ABSTRACT BACKGROUND: Up to 5% of familial Mediterranean fever (FMF) cases are unresponsive to colchicine, through resistance, side effects and toxicity. Anakinra is an alternative treatment for FMF patients whose disease remains uncontrolled with colchicine. We aimed to evaluate anti-interleukin-1 treatment regarding clinical findings, laboratory parameters and quality of life (QoL) among FMF patients presenting resistance and toxicity towards colchicine. DESIGN AND SETTING: Descriptive observational study at the rheumatology clinic, Adnan Menderes University Medical School, Aydın, Turkey. METHODS: Among the patients included, age, sex, MEFV genotypes, acute-phase reactants, hepatic/renal function tests, average colchicine dose, disease duration, attack frequency, attack duration, disease severity, proteinuria, amyloidosis and QoL were evaluated. Colchicine resistance was defined as > 6 typical episodes/year or > 3 per 4-6 months. Kolmogorov-Smirnov, Friedman and two-way analysis of variance tests were used for statistical analyses. RESULTS: Between 2015 and 2017, 14 FMF patients receiving anakinra were enrolled. The mean colchicine dose was 1.7 ± 0.3 mg/day before use of anakinra. Ten patients were attack-free after treatment, while three showed reductions of at least 50% in attack frequency, attack duration and disease severity. Proteinuria levels in all patients with renal amyloidosis decreased after treatment. QoL among patients with renal amyloidosis differed significantly from QoL among non-amyloidosis patients. Mean visual analogue scale scores significantly improved in both groups after use of anakinra. CONCLUSIONS: Use of anakinra reduced attack frequency and proteinuria and acute-phase reactant levels, and improved QoL, with only a few uncomplicated side effects among colchicine-resistant or intolerant FMF patients. Injection-site reactions of severity insufficient to require discontinuation of treatment were seen.

Resumo A primeira International Chapel Hill Consensus Conference (CHHC) ocorreu em 1994. Fizeram-se sugestões sobre a nomenclatura das vasculites sistêmicas. Na segunda CHHC, 2012, adicionaram-se importantes categorias à classificação da vasculite e fizeram-se várias mudanças em nomes de doenças que não estavam incluídas na CHCC 1994. Introduziu-se uma nova nomenclatura em vez de se usarem nomes como Churg-Strauss e Wegener. Também foram adicionadas novas categorias, como de Behçet e Cogan etc. Essas pessoas são homenageadas pela classificação. Elas contribuíram para a ciência com seus estudos de caso, artigos científicos e observações. Este artigo analisa apenas epônimos presentes na classificação atual das vasculites. O objetivo é prestar informações sobre os cientistas mencionados na classificação das vasculites.

Abstract The first International Chapel Hill Consensus Conference was held in 1994. There have been suggestions about the nomenclature of systemic vasculitis. Important categories were added to the classification of vasculitis, and many changes were made for disease names in the second Chapel Hill Consensus Conference 2012, which were not included in the Chapel Hill Consensus Conference 1994. The new nomenclature was introduced instead of being referred to by many names such as Churg-Strauss and Wegener"s. New categories such as Behçet"s and Cogan etc. were also added. These people are honored by the classification. They contribute to science through their case studies, scientific articles, and observations. This article reviews only eponyms present in the current classification of vasculitis. The aim of this paper is to give information about scientists mentioned in the classification of vasculitis.

Objective: CD20 expression was reported at different rates in patients with multiple myeloma. The importance of this B-cell antigen for plasma cells is still unknown. This study aimed to investigate CD20 expression of myeloma cells in bone marrow, and any relationship between the stage of disease, isotype and clinical features. Methods: Sixty-one patients who were admitted to the hematology clinic of the Adnan Menderes Medical School with the diagnosis of multiple myeloma according to the criteria of the "International Myeloma Working Group" were enrolled in this study. Age, gender, Durie-Salmon stage, history of autologous hematopoietic stem cell transplantation, and the distribution pattern and positivity of CD20 expression on multiple myeloma cells in bone marrow were evaluated. The Mann-Whitney U and chi-square tests were used for statistical analysis with a p-value < 0.05 being accepted as statistically significant. Results: Thirty patients (48.9%) had positive scores for CD20 with the distribution pattern being most likely interstitial in 55.6% of the cases. There was no statistically significant difference between immunohistochemical positivity for CD20 expression on multiple myeloma cells, immunoglobulin type, and the stage of disease. Conclusion: The combination of immunohistochemical studies with flow cytometry may reveal the importance of CD20 positivity in patients with multiple myeloma more clearly.