Background/Purpose: Anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) – granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA) have heterogenous clinic and biologic phenotypes. Sporadic reports indicate that some AAV patients may have other autoimmune diseases but the frequency of the association is unknown. Our purpose was to assess the frequency of other autoimmune diseases or autoantibodies in a well-defined cohort of AAV patients.

Methods: Retrospective survey of a cohort of patients regularly controlled at outpatient facility of an Autoimmune Disease Department in a 5-year period (2011-2016). Clinical and immunologic data were retrieved from electronic records. All patients were diagnosed and treated by the authors.

Results: We included 110 AAV patients – 36 (32.7%) GPA, 45 (40.9%) MPA and 29 (26.4%) EGPA. Manifestations with higher prevalence were: chest, ear-nose-throat (ENT), mucous membranes/eyes in GPA; renal in MPA, and chest, ENT, abdominal in EGPA. Regarding to ANCA specificity – 23 (20.9%) against proteinase 3 (PR3)-ANCA, 68 (61.8%) against myeloperoxidase (MPO)-ANCA and 19 (17.3%) were ANCA negative. An additional autoimmune disease (usually organ-specific) was identified in 52 patients (47.3%), 17 (32.7%) had more than one. Associated autoimmune diseases included hypothyroidism (19,17.3%), vitamin B12 deficiency (16,13.8%), Sjögren syndrome (8,7.3%), interstitial lung disease (4,3.6%), hyperthyroidism (3,2.7%), primary biliar cholangitis (3,2.7%), celiac disease (3,2.7%), autoimmune hepatitis (2,1.8%), autoimmune thrombocytopenia (2,1.8%), myositis (2,1.8%), cryoglobulinemia (2,1.8%), anti-GBM disease (1,0.9%), and vitiligo (1,0.9%). Autoantibodies detected with higher prevalence were: ASMA (65,59.1%), ANAs (54,49.1%), anti-parietal cells (26,23.6%), anti-peroxidase (14,12.8%), anti-thyroglobulin (7,6.4%), anti-SSa/Ro (5,4.5%), anti-SSb/La (3,2.7%) and anti-dsDNA (2,1.8%). Prevalence of organ-specific autoimmune disease was higher in MPA (29,64.4%,p=0.001). Vitamin B12 deficiency was also more prevalent in MPA (25.6%,p=0.008) and in MPO-ANCA (19.1%,p=0.005). Sjögren syndrome was more prevalent in MPA (11.1%,p=0.022). Some autoantibodies were detected with higher prevalence in MPA – ANAs (68.9%,p<0.001), anti-parietal cells (31.1%,p=0.005), anti-thyroglobulin (13.3%,p=0.044), anti-SSa/Ro (8.9%,p=0.015), anti-SSb/La (4.4%,p=0.044), anti-DNAds (4.4%,p=0.003); and in anti MPO carriers – ANAs (55.9%,p=0.039), anti-SSa/Ro (7.4%,p=0.010), anti-SSb/La (4.4%,p=0.020), anti-DNAds (2.9%,p=0.046).

Conclusion: A substantial percentage of AAV patients, particularly MPA and anti MPO carriers, have associated autoimmune diseases and autoantibodies. The limitations of our study (retrospective assessment and lack of comparator) do not allow accurate estimation of prevalence. The severity of AAV and difficulties in management, may lead to overlooking of associated autoimmune diseases which appear to be frequent. Associated autoimmune disease may contribute to additional burden in AAV patients. (Supported by SAF 2014 57708-R).

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/retrospective-survey-of-concomitant-autoimmune-diseases-and-autoantibodies-in-a-cohort-of-patients-with-anca-associated-vasculitis-aav/