Incidence of Systemic Vasculitis and Clinical Outcomes in Systemic Sclerosis: 2-Years Follow-Up of Asymptomatic Positive for Anti-Neutrophilic Cytoplasmic Antibody
Raiwin Suwannamajo, Ajanee Mahakkanukrauh, Siraphop Suwannaroj, Chingching Foocharoen Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, ThailandCorrespondence: Chingching Foocharoen, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 4000...
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| Format: | Article |
| Language: | English |
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Dove Medical Press
2024-01-01
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| Series: | Clinical, Cosmetic and Investigational Dermatology |
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| Online Access: | https://www.dovepress.com/incidence-of-systemic-vasculitis-and-clinical-outcomes-in-systemic-scl-peer-reviewed-fulltext-article-CCID |
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| author | Suwannamajo R Mahakkanukrauh A Suwannaroj S Foocharoen C |
| author_facet | Suwannamajo R Mahakkanukrauh A Suwannaroj S Foocharoen C |
| author_sort | Suwannamajo R |
| collection | DOAJ |
| description | Raiwin Suwannamajo, Ajanee Mahakkanukrauh, Siraphop Suwannaroj, Chingching Foocharoen Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, ThailandCorrespondence: Chingching Foocharoen, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand, Tel +6643363746, Email fching@kku.ac.thPurpose: Anti-neutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) overlap with systemic sclerosis (SSc) is uncommon. We aimed to determine the incidence of AAV and define clinical outcomes relevant to asymptomatic screening positive for ANCA in SSc after 2 years of follow-up.Patients and Methods: The study was a cohort study of 185 Thai adult SSc patients testing for ANCA and having a 2-year follow-up at the Scleroderma Clinic, Khon Kaen University, Thailand. The incidence of AAV and outcomes of those who tested positive for ANCA were evaluated.Results: A total of 185 SSc patients were tested for ANCA, of whom 21.6% were positive for either cytoplasmic ANCA, perinuclear ANCA (p-ANCA), anti-myeloperoxidase (anti-MPO), or anti-proteinase3 antibody. Only one 52-year-old female patient with dcSSc, negative for initial ANCA test, developed AAV (microscopic polyangiitis) 7 months after the first ANCA test for an incidence of AAV of 0.27 per 100-person-years (95% CI 0.01– 1.5). She was positive for p-ANCA and anti-MPO. Eight of those who had an initial test were positive for ANCA and underwent a repeated test. Only two cases persisted as positive for ANCA (1 anti-MPO and 1 anti-PR3) and had no clinicals suspicious of vasculitis. Four cases that had ANCA turned to a negative result.Conclusion: AAV is a rare complication in SSc, so ANCA may not have any role as a screening test for AAV as it cannot predict the development of AAV in SSc. We suggest testing for ANCA only in SSc patients with clinicals suspicious of AAV.Keywords: systemic sclerosis, scleroderma, anti-neutrophilic cytoplasmic antibody, ANCA- associated vasculitis, vasculitis |
| first_indexed | 2024-03-08T08:42:16Z |
| format | Article |
| id | doaj.art-5c3e3e2661084397a1624e2cbbd97314 |
| institution | Directory Open Access Journal |
| issn | 1178-7015 |
| language | English |
| last_indexed | 2024-03-08T08:42:16Z |
| publishDate | 2024-01-01 |
| publisher | Dove Medical Press |
| record_format | Article |
| series | Clinical, Cosmetic and Investigational Dermatology |
| spelling | doaj.art-5c3e3e2661084397a1624e2cbbd973142024-02-01T17:59:27ZengDove Medical PressClinical, Cosmetic and Investigational Dermatology1178-70152024-01-01Volume 1730130990083Incidence of Systemic Vasculitis and Clinical Outcomes in Systemic Sclerosis: 2-Years Follow-Up of Asymptomatic Positive for Anti-Neutrophilic Cytoplasmic AntibodySuwannamajo RMahakkanukrauh ASuwannaroj SFoocharoen CRaiwin Suwannamajo, Ajanee Mahakkanukrauh, Siraphop Suwannaroj, Chingching Foocharoen Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, ThailandCorrespondence: Chingching Foocharoen, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand, Tel +6643363746, Email fching@kku.ac.thPurpose: Anti-neutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) overlap with systemic sclerosis (SSc) is uncommon. We aimed to determine the incidence of AAV and define clinical outcomes relevant to asymptomatic screening positive for ANCA in SSc after 2 years of follow-up.Patients and Methods: The study was a cohort study of 185 Thai adult SSc patients testing for ANCA and having a 2-year follow-up at the Scleroderma Clinic, Khon Kaen University, Thailand. The incidence of AAV and outcomes of those who tested positive for ANCA were evaluated.Results: A total of 185 SSc patients were tested for ANCA, of whom 21.6% were positive for either cytoplasmic ANCA, perinuclear ANCA (p-ANCA), anti-myeloperoxidase (anti-MPO), or anti-proteinase3 antibody. Only one 52-year-old female patient with dcSSc, negative for initial ANCA test, developed AAV (microscopic polyangiitis) 7 months after the first ANCA test for an incidence of AAV of 0.27 per 100-person-years (95% CI 0.01– 1.5). She was positive for p-ANCA and anti-MPO. Eight of those who had an initial test were positive for ANCA and underwent a repeated test. Only two cases persisted as positive for ANCA (1 anti-MPO and 1 anti-PR3) and had no clinicals suspicious of vasculitis. Four cases that had ANCA turned to a negative result.Conclusion: AAV is a rare complication in SSc, so ANCA may not have any role as a screening test for AAV as it cannot predict the development of AAV in SSc. We suggest testing for ANCA only in SSc patients with clinicals suspicious of AAV.Keywords: systemic sclerosis, scleroderma, anti-neutrophilic cytoplasmic antibody, ANCA- associated vasculitis, vasculitishttps://www.dovepress.com/incidence-of-systemic-vasculitis-and-clinical-outcomes-in-systemic-scl-peer-reviewed-fulltext-article-CCIDsystemic sclerosissclerodermaanti-neutrophilic cytoplasmic antibodyanca- associated vasculitisvasculitis |
| spellingShingle | Suwannamajo R Mahakkanukrauh A Suwannaroj S Foocharoen C Incidence of Systemic Vasculitis and Clinical Outcomes in Systemic Sclerosis: 2-Years Follow-Up of Asymptomatic Positive for Anti-Neutrophilic Cytoplasmic Antibody Clinical, Cosmetic and Investigational Dermatology systemic sclerosis scleroderma anti-neutrophilic cytoplasmic antibody anca- associated vasculitis vasculitis |
| title | Incidence of Systemic Vasculitis and Clinical Outcomes in Systemic Sclerosis: 2-Years Follow-Up of Asymptomatic Positive for Anti-Neutrophilic Cytoplasmic Antibody |
| title_full | Incidence of Systemic Vasculitis and Clinical Outcomes in Systemic Sclerosis: 2-Years Follow-Up of Asymptomatic Positive for Anti-Neutrophilic Cytoplasmic Antibody |
| title_fullStr | Incidence of Systemic Vasculitis and Clinical Outcomes in Systemic Sclerosis: 2-Years Follow-Up of Asymptomatic Positive for Anti-Neutrophilic Cytoplasmic Antibody |
| title_full_unstemmed | Incidence of Systemic Vasculitis and Clinical Outcomes in Systemic Sclerosis: 2-Years Follow-Up of Asymptomatic Positive for Anti-Neutrophilic Cytoplasmic Antibody |
| title_short | Incidence of Systemic Vasculitis and Clinical Outcomes in Systemic Sclerosis: 2-Years Follow-Up of Asymptomatic Positive for Anti-Neutrophilic Cytoplasmic Antibody |
| title_sort | incidence of systemic vasculitis and clinical outcomes in systemic sclerosis 2 years follow up of asymptomatic positive for anti neutrophilic cytoplasmic antibody |
| topic | systemic sclerosis scleroderma anti-neutrophilic cytoplasmic antibody anca- associated vasculitis vasculitis |
| url | https://www.dovepress.com/incidence-of-systemic-vasculitis-and-clinical-outcomes-in-systemic-scl-peer-reviewed-fulltext-article-CCID |
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