Is There An Overlap Of Antineutrophil Cytoplasmic Antibody-Associated Vasculitides With Igg4-Related Disease Or Not?

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Do ANCA-associated vasculitides and IgG4-related disease really overlap or not?

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and immunoglobulin G4-related disease (IgG4-RD) have some common features. The co-occurrence/concurrence of AAV and IgG4-RD was recently published by the collaborative European Vasculitis Study Group. First, we aimed to investigate ANCA positivity of our IgG4-RD cohort. Second, a literature review of co-occurrence/concurrence of AAV and IgG4-RD was done. Methods: Data of 62 patients with IgG4-RD in Hacettepe Vasculitis Center Database were used. Patient dataset was designed to include demographic data, clinical characteristics, imaging and IgG4-RD, AAV and ANCA test results. At the next step, we performed a systematic literature review in PUBMED database covering the time period from 1976 until April 2018. Relevant publications were searched using these MeSH terms ''IgG4-related disease and Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis'', "IgG4-related disease and Eosinophilic Granulomatosis with Polyangiitis", "IgG4-related disease and Microscopic Polyangiitis" and "IgG4-related disease and Granulomatosis with Polyangiitis". Results: Three (10.3%) of 29 patients had low titer ANCA positivity. These three patients didn't have any findings of vasculitis and no granuloma was seen in biopsy. In the literature review, we found 17 cases had features of both IgG4-RD and AAV. These cases were re-evaluated according to the Comprehensive Diagnostic Criteria for IgG4-RD. ANCA were positive in 15 of 17 patients (88%). Conclusion: None of our IgG4-RD patients overlapped with AAV. Only two patients in the literature review seemed to be fully compatible with both diseases. Even though AAV and IgG4-RD share similar clinical features, we think this might be a co-occurrence instead of a histopathological link.PubMe

Enteropathic Arthritis Patients Under Bdmard Treatments Had Frequently Radiographic Sacroiliitis: Hur-Bio Real Life Results

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Comparison Of Survival Rates Among Subgroups Of Patients Evaluated For Fever Of Unknown Origin

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Application Of Auto-Inflammatory Disease Damage Index (Addi) To Patients With Fmf And Related Factors With Damage

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Prevalence, Underlying Factors And Damage Associated With Chronic Persistent Inflammation In Patients With Fmf

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Clinical Features Of Aneurysmal Behcet Disease

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Aneurysm In Behcet'S Disease: Management, Prognosis And Mortality

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Colchicine intolerance in FMF patients and primary obstacles for optimal dosing

Background/aim: Colchicine is the mainstay of treatment in FMF. However, in daily practice it is not easy to maintain effective colchicine doses in a substantial number of patients due to its side effects. In this study, we aimed to investigate prevalence and risk factors for colchicine side effects that limit optimal drug dosing and cause permanent discontinuation. Materials and methods: All patients were recruited from ``FMF in Central Anatolia{''} (FiCA) cohort, 915 adults with a minimum follow-up time of 6 months during which they had obeyed all treatment instructions. Demographic and anthropometric data, FMF disease characteristics, disease severity, complications, and treatment features were recorded on a web-based registry. Prevalence of colchicine intolerance and characteristics of intolerant patients were analyzed. Results: Effective colchicine doses cannot be maintained in 172 (18.7\%) subjects. Main side effects that limit optimal dosing were as follows: diarrhea in 99 (10.8\%), elevation in transaminases in 54 (5.9\%), leukopenia in 10 (\%1.1), renal impairment in 14 (1.3\%), myopathy in five (0.5\%), and allergic skin reaction in two. Colchicine had to be permanently ceased in 18 (2\%) patients because of serious toxicity. Male sex and obesity were found to be associated with liver toxicity, and having a normal body weight was associated with diarrhea. Chronic inflammation and proteinuria were more common in colchicine-intolerant patients, and they had reported more frequent attacks compared to those tolerating optimal doses. Conclusion: Colchicine intolerance is an important problem in daily clinical practice, mainly due to diarrhea and liver toxicity. Suboptimal colchicine dosing is associated with complications