Muhammet Cinar

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.

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

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.

Cross-Cultural Adaptation, Reliability, and Validity of the Turkish Version of the Compliance Questionnaire on Rheumatology in Patients With Behçet’s Disease

Purpose: The aim of this study was to examine the psychometric properties of the Turkish versionof the Compliance Questionnaire on Rheumatology (CQR-T) for patients with Behçet’s disease (BD). Method: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. Results: The CQR-T demonstrated acceptable internal consistency (Cronbach’s α = .832), adequate test–retest reliability (intraclass correlation coefficient = .630), and correlations with Morisky Medication Adherence Scale scores (r = −.389, p< .001), indicating convergent validity. Conclusion:The CQR-T was found to be a valid and reliable instrument for evaluating the compliance of Turkish BD patients with prescribed medications. Implications for Practice: 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.

Muscle involvement in two Behçet cases: magnetic resonance imaging and histology findings

Abstract Behçet’s disease (BD) is a systemic vasculitis with a classic trio of symptoms of oral aphthous ulcers, genital ulcers, and ocular lesions that present in a relapsing fashion. Despite these most frequently encountered symptoms of the disease, other systems such as vascular, gastrointestinal, and neurological involvements can also occur. Muscular involvement is rare, and there are only a few cases in the literature, which were reported mainly in a pediatric population. In this two-adult case report, muscular involvement of BD with an emphasis on magnetic resonance imaging and histology findings will be presented.

Investigation of the arterial stiffness and associated factors in patients with familial Mediterranean fever.

Objective: Because of the ongoing and recurring inflammatory state in familial Mediterranean fever (FMF), patients may experience a high risk of cardiovascular events. Our aim was to investigate the arterial stiffness and associated factors in patients with FMF. Methods: Sixty-nine consecutive FMF patients (including 11 females) and 35 controls (including 5 females) were enrolled in the study. The demographical, clinical, and laboratory data and genetic mutations of the patients were recorded. In the study, FMF patients according to the Tel-Hashomer criteria were included, whereas patients with other known inflammatory rheumatologic disease, atherosclerotic cardiovascular disease, hypertension, diabetes, those under the age of 18 years, or those refusing to participate in the study were excluded. Arterial stiffness measurements were performed using the TensioMed device (TensoMed Ltd, Budapest, Hungary). Results: The patient and control groups were similar in terms of the mean ages, BMIs, gender, systolic blood pressures, and smoking. FMF patients had a higher pulse wave velocity (PWV) (7.73±1.3 and 7.18±1.1 m/s; p=0.03) and lower brachial and aortic augmentation indexes (–64.6±14.6% and –54.6±25.9%, p=0.041 and 4.9±7.4% and 14.0±11.5%, p=0.025, respectively) compared with the controls. Thirty-one (45%) patients were in the “during-attack” state and had higher PWV (8.17±1.6 and 7.38±0.9 m/s; p=0.027) compared with the asymptomatic patients. PWV was correlated to serum CRP, WBC, ESR, fibrinogen, and neutrophil/lymphocyte ratios (r=0.666, 0.429, 0.441, 0.388, and 0.460, respectively). The genetic mutation and predominant attack type had no effect on arterial stiffness. Conclusion: FMF patients have increased arterial stiffness during attacks compared with asymptomatic patients and controls. The impaired arterial stiffness is correlated to the severity of the inflammatory state rather than to the attack type or genetic mutations.

Long-term efficacy and safety of Anakinra in a patient with liver transplant due to Adult onset Still's Disease

to control his symptoms, and the patient was hospitalized. At the time of hospitalization, his fever was 39 ° C. Multiple evanescent skin rashes were detected at his arms and trunk; and splenomegaly and arthritis in the metacarpophalengeal, proximal interphalengeal, and wrist joints were present. His laboratory results show the following abnormalities: leukocytosis with left shift (43 300/mm 3 , with 90% neutrophils), anemia (hemoglobin: 8.6 gr/dL), and thrombocytosis (830 000/mm 3 ). The blood biochemistry was normal, with the exception of hypoalbuminemia. The liver function tests were within normal limits, and actually never been found as increased from the time of the transplantation. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were markedly elevated (105 mm/h and 146,1 mg/ dL, respectively). Serum ferritin level was found to be increased (5160 ng/ml, normal range 22 – 322 ng/ml). Although these fi ndings were all highly suggestive for AOSD, we reassessed the patient for any underlying condition such as infections, other rheumatic diseases, or malignancies. We have obtained blood, sputum, pharynx swab, and urine cultures on three consecutive days and all were negative. The immunologic markers including anti-nuclear antibody and rheumatoid factor were studied and found as negative. Given the possible triggering role in AOSD, we have studied the viral markers including Epstein – Barr virus, varicella zoster virus, hepatitis B and C, parvovirus B19, and cytomegalovirus, and none of them was positive. After confi rming the diagnosis, to rapidly control the disease activity, methyl prednisolone pulse 1000 mg/day for 3 days was administered, and the dosage of oral prednisolone was increased to 1.5 mg/kg/ day. After transient relief maintained for about 1 week, all clinical and laboratory signs of disease activation reoccurred. Since he is a liver recipient, hepatotoxic drugs such as methotrexate and lefl unomide were avoided initially. However, because of the lack of information regarding the optimal treatment of a transplant patient experiencing AOSD reactivation, it was then decided to give methotrexate as 7.5 mg/week with close monitoring. The dose was titrated up to 12.5 mg/week, and used for 3 months with no response. At the third month of his hospitalization, the only treatment controlling his symptoms was pulse methyl prednisolone 1000 mg/day, which had to be given almost weekly. As expected, adverse events associated with the use of Mod Rheumatol, 2014; 24(6): 1030–1031

THU0378 Tocilizumab in the Treatment of Patients with AA Amyloidosis Secondary to Familial Mediterranean Fever: CASE Series

Background The most frequent underlying diseases responsible for AA amyloidosis worldwide are rheumatoid arthritis, juvenile idiopathic arthritis and ankylosing spondylitis, while familial Mediterranean fever (FMF) is responsible for almost 60% of the cases in Turkey and in countries where the FMF is prevalent. Tocilizumab (TCZ), an IL-6 antagonist, seems to be a promising agent in AA amyloidosis associated with rheumatic disorders, while there is no data regarding its use in amyloidosis due to FMF. Objectives We herein describe the short-term results of TCZ treatment in 8 patients with amyloidosis secondary to FMF. Methods We described a series of adult FMF patients complicated with amyloidosis and treated with TCZ (8 mg/kg once monthly) in one reference center. Diagnosis of AA amyloidosis was confirmed in the renal and/or rectal biopsy specimens in all patients with Congo red and immunohistochemical staining. Results The longest duration on the treatment with TCZ was 16 months in one patient, while 3 months in one patients with the shortest follow-up. At the beginning of TCZ treatment all patients had proteinuria with nephrotic range except case 4. Following treatment with TCZ, proteinuria was normalized in case 1 (Case with the longest duration of treatment), and considerably decreased in 4 cases, and increased in two (case 4 and 5). Serum creatine levels also increased in case 5, and patient refused to receive treatment after 6 cycles. In case 8, TCZ treatment was switched to anakinra due to frequent FMF attacks. The renal function tests were abnormal in 4 patients, and remained almost stable throughout the follow-up (Table 1). Serum albumin levels were normalized in all patients except case 5 and 8. Table 1. Demographic, clinical and laboratory features of study patients Cases Age MEFV gene mutation Treatment cycles Initial proteinuria (mg/24 hours) Final proteinuria (mg/24 hours) Initial albumin Final albumin Initial creatine Final creatine 1 32 M694V/ M694V 16 6810 84 2.8 4.5 0,82 0.64 2 20 M694V/ M680I 8 10485 3920 1.5 3.84 0,62 1,03 3 75 M694V/ M680I 11 4368 961 2.33 4.2 3,21 2.95 4 45 M694V/ M694V 6 1800 2520 3.73 3.94 1,32 1,46 5 38 M694V/ M694V 6 5648 6320 3.29 3.17 1,62 2.07 6 33 – 10 11960 5054 2.03 3.35 0,67 0.80 7 23 M694V/ M694V 7 29260 5670 1.4 3.79 4,06 2.87 8 22 M694V/ M694V 3 10850 10375 1.26 1.27 0.72 0.64 ND, not done. Conclusions To our knowledge this is the first report showing the efficacy of TCZ on renal amyloidosis secondary to FMF. While previous reports confirm the efficacy of TCZ in amyloidosis secondary to other rheumatic diseases, our findings suggest that this effect can be extended to other diseases. Disclosure of Interest : None declared DOI 10.1136/annrheumdis-2014-eular.4058

Lipid Storage Myopathy in Behçet's Disease: A Rare Cause of Elevated Serum Creatine Kinases Levels

Muscular involvement in Behçets disease is rare and there are only a few case reports in the literature. The causes of elevated muscle enzymes in a patient with Behcets disease are many, including myositis, drug-induced myopathy, metabolic myopathy, and the disease itself. We herein have defined an algorithmic approach to a patient with Behcets disease and elevated muscle enzymes and report a case of coexisting of lipid storage myopathy.