Data from two centers in Northwestern Turkey show that the mortality rate for SLE is similar to the rate in Western countries.
In this retrospective study, we evaluated the clinical features and the effects of various treatment modalities on the clinical course in patients diagnosed with idiopathic thrombocytopenic purpura (ITP) at our center between 1984-2000. We retrospectively examined the medical records of 321 (229 females, 92 males) ITP patients. One hundred and seventy-one (53.3%) patients were lost to follow-up. When evaluating the clinical features, all 321 patients were included; however, when the response to treatment modalities was evaluated only 150 patients followed up regularly were considered. The median age of the patients on initial diagnosis was 34 years (range: 14-78). At initial diagnosis, 235 (73.2%) patients had signs of bleeding. Of patients diagnosed with ITP initially, six later turned out to have systemic lupus erythematosus (SLE) and two myelodysplastic syndrome (MDS). The median follow-up of 150 patients followed up regularly was 30 months (range: 4-396). One hundred and thirty-seven of these subjects had an indication for treatment and 94.2% of them were administered either standard or high-dose steroids as the first-line therapy. Complete remission (CR) was defined as any platelet count >100,000/mm(3) lasting for 3 months or longer without treatment. CR was achieved in 51.9% of the patients given steroids as the initial therapy. During a median follow-up of 33 months, relapse occurred in 58.2% of these patients, and after a median follow-up of 11 months the rest of them were still in remission. Ninety-eight patients followed up regularly were administered second-line therapies. CR was obtained in 44.4% of the patients who used steroids as second-line therapy. Within a median follow-up of 15 months, 20.8% of these patients relapsed. Splenectomy was performed in 76 patients and CR was obtained in 68.4% of the regularly followed up patients. Relapse occurred within a median of 96 months in 15.4% of the patients who had CR. Kaplan-Meier curves showed that the duration of CR obtained by splenectomy was significantly higher than that obtained by steroids (p<0.001). The 10-year disease-free survivals in patients who used steroids and who underwent splenectomy were, respectively, 13% and 58%. In our adult ITP patients, steroids induced nearly similar rates of CR both as first-and second-line therapies. Splenectomy seems to be effective in patients unresponsive to steroids. The duration of CR obtained by splenectomy is significantly longer when compared with the duration of CR obtained by steroid therapy.
It is quite rare to diagnose secondary amyloidosis during the course of Castleman's disease (CD). A 51-year-old female who complained of fatigue, weight loss, and fever was diagnosed with CD -- plasma cell type -- in our hospital in 1993. One year after diagnosis, she developed nephrotic syndrome, the etiology of which was found to be secondary amyloidosis based on renal biopsy. As the patient rejected therapy, she was discharged after only symptomatic treatment. At her last follow-up in March 2001, she had no complaints; physical examination, blood chemistries, and urinalysis were normal. Abdominopelvic tomography revealed no lymphadenopathy in the abdomen, which had been previously present. We could identify 17 other cases of CD with secondary amyloidosis in the literature. Ours is the 18th such case and the 2nd case of multicentric CD leading to amyloidosis. This case also shows that CD might sometimes run a relatively benign course being cured with no therapy, whereas it might have a rapidly fatal downhill course -- even with therapy -- in others. Still, effective treatment strategies need to be developed.
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.
In spite of functional disability, FM does not cause a decrease in BMD. The presence of FM in RA patients does not result in a change in BMD.
Background Adult onset Still disease (AOSD) is a rare disease. Its management is mostly based on expert opinion. Objectives To assess clinical features and management of AOSD in a large sample of patients. Methods Patients from 19 centers in Turkey participated this in retrospective study. All the patients fulfilled the Yamagushi criteria for AOSD. A case report form which included clinical data, laboratory features, disease patterns, and remission and relapse rates and treatment protocols was generated and sent to the participating centers. Responsible investigators of all centers searched their local database for AOSD. Disease patterns were assessed in patients with a follow-up longer than 12 months. Results A total of 356 patients, (210 female, 59%) with a mean (± SD) disease onset age of 31 ± 15 years and median follow-up duration of 22 (range: 0-180) months were enrolled. Of the 356 patients, remission rate after initial therapy could be assesed in 306 (85,9%) and data regarding the last visit were available in 254 patients (71.3%). Median lead time to diagnosis was 1 (range; 0-120) month. Main clinical features were as follows: fever (96%), arthralgia (95%), rash (67%), arthritis (65%), sore throat (64%), lymphadenopathy (28%), splenomegaly (25%). Abnormal laboratory findings were leucocytosis (85%), neutrophilia >80% (68%), anemia (65%), increased AST (49%), ALT (46%) and ferritin (96.7%). Disease pattern was assessed in 230 patients [chronic 19%, relapsing-remitting 32% and monocyclic 49%]. Patients with lead time to diagnosis longer than 3 months had more frequently chronic or relapsing pattern (21.8% vs 44.3% vs 51.1%, p<0.001). Patients with chronic pattern had more joint count than those with monocyclic pattern (6.6±7.4 vs 3.1±4.2, p<0.001). Remission was achieved in 255 patients (83.3%) with the initial treatment [steroid + MTX (84/99, 87.9%), steroid + MTX + HCQ (67/80, 83.7%), medium-high dose steroid (41/54, 85.2%), steroid + HCQ (37/38, 97.3%), NSAID (4/18, 22.2%)]. Females reached remission more frequently (91% vs 72%, p<0.001). Patients under steroid treatment had a higher remission rate than those without such treatment (87.5% vs 40.7%, p<0.001). Remission could not be achieved in 51 patients (16.7%) with the initial therapy and biological therapy was given to 18 of them (35%). Relapses occurred in 74 (32%) patients during follow-up. Median duration to relapse was 18 (3-180) months. Only, 19 patients (7.5%) had active disease at the last visit. Conclusions Monocyclic pattern was observed more frequently than reported in the literature. This may be due to the aggressive treatment protocols (steroid plus MTX and/or HCQ). Chronic/relapsing pattern seems to be associated with a longer delay in diagnosis. Early diagnosis and aggressive treatment are important for the management of AOSD. Disclosure of Interest None Declared
BackgroundPrevalence of systemic lupus erythematosus (SLE) is an important measure for monitoring the disease, for investigating differences across populations.ObjectivesWe 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.MethodsWe 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).ResultsOf 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.ConclusionsOur 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 InterestNone declared
BackgroundIn 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.ObjectivesIn this study we investigated how gout is treated in Turkey.Methods319 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.Results53 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).ConclusionsTreatment 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 InterestNone declared
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