Mehmet Ali Balcı

The incidence and prevalence of systemic lupus erythematosus in Thrace, 2003-2014: A 12-year epidemiological study.

Background We estimated the prevalence and incidence, clinical features, treatment, and prognosis of systemic lupus erythematosus (SLE) patients in the Thrace region of Turkey. Methods We retrospectively evaluated 331 patients (307 female, 24 male, mean age 38.5 years) diagnosed with SLE between 2003 and 2014. Clinical features, treatments, and response to various treatment modalities were recorded. Our hospital has been the only tertiary referral center for rheumatological diseases for a mixed rural and urban population of 620,477 people (306,036 females, 314,411 males) for more than 16 years. Results The mean annual incidence of SLE was 4.44/100,000 (females, 8.4/100,000; males, 0.6/100,000). The overall prevalence of SLE was 51.7/100,000 (females, 97.7/100,000; males, 7/100,000). Major organ involvement was present in the following percentages: neurologic involvement: 20.1%; renal involvement: 28.2%; autoimmune hemolytic anemia: 9.6%; thrombocytopenia: 14.7%. Seventeeen SLE patients (13 females, four males) died at a median follow-up of 48 months. The five-year survival was 94.5%, and the ten-year survival was 89.9%. According to Kaplan–Meier survival analysis, poor prognostic factors were: male gender (p = 0.015); smoking (p = 0.02); pleural involvement (p = 0.011); thrombocytopenia (p = 0.021); myocarditis (p = 0.028); renal involvement (p = 0.037); treatment with cyclophosphamide (p = 0.011); and an initial high SLEDAI score (>4) (p = 0.02). Lymphopenia at the time of diagnosis appeared as a favorable prognostic factor (p = 0.008). Cox regression analysis revealed myocarditis (OR: 20.4, p = 0.018) and age at diagnosis (OR: 1.11, p = 0.035) to be poor, and lymphopenia at the time of diagnosis to be good prognostic factors (OR:0.13, p = 0.031). Conclusions The annual incidence and prevalence of SLE in the Thrace region of Turkey is lower than those reported in North America, however they are similar to those reported for European countries. Clinical manifestations appear to be milder, whereas survival was similar to those recorded in Western countries.

A Nationwide Experience With The Off‐label Use of Interleukin‐1 Targeting Treatment in Familial Mediterranean Fever Patients

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.

Development of primary central nervous system lymphoma in a systemic lupus erythematosus patient after treatment with mycophenolate mofetil and review of the literature

Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma and four cases of PCNSL have previously been described in association with mycophenolate mofetil. We report the fifth case of PCNSL in a patient with lupus nephropathy while on mycophenolate mofetil treatment.

The epidemiology of dermatomyositis in northwestern Thrace region in Turkey: epidemiology of dermatomyositis in Turkey

Dermatomyositis (DM) is a rare disease that may affect the skeletal muscles and the skin. Literature data on its incidence and prevalence are limited. There are no data on its incidence or prevalence in Turkey. Patients diagnosed with DM at the Trakya University Medical Faculty, Department of Rheumatology from November 2004 to November 2014 were reviewed retrospectively. Patients’ clinical and demographic features, laboratory data, treatment modalities, follow-up durations, disease courses, outcomes, and complications were evaluated. Our study included 23 patients with DM; 14 were females and 9 were males (female/male: 1.55). Over the course of the study, the annual incidence of DM was 3.7 per million (95% CI 0–18.8) person years, and the overall prevalence was 32.2 per million (95% CI 18.1–46.3). Incidence in women was higher (4.6/1,000,000 person years) compared to men (2.9/1,000,000 person years). The frequencies of most common findings were as follows: heliotrope rash (82.6%), Gottron papules (87%), proximal myopathy (78.3%), and facial erythema (60.9%). In our hospital-based study, the frequency of DM was lower than those reported in North America; however, they were similar to European countries.

Development of systemic sclerosis in a patient with common variable immunodeficiency

Common variable immunodeficiency (CVID) is an immune disease which is characterized by disruption in B-cell differentiation and hypogammaglobulinemia. This disease may cause various clinical manifestations such as recurrent infections, chronic pulmonary and gastrointestinal diseases. Furthermore, autoimmune diseases often accompany CVID and almost in one fourth of CVID patients autoimmunity symptoms can be detected. [1]. The autoimmune diseases that often accompany CVID are autoimmune hemolytic anemia [1], immune thrombocytopenia [2] and rheumatoid arthritis [3]. However, when literature reviewed, therewas no CVID case associatedwith systemic sclerosis (SSc). SSc is a chronic autoimmune multisystem disease characterized by fibrosis. The relation between SSc and primary immunodefiencies is not well-known. We previously reported on the first patient with SSc and X-linked agammaglobulinemia [4]. Here, we present our patient with SSc and CVID. A 53-year-old female patient was admitted with the complaints of thickening of skin over the hands and face, and Raynauds phenomenon. Physical examination showed sclerodactyly on hands, thickening and telangiectasias of the face. Laboratory analysis was as follows: hemoglobin, 13 g/dl; hematocrit, 36.9%; leucocytes, 9300/mm; neutrophils, 7500/mm; platelets, 417,000/mm; CRP, 15.3 mg/dl; erythrocyte sedimentation rate, 57 mm/h, creatinine, 0.9 mg/dl, ALT, 31 U/l. While antinuclear antibodywas 1/1280 positive, anti-centromerewas determined positive; rheumatoid factor, anti-CCP, ANCA tests, hepatitis B and C serologies were negative. In pulmonary function tests; forced vital capacity was as 51%; forced expiratory volume 1 was as 36%; DLCO was as 60%. In the echocardiography, the left ventricular type 1 diastolic dysfunction was detected and pulmonary arterial pressure were measured as 70 mm Hg. Upon the diagnosis of SSc; nifedipine, low dose aspirin, bosentan and methotrexate weekly were started. During follow-up, it was learned that the patient had frequently recurrent fever, cough and dyspnea and these complaints had been recurring approximately for 20 years. In the physical examination, besides SSc findings, there were bilateral rales in the low and middle zone of lungs. In chest x-ray, prominence of the bronchial prospect in the parenchyma of both lungs was recorded. In the high-resolution CT, no evidence for interstitial lung disease was determined, on the other hand, common tubular bronchiectasis was detected on both parenchyma of the lungs. In the sputum culture; reproduction of Moraxella catarrhalis was detected and intravenous ampicillin–sulbactam was started. In the follow-up period, severe pulmonary infection which required hospitalization occurred 3–4 times a year. Except Moraxella; including Haemophilus influenzae and Streptococcus pneumoniae grew multiple

SAT0416 The Epidemiological and Clinical Features of Systemic Lupus Erythematosus Patients Followed up at a Single Center in Northwestern Turkey

Background Prevalence of systemic lupus erythematosus (SLE) is an important measure for monitoring the disease, for investigating differences across populations. Objectives We evaluated the clinical features, treatment modalities, treatment responses and prognosis of SLE patients diagnosed at our center in Thrace region of Turkey. We also estimated prevalence and incidence of SLE in our region. Methods We retrospectively evaluated 331 patients (304F, 27M, mean age: 38.5±12.3 years) diagnosed with SLE between 2003-2014. Clinical features, treatments and responses to various treatment modalities were recorded. Our hospital has been the only tertiary referral center for rheumatological diseases for a mixed rural and urban population of 620477 people for >16 years (306036 females, 314411 males). Results Of 331 SLE patients, 307 (92.7%) were females and 24 (7.3%) were males (female/male: 12.8). During the study period, the mean annual incidence rate of SLE was 4.44/100,000 (95%CI: 2.78-6.1). The mean annual incidence of SLE in women was 8.4/100,000 (95%CI: 5.2-11.6), and in men it was 0.64/100,000 (95%CI: 0-1.52). By November 2013, the overall prevalence of SLE in our region was 51.7/100,000 (95%CI: 46-57.4) in population aged >16 years. The prevalence in women (97.7/100,000, 95%CI: 86.6-108.8) was higher than the prevalence in men (7/100,000, 95%CI: 4.1-9.9). Major organ involvement was present in following percentages: renal involvement, 28.4%; neurologic involvement, 18.7%; thrombocytopenia, 15.1%; and autoimmune hemolytic anemia, 13.9%. Seventeen SLE patients (14 females, 3 males) died at a median follow-up of 48 months (2-278 months). The 5-year survival was 94.5%, and the 10-year survival was 89.9%. According to Kaplan-Meier survival analysis, poor prognostic factors were being male (10-year: 80.9% vs. 90.6%, p=0.05); smoking (10 year, 78.2% vs. 94.1, p=0.02); thrombocytopenia (10-year: 76.9% vs. 93.2%, p=0.021), pleural involvement (10-year: 74.9% vs. 93.4%, p=0.011); renal involvement (10-year: 85.6% vs. 92.9%, p=0.037); myocarditis (10-year: 71.4% vs 91.1, p=0.028); an initial SLEDAI score >4 (10-year: 85.8% vs 93.8%, p=0.02) and the usage of cyclophosphamide (10-year: 81.7% vs. 94.7%, p=0.011). Lymphopenia at the time of diagnosis seemed to be a good prognostic factor according to Kaplan-Meier analysis (10-year: 94.6% vs. 80.6%, p=0.008). According to multivariate Cox regression analysis, myocarditis (OR: 20.4, 95%CI: 1.9-200, p=0.12), and age at diagnosis (OR: 1.11, 95%CI: 1.008-1.23, p=0.035) were independently poor prognostic parameters; lymphopenia at the time of diagnosis (OR: 0.13, 95%CI: 0.02-0.82) was a good independent prognostic factor. Conclusions Our study demonstrated that the annual incidence and prevalence of SLE in Thrace region of Turkey were lower than in North America, however, they were similar to European data. Survival was similar to data from western countries. Disclosure of Interest None declared

AB0914 Management of Gout in Different Clinical Specialties in Turkey

Background In contrast to many European countries, patients can easily admit to secondary and tertiary centers without referral by the primary care in Turkey. Therefore we also compared the management options for gout preferred in different clinical specialties. Objectives In this study we investigated how gout is treated in Turkey. Methods 319 consecutive patients were included in this multicenter study (mean age 58.60±12.8 years, 44 females, 272 males). All patients filled a standard questionnaire. Results 53 patients were first admitted to primary care (16.6%), 101 patients to orthopedics (31.7%), 29 patients to physical therapy and rehabilitation (9.1%), 70 patients to internal medicine (21.9%), 49 patients to rheumatology departments (15.4%), and 17 patients to other clinical specialties (5.3%). Among those 313 patients admitting to health care with acute gout attack, 40 patients were referred the patient to another center without any treatment (12.8%). Referral rate remarkably higher in the primare care (%28.8). NSAIDs were the most common drugs prescribed for acute attack (60.06%), followed by colchicine (58.15). Allopurinol was given in 12.8%, and steroids in 7.99% of patients during acute attack. Regarding long term treatment, 92 patients had never been treated with allopurinol (28.8%). 29.1% (37/127) patients having less than 2 attacks per year and 28.6% (55/192) of patients having two or more attacks per year had never been treated with allopurinol (p>0.05). Only 89 patients (27.9%) were treated with allopurinol by their first physicians, and 138 patients (43.3%) were treated with allopurinol later in a different specialty. Prescription of allopurinol was more common among the rheumatologists. Diet and life style modifications were recommended in 118 of the patients (37%) by their first physicians. 171 patients (53.6%) were later recommended diet and life style modifications during their follow up in a different clinical specialty (total 289 patients, 90.6%). Diet and life style modifications were recommended more commonly in rheumatology (41 patients, 83.7%). 183 (57.4%) were treated with colchicine by their first physicians, and 114 patients (35.7%) were treated with colchicine by a physician of different specialty (total 297 patients, 93.1%). Significantly more patients were treated with colchicine than with allopurinol during long term management (p<0.001). Conclusions Treatment of gout appears suboptimal in primary care, and more than 25% of patients were referred without any treatment. Long term management also appears suboptimal in both primary care and among some specialists such as orthopedics. Only a minority of patients were recommended allopurinol and/or life style modifications by the doctors first diagnosed gout. Although long term treatment appears better among rheumatologist, still a considerable number of patients were not recommended uric acid lowering approaches in the rheumatology clinics. Disclosure of Interest None declared