Abstract:

Family Mediterranean Fever (AAA) is a common one with repeated fever, serositis, arthritis attacks, auto-inflammatory diseases. In addition to the classic findings, the increasing rate and variety of accompanying diseases are seen together. The purpose of our study is to investigate the diseases accompanied by AAA-known children's patients who apply to our children's romatology center. Method and means: The study followed with 586 diagnosed AAAs of 107 patients with accompanying disease. Patient files recorded age, AAA diagnosis age, clinical characteristics, accompanying disease diagnosis ages, application clinical and laboratory characteristics and treatments. The average age of patients was 16 (IQR: 11.5-18) years and the diagnosed age of AAA was 7 (IQR: 4-10) years. The average diagnosed age of the first associated disease was 10 (IQR:7-14), the second 11 (IQR:7-15), and the third 8 (IQR:8-9) years. 96 of the patients (89.71) had exon 10 mutations. IgA vasculitis 9(%1.53), Behcet disease 2(%0.34), polyarthritis nodosa (PAN) 1(%0.17), cutanous PAN 2(%0.34), urticeral vasculitis 1(%0.17), oligoarticular juvenile idiopathic arthritis(JIA) 6(%1.02), polyarthric JIA 3(%0.51), EIA 37(%6.31), chronic non-infectious osteomyelitis 7(%1.19), inflammatory intestinal disease 3(%0.51), resistant AAA 33(%5.63), amyloidosis 4(%0.68), acute romatismic fever 3(%0.51), PAPA 4(%0.86); polyarticular JIA 3(%0.51), EIA 37(%6.31), chronic non-infectious osteomyelitis 7(%1.19), inflammatory intestinal disease 3(%0.51), resistant AAA 33(%5.63), amyloidosis 4(%068), acute The result: the systematic surveys and examinations of the patients followed by the AAA diagnosis should be carefully carried out in terms of attack conditions, medicinal compatibility and accompanying diseases.