, an effective social support system should be introduced.

Relationship of ultrasonographic findings with synovial angiogenesis modulators in different forms of knee arthritides

Angiogenesis is controlled by a variety of angiogenesis stimulators and inhibitors. The increased power Doppler (PD) signals determined by ultrasonography is an indirect marker of synovial vascularity in arthritis. We aimed to investigate relationship between ultrasonographic findings and synovial angiogenesis modulators. Thirteen Behcet’s disease (BD), 15 spondyloarthropathy, 21 rheumatoid arthritis (RA), and 15 osteoarthritis (OA) patients with knee arthritis were included. Cumulative effusion, synovial hypertrophy, and PD signal scores were calculated in arthritic joints. In synovial fluid samples, angiogenesis inhibitors (angiostatin, thrombospondin-1, and endostatin) and stimulators [bFGF (basic fibroblast growth factor), angiopoietin-1] were studied. The comparisons between groups were made by Kruskal–Wallis test, and correlation analysis was calculated with Pearson and Spearman tests. Effusion scores were significantly higher in inflammatory arthritis than in OA. Synovial hypertrophy scores were higher in RA and spondylarthritis than in OA and BD. PD scores were not different between the groups. Synovial angiostatin and bFGF levels were significantly higher in patients with inflammatory arthritis than in OA. Cumulative effusion scores were positively correlated with angiopoietin-1, angiostatin, and bFGF and negatively correlated with thrombospondin-1 levels. Synovial hypertrophy scores were positively correlated with angiostatin and bFGF levels and negatively correlated with thrombospondin-1. No correlation was found between PD scores and modulators of angiogenesis. In large joints like knee, detecting PD signals alone was not sufficient to assess the angiogenesis. However, cumulative activity scores were positively correlated with angiogenesis stimulators. Therefore, when investigating the angiogenesis, PD technique should be added to gray-scale examinations.

Diagnostic dilemma of paraneoplastic arthritis: case series.

Paraneoplastic arthritis (PA) may mimic rheumatic diseases. While presenting the demographic and laboratory features of the patients diagnosed with PA, this study also aims to provide possible appropriate tools to differentiate the PA cases from early rheumatoid arthritis (ERA).

Response rate of initial conventional treatments, disease course, and related factors of patients with adult-onset Still's disease: Data from a large multicenter cohort.

BACKGROUND Adult-onset Stills disease (AOSD) is a rare condition, and treatment choices are frequently dependent on expert opinions. The objectives of the present study were to assess treatment modalities, disease course, and the factors influencing the outcome of patients with AOSD. METHODS A multicenter study was used to reach sufficient patient numbers. The diagnosis of AOSD was based on the Yamaguchi criteria. The data collected included patient age, gender, age at the time of diagnosis, delay time for the diagnosis, typical AOSD rash, arthralgia, arthritis, myalgia, sore throat, lymphadenopathy, hepatomegaly, splenomegaly, pleuritis, pericarditis, and other rare findings. The laboratory findings of the patients were also recorded. The drugs initiated after the establishment of a diagnosis and the induction of remission with the first treatment was recorded. Disease patterns and related factors were also investigated. A multivariate analysis was performed to assess the factors related to remission. RESULTS The initial data of 356 patients (210 females; 59%) from 19 centers were evaluated. The median age at onset was 32 (16-88) years, and the median follow-up time was 22 months (0-180). Fever (95.8%), arthralgia (94.9%), typical AOSD rash (66.9%), arthritis (64.6%), sore throat (63.5%), and myalgia (52.8%) were the most frequent clinical features. It was found that 254 of the 306 patients (83.0%) displayed remission with the initial treatment, including corticosteroids plus methotrexate with or without other disease-modifying antirheumatic drugs. The multivariate analysis revealed that the male sex, delayed diagnosis of more than 6 months, failure to achieve remission with initial treatment, and arthritis involving wrist/elbow joints were related to the chronic disease course. CONCLUSION Induction of remission with initial treatment was achieved in the majority of AOSD patients. Failure to achieve remission with initial treatment as well as a delayed diagnosis implicated a chronic disease course in AOSD.

AB0658 Cross-Cultural Adaptation, Reliability, and Validity of the Turkish Version of the Compliance Questionnaire on Rheumatology (CQR-T) in Patients with Behçet's Disease

Background Compliance to treatment can be defined as the degree to which patient behavior is consistent with the recommendations made by healthcare providers. Determining the level of the patients compliance will improve the management of a certain disease. Currently, the only self-reported questionnaire for assessing the compliance of patients with rheumatic disease is the 19-item Compliance Questionnaire on Rheumatology (CQR). Objectives The aim of this study was to examine the psychometric properties of the Turkish version of the Compliance Questionnaire on Rheumatology (CQR-T) for patients with Behçets disease (BD) Methods A sample of 105 Turkish patients with BD participated in this study. The scale was cross-culturally adapted through a process including translation, comparison with versions in other languages, back translation, and pretesting. Construct validity was evaluated by factor analysis, and criterion validity was evaluated using the Morisky Medication Adherence Scale (MMAS). Results The mean age of the study group was 30.87±8.06 years. The majority of the participants was male (89.5%). Mean disease duration was 5.67±4.68 years.Among the current medical treatment agents, colchicine and azathioprine were the most commonly used (79 and 50 patients, respectively). The CQR-T demonstrated acceptable internal consistency (Cronbachs alpha coefficient = 0.832), adequate test–retest reliability (intraclass correlation coefficient = 0.630), and correlations with MMAS scores (r=−0.389; p<0.001), indicating convergent validity. Conclusions The CQR-T was found to be a valid and reliable instrument for evaluating the compliance of Turkish BD patients with prescribed medications. The CQR-T might be a helpful tool in two ways: for determining the level of compliance of patients with BD and for adjusting their management and follow-up based on the results. References de Klerk, E., van der Heijde, D., Landewé, R., van der Tempel, H., & van der Linden, S. (2003). The compliance-questionnaire-rheumatology compared with electronic medication event monitoring: a validation study. The Journal of Rheumatology, 30, 2469-2475. de Klerk, E., van der Heijde, D., van der Tempel, H., & van der Linden, S. (1999). Development of a questionnaire to investigate patient compliance with antirheumatic drug therapy. The Journal of Rheumatology, 26, 2635–2641. Fialko, L., Garety, P.A., Kuipers, E., Dunn, G., Bebbington, P.E., Fowler, D., & Freeman, D. (2008). A large-scale validation study of the Medication Adherence Rating Scale (MARS). Schizophrenia Research,100, 53-59. Disclosure of Interest None declared

early intervention in the treatment of rheumatoid arthritis: focus on tocilizumab

Tocilizumab is a fully humanized monoclonal antibody against interleukin-6 receptors that was approved for the treatment of patients with rheumatoid arthritis (RA). Several lines of evidence, obtained both from conventional disease-modifying anti-rheumatic drugs (DMARDs) and tumor necrosis factor (TNF) inhibitors, have supported the concept of “window of opportunity” as showing that these therapies consistently work better in early disease as compared to established RA. This review addresses the question of whether a window of opportunity gained with conventional DMARDs and TNF inhibitors can also be achieved with tocilizumab. To this end, data regarding the use of tocilizumab in early RA patients are summarized. Currently available data suggest that the earlier the treatment with tocilizumab, the better the clinical outcome can be, which may have implications for various aspects of RA treatment strategies.

Radiological Followup of the Evolution of Inflammatory Process in Sacroiliac Joint with Magnetic Resonance Imaging: A Case with Pyogenic Sacroiliitis

Pyogenic sacroiliitis (PS) is an acute form of sacroiliitis that mostly starts with very painful buttock pain. Here in this case, the followup magnetic resonance (MR) images of a 49-year-old male patient with PS is displayed. After his sacroiliitis was documented by MR images, he was treated with the combination of rifampicin plus streptomycin and moxifloxacin. Serial MR investigations were done to disclose acute and subsequent imaging changes concerning sacroiliac joint and surrounding bone structures. Although after treatment all the symptoms were completely resolved, 20 months later changes suggesting active sacroiliitis on MR images were continuing.

Apparent diffusion coefficients of sacroiliitis in patients with established ankylosing spondylitis

AIM To compare apparent diffusion coefficients (ADCs) of sacroiliac joints (SIJs) in ankylosing spondylitis (AS) patients during advanced-active and advanced-nonactive stages. MATERIALS-METHODS AS patients with chronic-active (n=19), chronic-nonactive (n=6), and controls with normal SIJs (n=8) were included. Mean ADCs through 43 subchondral bone marrow edema lesions (SBMELs) were calculated. RESULTS Mean ADCs were 1.60+/-0.32 × 10-3 mm(2)/s over SBMELs, 0.57+/-0.23 × 10-3 mm(2)/s at periphery of SBMELs, 0.57+/-0.24 × 10-3 mm(2)/s in chronic-nonactive group, and 0.61+/-0.19 × 10-3 mm(2)/s for controls. CONCLUSION ADCs lower than 0.69 × 10-3 mm(2)/s, obtained at subchondral aspect of SIJs of established AS patients with chronic changes, which this number represents the receiver operating characteristic (ROC) best cutoff value, can be considered as normal without possible residual inflammation of concern.

Ulnar artery aneurysm in a patient with Behçet’s disease

Behçet’s disease is a systemic disease characterized by oral aphthosis, genital ulcers, ocular lesions, gastrointestinal, musculoskeletal, neurological and major vessel involvement. Arterial involvement, aneurysms and arterial thrombosis have been reported in 1.5–3% of patients. In this case report, we present a patient with ulnar arterial aneurysm associated with Behçet’s disease.