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ANCA testi pozitif olan hastaların otoimmünite ve vaskülit açısından klinik olarak incelenmesi

Year 2025, Volume: 16 Issue: 3, 486 - 492, 30.09.2025
https://doi.org/10.18663/tjcl.1679367

Abstract

Amaç: Antinötrofil sitoplazmik antikorlar (ANCA), nötrofil ve monositlerde görülen lizozomal granülleri spesifik olarak hedef alan otoantikorlardır. Çalışmanın amacı, ANCA testi pozitif olan hastaları otoimmün bozukluklar ve vaskülit açısından araştırmaktır.
Gereç ve Yöntemler: Bu 4 yıllık retrospektif kohort araştırmasında üçüncü basamak bir hastaneye gönderilen 9.480 serum örneği analiz edilmiştir. Başlangıç değerlendirmesi, vaskülit ve diğer otoimmün ve otoimmün olmayan hastalıkları olan 218 uygun ANCA-pozitif hastayı içeriyordu.
Bulgular: ANCA ilişkili vaskülit (AAV) bireylerine göre diğer iki grup daha az pulmoner, renal, kulak burun boğaz (KBB), oftalmik ve nörolojik tutuluma sahiptir. AAV ve otoimmün hastalık hastalarında eklem ve cilt tutulumu otoimmün olmayan hastalık hastalarına göre daha yüksektir. Hipertansiyon ve kronik böbrek yetmezliği AAV ve otoimmün olmayan bireylerde otoimmün olanlara göre daha yaygındır. Kronik obstrüktif hastalık (KOAH) ve proteinüri AAV hastalarında otoimmün hastalık hastalarına göre daha yüksektir. AAV hastalarında diğer ikisine göre daha fazla hematüri vardır. Otoimmün olmayan hastalıkta miyelopereoksidaz (MPO) titresi ve eritrosit sedimentasyon hızı (ESR) otoimmün hastalığa göre daha yüksektir. Yüksek proteinaz 3 (PR3) titreleri AAV ve otoimmün hastalıklarla ilişkilidir. AAV hastalarında C reaktif protein (CRP) ve kompleman 3 (C3) diğer iki gruba göre daha yüksektir. AAV'de C4 ve romatoid faktör (RF) otoimmün olmayan hastalığa göre daha yüksektir. Otoimmün hastalığı olanların başlangıç glomerüler filtrasyon hızı (GFR) diğer iki gruptan daha yüksektir.
Sonuç: ANCA'nın birçok AAV benzeri hastalıkta pozitif olabileceği sonucuna vardık. Üçüncü basamak bir hastanede ANCA titreleri, özellikle PR3 ile AAV klinik tanısı arasında güçlü bir korelasyon bulunmuştur. ANCA titresi ve organ sistemi kapsamı klinik AAV tanı göstergeleri olabilir.

Ethical Statement

Etik kurul tarafından çalışma onaylanmıştır.E2-23-4480 nolu etik kurul örneğidir.

Supporting Institution

Destekleyen kurum yoktur.

References

  • Tsukui D, Kimura Y, Kono H. Pathogenesis and pathology of anti-neutrophil cytoplasmic antibody(ANCA - associated vasculitis. J Transl Autoimmun 2021; 4:1 00094.
  • de Groot K, Csernok E, van der Woude D. History of antineutrophil cytoplasmic autoantibodies: Milestones in rheumatology. Z Rheumatol 2025; 84: 219-224.
  • Savige J, Trevisin M, Pollock W. Testing and reporting antineutrophil cytoplasmic antibodies (ANCA) in treated vasculitis and non-vasculitic disease. J Immunol Methods. 2018; 458: 1-7.
  • Folci M, Ramponi G, Solitano V, Brunetta E. Serum ANCA as Disease Biomarkers: Clinical Implications Beyond Vasculitis. Clin Rev Allergy Immunol 2022; 63: 107-123.
  • Qi F, Hao J, Wei W. Impact of different ANCA serotypes on the long-term outcome of ANCA-associated vasculitis patients. Ann Med 2023; 55: 2289614.
  • Jiang C, Liu J, Wang H, Liu H, Fu Z, Li M et al. Anti-neutrophil cytoplasmic antibody patterns can predict clinical relapse in ANCA-associated vasculitis: overall population and subgroups. Clin Exp Rheumatol 2023; 41: 848-55.
  • Walulik A, Łysak K, Błaszkiewicz M, Górecki I, Gomułka K. The Role of Neutrophils in ANCA-Associated Vasculitis: The Pathogenic Role and Diagnostic Utility of Autoantibodies. Int J Mol Sci 2023 7; 24: 17217.
  • Sun XJ, Li ZY, Chen M. Pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis. Rheumatol Immunol Res 2023; 4: 11-21.
  • Kitching AR, Anders HJ, Basu N, Brouwer E, Gordon J, Jayne DR et al. ANCA-associated vasculitis. Nat Rev Dis Primers 2020; 6: 71.
  • Morita TCAB, Trés GFS, Criado RFJ, Sotto MN, Criado PR. Update on vasculitis: an overview and dermatological clues for clinical and histopathological diagnosis - part I. Ann Bras Dermatol 2020; 95: 355-371.
  • Sayınalp B, Bilgin E, Güler AC, Kalyoncu U. c-ANCA positivity in a patient with relapsing polychondritis: Co-incidence or not? A case and review of the literature. J Turk Soc Rheumatol 2021; 13: 88-90.
  • Huang F, Lv Y, Liu S, Wu H, Liu Q. Animal models for anti-neutrophil cytoplasmic antibody-associated vasculitis: Are current models good enough? Animal Model Exp Med 2023; 6: 452-63.
  • Shochet L, Holdsworth S, Kitching AR. Animal Models of ANCA Associated Vasculitis. Front Immunol 2020; 11: 525.
  • Ashok A, Russell L, Dey M, Kouranloo K. Incidence, clinical features, management and outcomes of ANCA-associated vasculitis in pregnancy- a systematic literature review. Sarcoidosis Vasc Diffuse Lung Dis 2023; 40: e2023040.
  • Paroli M, Gioia C, Accapezzato D. New Insights into Pathogenesis and Treatment of ANCA-Associated Vasculitis: Autoantibodies and Beyond. Antibodies (Basel) 2023; 12: 25.
  • Tota M, Donizy P, Byrska M, Krajewska M, Kusztal M. An unsuspected histopathological finding -concomitant IgA nephropathy in a patient with ANCA-associated vasculitis: a case report and literature review. Front Immunol 2023; 14: 1227878.
  • Shobha V, Fathima S, Prakash R. Granulomatosis with polyangiitis: clinical course and outcome of 60 patients from a single center in South India. Clin Exp Med 2018; 18: 347-53.
  • Kim HW, Song YW. ANCA-associated vasculitis: report from Korea. Clin Exp Nephrol 2013; 17: 708-11.
  • Holle JU, Gross WL, Latza U, Nölle B, Ambrosch P, Heller M, Fertmann R, Reinhold-Keller E. Improved outcome in 445 patients with Wegener's granulomatosis in a German vasculitis center over four decades. Arthritis Rheum 2011; 63: 257-66.
  • Koç E, Aşık MA, Erdoğan EK, Arslan D, Özbek S, Özer HTE. ANCA associated vasculitis: clinical course and outcome of 44 patients from a single center in Turkey. Rheumatol Q 2023; 1: 6-13.
  • Merindol J, Levraut M, Seitz-Polski B, Morand L, Martis N. Diagnostic significance of antineutrophil cytoplasmic antibody (ANCA) titres: a retrospective case-control study. RMD Open 2023; 9: e003113.
  • Houben E, Bax WA, van Dam B, Slieker WAT, Verhave G, Frerichs FCP, van Eijk IC, Boersma WG, de Kuyper GTM, Penne EL. Diagnosing ANCA-associated vasculitis in ANCA positive patients: A retrospective analysis on the role of clinical symptoms and the ANCA titre. Medicine (Baltimore) 2016; 95: e5096.
  • Bansal PJ, Tobin MC. Neonatal microscopic polyangiitis secondary to transfer of maternal myeloperoxidase-antineutrophil cytoplasmic antibody resulting in neonatal pulmonary hemorrhage and renal involvement. Ann Allergy Asthma Immunol 2004; 93: 398-401.
  • Cui Z, Zhao MH, Segelmark M, Hellmark T. Natural autoantibodies to myeloperoxidase, proteinase 3, and the glomerular basement membrane are present in normal individuals. Kidney Int 2010; 78: 590-7.
  • Roth AJ, Ooi JD, Hess JJ, van Timmeren MM, Berg EA, Poulton CE et al. Epitope specificity determines pathogenicity and detectability in ANCA-associated vasculitis. J Clin Invest 2013; 123: 1773-83.
  • Hilhorst M, van Paassen P, Tervaert JW; Limburg Renal Registry. Proteinase 3-ANCA Vasculitis versus Myeloperoxidase-ANCA Vasculitis. J Am Soc Nephrol 2015; 26: 2314-27.
  • FijoŁek J, Wiatr E. Antineutrophil cytoplasmic antibodies (ANCA) - their role in pathogenesis, diagnosis, and treatment monitoring of ANCA-associated vasculitis. Cent Eur J Immunol 2020; 45: 218-27.

Clinical investigation of patients with positive antineutrophil cytoplasmic antibodies (ANCA) test for autoimmunity and vasculitis

Year 2025, Volume: 16 Issue: 3, 486 - 492, 30.09.2025
https://doi.org/10.18663/tjcl.1679367

Abstract

Aim: Antineutrophil cytoplasmic antibodies (ANCA) autoantibodies target neutrophil and monocyte lysosomal granules. The objective of the study is to investigate patients with a positive ANCA test for autoimmune disorders and vasculitis.
Materials and Methods: This 4-year retrospective cohort research analyzed 9.480 Turkish tertiary hospital serum samples. The baseline evaluation included 218 eligible ANCA-positive patients with vasculitis (AAV) and other autoimmune and non-autoimmune diseases.
Results: The other two groups have less pulmonary, renal, ear nose and throat (ENT), ophthalmic, and neurologic involvement than AAV individuals. AAV and autoimmune disease patients have higher joint and skin involvement than non-autoimmune disease patients. Hypertension and chronic renal failure are more common in AAV and non-autoimmune individuals than autoimmune ones. Chronic obstructive pulmonary disease (COPD) and proteinuria are higher in AAV patients than autoimmune disease patients. AAV patients have more hematuria than the other two. For non-autoimmune disease, myeloperoxidase (MPO) titer and erythrocyte sedimentation rate (ESR) are higher than for autoimmune disease. High proteinase 3 (PR3) titers are associated with AAV and autoimmune disorders. AAV patients have higher CRP and complement 3(C3) than the other two groups. In AAV, C4 and rheumatoid factor (RF) are higher than in non-autoimmune disease. Patients with autoimmune disease had higher initial GFR than the other two groups.
Conclusion: We concluded that ANCA may be positive in numerous AAV-like diseases. A tertiary hospital found a strong correlation between ANCA titers, particularly PR3, and AAV clinical diagnosis. ANCA titre and organ system extent may be clinical AAV diagnostic indicators.

Ethical Statement

The Non-Interventional Clinical Research Ethics Committee of Ankara Bilkent City Hospital approved this study on July 12, 2023, under Decision Number. E2-23-4480.

Supporting Institution

None

References

  • Tsukui D, Kimura Y, Kono H. Pathogenesis and pathology of anti-neutrophil cytoplasmic antibody(ANCA - associated vasculitis. J Transl Autoimmun 2021; 4:1 00094.
  • de Groot K, Csernok E, van der Woude D. History of antineutrophil cytoplasmic autoantibodies: Milestones in rheumatology. Z Rheumatol 2025; 84: 219-224.
  • Savige J, Trevisin M, Pollock W. Testing and reporting antineutrophil cytoplasmic antibodies (ANCA) in treated vasculitis and non-vasculitic disease. J Immunol Methods. 2018; 458: 1-7.
  • Folci M, Ramponi G, Solitano V, Brunetta E. Serum ANCA as Disease Biomarkers: Clinical Implications Beyond Vasculitis. Clin Rev Allergy Immunol 2022; 63: 107-123.
  • Qi F, Hao J, Wei W. Impact of different ANCA serotypes on the long-term outcome of ANCA-associated vasculitis patients. Ann Med 2023; 55: 2289614.
  • Jiang C, Liu J, Wang H, Liu H, Fu Z, Li M et al. Anti-neutrophil cytoplasmic antibody patterns can predict clinical relapse in ANCA-associated vasculitis: overall population and subgroups. Clin Exp Rheumatol 2023; 41: 848-55.
  • Walulik A, Łysak K, Błaszkiewicz M, Górecki I, Gomułka K. The Role of Neutrophils in ANCA-Associated Vasculitis: The Pathogenic Role and Diagnostic Utility of Autoantibodies. Int J Mol Sci 2023 7; 24: 17217.
  • Sun XJ, Li ZY, Chen M. Pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis. Rheumatol Immunol Res 2023; 4: 11-21.
  • Kitching AR, Anders HJ, Basu N, Brouwer E, Gordon J, Jayne DR et al. ANCA-associated vasculitis. Nat Rev Dis Primers 2020; 6: 71.
  • Morita TCAB, Trés GFS, Criado RFJ, Sotto MN, Criado PR. Update on vasculitis: an overview and dermatological clues for clinical and histopathological diagnosis - part I. Ann Bras Dermatol 2020; 95: 355-371.
  • Sayınalp B, Bilgin E, Güler AC, Kalyoncu U. c-ANCA positivity in a patient with relapsing polychondritis: Co-incidence or not? A case and review of the literature. J Turk Soc Rheumatol 2021; 13: 88-90.
  • Huang F, Lv Y, Liu S, Wu H, Liu Q. Animal models for anti-neutrophil cytoplasmic antibody-associated vasculitis: Are current models good enough? Animal Model Exp Med 2023; 6: 452-63.
  • Shochet L, Holdsworth S, Kitching AR. Animal Models of ANCA Associated Vasculitis. Front Immunol 2020; 11: 525.
  • Ashok A, Russell L, Dey M, Kouranloo K. Incidence, clinical features, management and outcomes of ANCA-associated vasculitis in pregnancy- a systematic literature review. Sarcoidosis Vasc Diffuse Lung Dis 2023; 40: e2023040.
  • Paroli M, Gioia C, Accapezzato D. New Insights into Pathogenesis and Treatment of ANCA-Associated Vasculitis: Autoantibodies and Beyond. Antibodies (Basel) 2023; 12: 25.
  • Tota M, Donizy P, Byrska M, Krajewska M, Kusztal M. An unsuspected histopathological finding -concomitant IgA nephropathy in a patient with ANCA-associated vasculitis: a case report and literature review. Front Immunol 2023; 14: 1227878.
  • Shobha V, Fathima S, Prakash R. Granulomatosis with polyangiitis: clinical course and outcome of 60 patients from a single center in South India. Clin Exp Med 2018; 18: 347-53.
  • Kim HW, Song YW. ANCA-associated vasculitis: report from Korea. Clin Exp Nephrol 2013; 17: 708-11.
  • Holle JU, Gross WL, Latza U, Nölle B, Ambrosch P, Heller M, Fertmann R, Reinhold-Keller E. Improved outcome in 445 patients with Wegener's granulomatosis in a German vasculitis center over four decades. Arthritis Rheum 2011; 63: 257-66.
  • Koç E, Aşık MA, Erdoğan EK, Arslan D, Özbek S, Özer HTE. ANCA associated vasculitis: clinical course and outcome of 44 patients from a single center in Turkey. Rheumatol Q 2023; 1: 6-13.
  • Merindol J, Levraut M, Seitz-Polski B, Morand L, Martis N. Diagnostic significance of antineutrophil cytoplasmic antibody (ANCA) titres: a retrospective case-control study. RMD Open 2023; 9: e003113.
  • Houben E, Bax WA, van Dam B, Slieker WAT, Verhave G, Frerichs FCP, van Eijk IC, Boersma WG, de Kuyper GTM, Penne EL. Diagnosing ANCA-associated vasculitis in ANCA positive patients: A retrospective analysis on the role of clinical symptoms and the ANCA titre. Medicine (Baltimore) 2016; 95: e5096.
  • Bansal PJ, Tobin MC. Neonatal microscopic polyangiitis secondary to transfer of maternal myeloperoxidase-antineutrophil cytoplasmic antibody resulting in neonatal pulmonary hemorrhage and renal involvement. Ann Allergy Asthma Immunol 2004; 93: 398-401.
  • Cui Z, Zhao MH, Segelmark M, Hellmark T. Natural autoantibodies to myeloperoxidase, proteinase 3, and the glomerular basement membrane are present in normal individuals. Kidney Int 2010; 78: 590-7.
  • Roth AJ, Ooi JD, Hess JJ, van Timmeren MM, Berg EA, Poulton CE et al. Epitope specificity determines pathogenicity and detectability in ANCA-associated vasculitis. J Clin Invest 2013; 123: 1773-83.
  • Hilhorst M, van Paassen P, Tervaert JW; Limburg Renal Registry. Proteinase 3-ANCA Vasculitis versus Myeloperoxidase-ANCA Vasculitis. J Am Soc Nephrol 2015; 26: 2314-27.
  • FijoŁek J, Wiatr E. Antineutrophil cytoplasmic antibodies (ANCA) - their role in pathogenesis, diagnosis, and treatment monitoring of ANCA-associated vasculitis. Cent Eur J Immunol 2020; 45: 218-27.
There are 27 citations in total.

Details

Primary Language English
Subjects Autoimmunity, Immunology (Other), Clinical Microbiology
Journal Section Research Article
Authors

Emrah Salman 0000-0002-5293-0803

Alparslan Toyran 0000-0002-0218-4881

Publication Date September 30, 2025
Submission Date April 18, 2025
Acceptance Date August 14, 2025
Published in Issue Year 2025 Volume: 16 Issue: 3

Cite

APA Salman, E., & Toyran, A. (2025). Clinical investigation of patients with positive antineutrophil cytoplasmic antibodies (ANCA) test for autoimmunity and vasculitis. Turkish Journal of Clinics and Laboratory, 16(3), 486-492. https://doi.org/10.18663/tjcl.1679367
AMA Salman E, Toyran A. Clinical investigation of patients with positive antineutrophil cytoplasmic antibodies (ANCA) test for autoimmunity and vasculitis. TJCL. September 2025;16(3):486-492. doi:10.18663/tjcl.1679367
Chicago Salman, Emrah, and Alparslan Toyran. “Clinical Investigation of Patients With Positive Antineutrophil Cytoplasmic Antibodies (ANCA) Test for Autoimmunity and Vasculitis”. Turkish Journal of Clinics and Laboratory 16, no. 3 (September 2025): 486-92. https://doi.org/10.18663/tjcl.1679367.
EndNote Salman E, Toyran A (September 1, 2025) Clinical investigation of patients with positive antineutrophil cytoplasmic antibodies (ANCA) test for autoimmunity and vasculitis. Turkish Journal of Clinics and Laboratory 16 3 486–492.
IEEE E. Salman and A. Toyran, “Clinical investigation of patients with positive antineutrophil cytoplasmic antibodies (ANCA) test for autoimmunity and vasculitis”, TJCL, vol. 16, no. 3, pp. 486–492, 2025, doi: 10.18663/tjcl.1679367.
ISNAD Salman, Emrah - Toyran, Alparslan. “Clinical Investigation of Patients With Positive Antineutrophil Cytoplasmic Antibodies (ANCA) Test for Autoimmunity and Vasculitis”. Turkish Journal of Clinics and Laboratory 16/3 (September2025), 486-492. https://doi.org/10.18663/tjcl.1679367.
JAMA Salman E, Toyran A. Clinical investigation of patients with positive antineutrophil cytoplasmic antibodies (ANCA) test for autoimmunity and vasculitis. TJCL. 2025;16:486–492.
MLA Salman, Emrah and Alparslan Toyran. “Clinical Investigation of Patients With Positive Antineutrophil Cytoplasmic Antibodies (ANCA) Test for Autoimmunity and Vasculitis”. Turkish Journal of Clinics and Laboratory, vol. 16, no. 3, 2025, pp. 486-92, doi:10.18663/tjcl.1679367.
Vancouver Salman E, Toyran A. Clinical investigation of patients with positive antineutrophil cytoplasmic antibodies (ANCA) test for autoimmunity and vasculitis. TJCL. 2025;16(3):486-92.