FMF Is Associated With a Wide Spectrum of MHC Class I- and Allied SpA Disorders but Not With Classical MHC Class II-Associated Autoimmune Disease: Insights From a Large Cohort Study

摘要: Objectives: To test the hypothesis that familial Mediterranean fever (FMF)-associated autoinflammation may exaggerate tendency toward adaptive immunopathology or spondyloarthritis (SpA)-associated disorders including major histocompatibility complex (MHC) class I associated but not classical MHC II-associated exhibit transplacental autoimmunity myasthenia gravis and pemphigus. Methods: Seven thousand seven hundred forty-seven FMF patients 10,080 age- sex-matched controls in Clalit Health Services medical database were identified compared terms of prevalence SpA-associated disorders. We also evaluated four strong disorders, namely, pemphigus vulgaris, gravis, sarcoidosis, pernicious anemia, to ascertain whether such associations with SpA-spectrum disease specific merely reflected non-specific consequences innate immune system activation on driving divergent types immunity. The diagnosis was based records genetically proven. Results: showed a association I-related diseases: odds ratio (OR) 28.58 [95% confidence interval (95% CI), 6.93-117.87; p < 0.0001] for Behcet's disease, OR 10.33 CI, 4.09-26.09; 0.0001) ankylosing spondylitis, 1.67 1.19-2.33; = 0.0029) psoriasis. For weakly I-linked diseases, an 3.76 2.48-5.69; Crohn's 2.64 1.52-4.56; 0.0005) ulcerative colitis found. No found between autoimmune Conclusion: are increased risk SpA-related MHC-I-opathies MHC-II-associated suggesting tissue-specific dysregulation immunity share SpA spectrum drive I-associated conditions.