The Increased Frequency of Familial Mediterranean Fever in Patients Diagnosed with Immunoglobulin a Vasculitis (Henoch-Schönlein Purpura)

Abstract

GIRIS ve AMAC: Immunoglobulin A vaskuliti cocukluk caginin en sik vaskulitidir. Trombositopenik olmayan purpura, karin agrisi, gastrointestinal kanama, artrit veya artralji ve nefrit ile karakterizedir. Ailevi Akdeniz atesi (AAA) ise en sik gorulen otoinflamatuvar hastalik olup, tekrarlayan ates ve serozit ataklari hastaligin temel ozellikleridir. Calismamizda klinigimizde IgAV/HSP tanisi ile takipli olan olgularin genel ozelliklerini, MEFV mutasyon sikligini tespit etmeyi, AAA+IgAV/HSP olan olgularla sadece IgAV/HSP olan olgularin klinik, laboratuvar ve tedavi ozelliklerini karsilastirmayi amacladik. GEREC ve YONTEMLER: Calismaya 2014-2016 yillari arasinda IgAV/HSP tanisi almis olan 159 olgudan MEFV analizi yapilmis olan 114 olgu dâhil edildi. IgAV/HSP tanisi ile takipli olgularin geriye donuk olarak MEFV mutasyon sonuclari, demografik, klinik ozellikleri ve tani anindaki laboratuvar bulgulari kaydedildi. Ayrica IgAV/HSP+AAA tanisi olan hastalar ile sadece IgAV/HSP olan hastalar karsilastirildi. BULGULAR: 88 olguda MEFV mutasyonu tespit edilmedi. 26 (%22,9) olgunun ise 9’u M694V/- (%7,9), 6’si M694V/M694V (%5,3), 3’u M680I/- (%2,6),3’u V726A/- (%2,6), 2’si E148Q/- (%1,8), biri M694V/M680I (%0,9), A744S/- (%0,9), biri ise R761H/- (%0,9) idi. 9 olguda (%7,9) AAA tanisi mevcuttu. IgAV/HSP+AAA hastalarinda medyan CRP degerinin ve nonsteroidal anti-inflamatuvar ilac (NSAII) ve kolsisin tedavisi alma oraninin daha yuksek oldugu saptandi. TARTISMA ve SONUC: Calismamizda IgAV/HSP+AAA olan olgularda, IgAV/HSP surecinde hastalarin CRP degerlerinin daha yuksek ve NSAII ihtiyacinin daha sik olabilecegini gozlemledik. IgAV/HSP tanisi ile takipli olan olgularda MEFV mutasyon varliginin gosterilmesi erken tani ve etkin inflamasyon kontrolu ile olasi komplikasyonlarin onune gecilmesine yardimci olabilir. ABSTRACT INTRODUCTION: Immunoglobulin A vasculitis/Henoch Schonlein purpura (IgAV/HSP) is the most common vasculitis in childhood. It is characterized by non-thrombocytopenic purpura, abdominal pain, gastrointestinal bleeding, arthritis or arthralgia and nephritis. Familial Mediterranean fever (FMF), is the most common autoinflammatory disease, manifesting with recurrent fever episodes and serositis. In this study, we aimed to determine the general characteristics and MEFV (Mediterranean FeVer) mutation occurrence rate in patients diagnosed with IgAV/HSP and to compare the clinical, laboratory and treatment characteristics of cases with FMF+IgAV/HSP and cases with only IgAV/HSP. METHODS: A total of 114 cases analyzed for MEFV out of 159 cases diagnosed with IgAV/HSP between the years 2014-2016 were included in the study. MEFV gene analysis, demographic, clinical, and laboratory findings were retrospectively recorded. In addition, patients with AAA and only IgAV / HSP cases were compared. RESULTS: The MEFV mutation was not detected in 88 cases. 9 of the 26 (22.9%) cases were M694V/- (7.9%), 6 were M694V/M694V (5.3%), 3 were M680I/- (2.6%), 3 were V726A/- (2.6%), 2 were E148Q/- (1.8%), one was M694V/M680I (0.9%) and one was R761H/- (0.9%). 9 cases (7.9%) were diagnosed with FMF. The median C-reactive protein (CRP) value was higher in IgAV/HSP+FMF patients and they had a higher rate of using nonsteroidal anti-inflammatory drugs (NSAIDs) and colchicine treatment. DISCUSSION and CONCLUSION: Patients with IgAV/HSP+FMF had higher CRP levels and needed NSAIDs more frequently during the course of IgAV/HSP. Demonstrating the presence of the MEFV mutation in cases diagnosed with IgAV/HSP may help to prevent possible complications through early diagnosis and effective control of inflammation. Key words: Immunoglobulin A vasculitis / Henoch Schonlein purpura; familial Mediterranean fever;