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 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%. Seventeen 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.

Epidemiology and outcome of adult-onset Still's disease in Northwestern Thrace region in Turkey.

OBJECTIVES: Adult-onset Still's disease (AOSD) is a rare disease that is classified among the multifactorial autoinflammatory disorders. It is characterised by fever, arthritis and, a typical salmon-coloured rash, and is accompanied by fever at nights. Currently, there is limited data on the prevalence of AOSD. METHODS: Patients diagnosed with AOSD at the Department of Rheumatology of Trakya University Medical Faculty, between 2003 to 2014 were reviewed retrospectively. Patients' clinical features, laboratory measurements, demographics, treatments, follow-up durations, disease courses, outcomes and complications were evaluated. RESULTS: Our study included 42 patients with AOSD of whom, 32 (76.2%) were females and 10 (23.8%) were males (female to male ratio: 3.2). Over the course of the study, the annual incidence of AOSD was 0.62/100,000; and the overall prevalence was 6.77/100,000. The most common findings were fever (97.6%), arthralgia (95.2%), arthritis (76.2%), rash (73.8%) and sore throat (40.5%). CONCLUSIONS: In our hospital-based study on AOSD which is a disease with very limited epidemiological data, the frequency of AOSD was found to be significantly higher than in other series. Female gender was more common in our series; and polycyclic pattern was more common in patients with longer follow-ups.