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Title: | Significance of anti-neutrophil cytoplasmic antibodies in systemic sclerosis. | Authors: | Wilson M.;Nikpour M.;Strickland G.;Ross L.;Stevens W.;Roddy J.;Major G.;Moxey J.;Huq M.;Proudman S.;Sahhar J. ;Ngian G.-S.;Walker J. | Institution: | (Moxey, Huq, Ross, Nikpour) University of Melbourne, 41 Victoria Parade Fitzroy, Melbourne, Victoria 3065, Australia (Moxey, Wilson, Ross, Stevens, Nikpour) St. Vincent's Hospital Melbourne, 41 Victoria Parade Fitzroy, Melbourne, VIC 3065, Australia (Proudman) University of Adelaide, Adelaide, SA, Australia (Proudman) Royal Adelaide Hospital, Adelaide, SA, Australia (Sahhar, Ngian) Monash Health, Melbourne, VIC, Australia (Sahhar, Ngian) Monash University, Melbourne, VIC, Australia (Walker) Flinders Medical Centre, Adelaide, SA, Australia (Strickland) Barwon Rheumatology Service, Geelong, VIC, Australia (Major) Royal Newcastle Centre John Hunter Hospital, Newcastle, NSW, Australia (Major) University of Newcastle, Newcastle, NSW, Australia (Roddy) Fiona Stanley Hospital, Perth, WA, Australia | Issue Date: | 10-Apr-2019 | Copyright year: | 2019 | Publisher: | BioMed Central (E-mail: info@biomedcentral.com) | Place of publication: | United Kingdom | Publication information: | Arthritis Research and Therapy. 21 (1) (no pagination), 2019. Article Number: 57. Date of Publication: 14 Feb 2019. | Journal: | Arthritis Research and Therapy | Abstract: | Background: Up to 12% of patients with systemic sclerosis (SSc) have anti-neutrophil cytoplasmic antibodies (ANCA). However, the majority of these patients do not manifest ANCA-associated vasculitis (AAV) and the significance of ANCA in these patients is unclear. The aim of this study is to determine the prevalence of ANCA in a well-characterised SSc cohort and to examine the association between ANCA and SSc clinical characteristics, other autoantibodies, treatments and mortality. Method(s): Clinical data were obtained from 5 centres in the Australian Scleroderma Cohort Study (ASCS). ANCA positive was defined as the presence of any one or combination of cytoplasmic ANCA (c-ANCA), perinuclear ANCA (p-ANCA), atypical ANCA, anti-myeloperoxidase (anti-MPO) or anti-proteinase-3 (anti-PR3). Associations of demographic and clinical features with ANCA were investigated by logistic or linear regression. Survival analysis was performed using Kaplan-Meyer curves and Cox regression models. Result(s): Of 1303 patients, 116 (8.9%) were ANCA positive. Anti-PR3 was more common than anti-MPO (13.8% and 11.2% of ANCA-positive patients, respectively). Only 3 ANCA-positive patients had AAV. Anti-Scl-70 was more common in ANCA positive vs ANCA negative (25% vs 12.8%, p < 0.001), anti-MPO positive vs anti-MPO negative (38.5% vs 13.6%, p = 0.006) and anti-PR3 positive vs anti-PR3 negative patients (44.4% vs 13.4%, p < 0.001). A higher prevalence of interstitial lung disease (ILD) was found in the ANCA positive (44.8% vs 21.8%, p < 0.001) and the anti-PR3 positive groups (50.0% vs 23.4%, p = 0.009). In multivariable analysis, ANCA-positive status remained associated with ILD after adjusting for anti-Scl-70 antibodies. Pulmonary embolism (PE) was more common in ANCA-positive patients (8.6% vs 3.0%, p = 0.002) and anti-PR3-positive patients (16.7% vs 3.3%, p = 0.022). ANCA-positive status remained associated with PE in a multivariable analysis adjusting for anti-phospholipid antibodies. Kaplan-Meier analysis revealed increased mortality in ANCA-positive patients (p = 0.006). In Cox regression analysis, ANCA was associated with increased mortality, after adjusting for age and sex. Conclusion(s): ANCA is associated with increased prevalence of ILD and PE in SSc. ANCA should be tested in SSc, as it identifies individuals with worse prognosis who require close monitoring for adverse outcomes.Copyright © 2019 The Author(s). | DOI: | http://monash.idm.oclc.org/login?url=http://dx.doi.org/10.1186/s13075-019-1839-5 | PubMed URL: | 30764870 [http://www.ncbi.nlm.nih.gov/pubmed/?term=30764870] | ISSN: | 1478-6354 | URI: | https://repository.monashhealth.org/monashhealthjspui/handle/1/35301 | Type: | Article | Subjects: | azathioprine/dt [Drug Therapy] biological product/dt [Drug Therapy] calcium channel blocking agent/dt [Drug Therapy] cyclophosphamide/dt [Drug Therapy] iloprost/dt [Drug Therapy] mycophenolic acid/dt [Drug Therapy] myeloblastin/ec [Endogenous Compound] myeloperoxidase/ec [Endogenous Compound] *neutrophil cytoplasmic antibody/ec [Endogenous Compound] penicillamine/dt [Drug Therapy] phospholipid antibody/ec [Endogenous Compound] prednisolone/dt [Drug Therapy] rituximab/dt [Drug Therapy] scl 70 antibody/ec [Endogenous Compound] tocilizumab/dt [Drug Therapy] tumor necrosis factor inhibitor/dt [Drug Therapy] unclassified drug vasodilator agent/dt [Drug Therapy] proteinase 3 antibody/ec [Endogenous Compound] myeloperoxidase antibody/ec [Endogenous Compound] adult age ANCA associated vasculitis/co [Complication] article clinical feature cohort analysis comorbidity disease association female human interstitial lung disease/co [Complication] lung embolism/co [Complication] major clinical study male middle aged mortality rate prevalence *prognosis prognostic assessment sex survival analysis synovitis/co [Complication] *systemic sclerosis/dt [Drug Therapy] abatacept/dt [Drug Therapy] autoantibody/ec [Endogenous Compound] lung embolism / complication major clinical study male middle aged mortality rate prevalence *prognosis prognostic assessment sex ANCA associated vasculitis / complication synovitis / complication *systemic sclerosis / *drug therapy age adult survival analysis Article clinical feature cohort analysis comorbidity disease association female human interstitial lung disease / complication |
Type of Clinical Study or Trial: | Observational study (cohort, case-control, cross sectional or survey) |
Appears in Collections: | Articles |
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