ABSTRACT

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.

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.

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.

Immunoglobulin A vasculitis/Henoch Schönlein 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.