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Results A 15-month-old male patient was referred to pediatric rheumatology for evaluation of non-pruritic skin eruption most prominent on arm and leg, fever and lymphadenopathy. These symptoms began when the patient was four months old. Several antibiotics therapies had been examined. The maximal convelescence period was a month. There were no consanguinity and family history. Physical examination showed generalized, maculopapulary eruption especially on arm and leg, lymph node on left cervical and axillary region, and persistant fever. In laboratory examinations, acute phase reactans were found to be high. The serology of EBV, CMV, toxoplasma, brucella, tularemia, bartonella henselae, and Quantiferron test results were negative. Immunologic evaluations contained hemogram, blood smear, immunoglobulins, fagotest, and CD panel were normal. Urine calcium/creatinin ratio, serum ACE levels were normal. A skin biopsy taken from a papule showed subepidermal nonlangerhas histiocytes and a non-caseating granuloma. A lymph node biopsy showed granulomatous inflammation. Mediastinal lymph node was absent on thorax CT evaluation. His ophtalmological examination was normal. The granulomatous autoinflammatory disease was diagnosed. Prednisolone 2 mg/kg was administered which reduced symptoms and patient’s complaints. Then, the steroid dose was dropped. The fever and rash were absent, but the acute phase reactans were still high. His blood samples were referred for genetic testing NOD2 mutations. Methotrexate was added to the treatment, but the flair was occured two mounths later. NOD2 mutations was detected to be P268S heterozigot and V955I heterozigot. Since the patient’s response to anakinra was poor, TNF bloker was later added to the treatment. Conclusion Granulomatous autoinflammatory diseases include Blau syndrome and early-onset sarcoidosis, both are caused by mutations in the NOD2/CARD15 gene, inherited autosomal dominant and sporodic form of disease, respectively. Clinical triads are uveitis, arthritis and granulomatous dermatitis whereas the symptops we observed were fever, granulomatous dermatitis, and lymphadenopathy.

Objective The optimal level of adherence necessary to achieve acceptable disease and quality-of-life outcomes for patients is not known. In order to identify these optimal levels, we need reliable and valid measures of adherence. Medication Adherence Scale in FMF (MASIF) is an instrument designed to measure adherence to treatment in children with Familial Mediterranean Fever (FMF). We have developed this scale for children with FMF and found valid and reliable. In this study, it was aimed to assess the validity and reliability of this adherence scale for medical treatment in adult FMF patients.

The patients characterized with chronic subclinical inflammation even during attack-free periods, are now considered to have an increased risk of atherosclerotic complications as well as other autoinflammatory disease. Damage to the arterial wall due to atherosclerosis causes increased arterial stiffness. Pulse wave velocity (PWV), a noninvasive measure of arterial stiffness, is accepted to be an indicator of subclinical atherosclerosis. Cardiovascular disease included various risk markers; blood biomarkers and genetic markers. Matrix metalloproteinases (MMPs) are closely related proteinases that together are able to degrade all macromolecules of the extracellular matrix. MMPs are potentially implicated in atherogenesis, progression of atherosclerosis. The gene encoding MMP-3 is polymorphic and an insertion (6A)/deletion (5A) polymorphism (5A/6A polymorphism) in the MMP-3 gene may have functional significance in the regulation of its expression. The 5A allele was associated with higher and the 6A allele with lower transcriptional activity. Up to date, the 6A/6A and 5A/6A genotypes were associated with coronary artery disease and carotid atherosclerosis in adults.