Skip to main content
SpringerLink
Log in

Granulomatöse Vaskulitiden und Vaskulitiden mit extravaskulärer Granulomatose

Granulomatous vasculitides and vasculitides with extravascular granulomatosis

  • Leitthema
  • Published:
Zeitschrift für Rheumatologie Aims and scope Submit manuscript

Zusammenfassung

Bei den Vaskulitiden handelt es sich um entzündliche Erkrankungen von Blutgefäßen, hervorgerufen durch autoimmune oder infektiöse Prozesse, die mit einer Alteration und Zerstörung der Gefäßwand einhergehen. Unter histopathologischen Gesichtspunkten werden hinsichtlich des Entzündungsmusters granulomatöse von nekrotisierenden Vaskulitiden unterschieden. Granulomatöse Vaskulitiden zeichnen sich durch intramurale vorwiegend lymphohistiozytäre Infiltrate mit Ausbildung von Riesenzellen aus. Hierzu zählen die Riesenzellarteriitis (RZA) sowie Takayasu-Arteriitis (TAK). Demgegenüber zählt die Anti-Neutrophile zytoplasmatische Autoantikörper(ANCA)-assoziierte Vaskulitis (AAV) zur Gruppe der nekrotisierenden Vaskulitiden. Die AAV umfasst die Granulomatose mit Polyangiitis (GPA), mikroskopische Polyangiitis (MPA) und eosinophile Granulomatose mit Polyangiitis (EGPA). Die GPA und EGPA sind neben der systemischen nekrotisierenden Kleingefäßvaskulitis durch eine extravaskuläre granulomatöse nekrotisierende Entzündung charakterisiert, die hauptsächlich den oberen und/oder unteren Respirationstrakt betrifft, bei EGPA überdies mit eosinophiler Komponente. Die granulomatösen Läsionen sind Teil des autoimmunen Prozesses und gehen mit Gewebsschäden einher.

Abstract

Vasculitides are inflammatory diseases of blood vessels caused by autoimmune or infectious processes, which are associated with alterations and destruction of the vascular wall. From a histopathological point of view, granulomatous vasculitides can be distinguished from necrotizing vasculitides with respect to the pattern of inflammation. Granulomatous vasculitides are characterized by intramural, predominantly lymphohistiocytic infiltrates with the formation of giant cells. They include giant cell arteritis (GCA) and Takayasu arteritis (TAK). By contrast, anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) belongs to the group of necrotizing vasculitides. AAV includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). In addition to systemic necrotizing small vessel vasculitis, GPA and EGPA are characterized by extravascular granulomatous necrotizing inflammation mainly affecting the upper and/or lower respiratory tract, in EGPA with eosinophilic infiltrates. These granulomatous lesions are part of the autoimmune process and associated with tissue damage.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Abb. 1
Abb. 2
Abb. 3
Abb. 4
Abb. 5
Abb. 6

Literatur

  1. Arnold S, Holl UK, Lamprecht P (2020) Pathogenese der Großgefäßvaskulitiden (Pathogenesis of large vessel vasculitides). Z Rheumatol 79(6):505–515. https://doi.org/10.1007/s00393-020-00809-z

    Article  CAS  PubMed  Google Scholar 

  2. Arnold S, Holl-Ulrich K, Müller A et al (2022) Update Ätiopathogenese der Kleingefäßvaskulitis (Update on etiopathogenesis of small vessel vasculitis). Z Rheumatol. https://doi.org/10.1007/s00393-021-01155-4

    Article  PubMed  Google Scholar 

  3. Banerjee S, Grayson PC (2017) Vasculitis around the world: epidemiologic insights into causality and a need for global partnerships. J Rheumatol 44(2):136–139. https://doi.org/10.3899/jrheum.161468

    Article  PubMed  Google Scholar 

  4. Chauhan SK, Singh M, Nityanand S (2007) Reactivity of gamma/delta T cells to human 60-kd heat-shock protein and their cytotoxicity to aortic endothelial cells in Takayasu arteritis. Arthritis Rheum 56(8):2798–2802. https://doi.org/10.1002/art.22801

    Article  CAS  PubMed  Google Scholar 

  5. Fagni F, Bello F, Emmi G (2021) Eosinophilic granulomatosis with polyangiitis: dissecting the pathophysiology. Front Med. https://doi.org/10.3389/fmed.2021.627776

    Article  Google Scholar 

  6. Furuta S, Iwamoto T, Nakajima H (2019) Update on eosinophilic granulomatosis with polyangiitis. Allergol Int 68(4):430–436. https://doi.org/10.1016/j.alit.2019.06.004

    Article  CAS  PubMed  Google Scholar 

  7. Hellmich B, Lamprecht P, Spearpoint P et al (2021) New insights into the epidemiology of ANCA-associated vasculitides in Germany: results from a claims data study. Rheumatology. https://doi.org/10.1093/rheumatology/keaa924

    Article  PubMed  PubMed Central  Google Scholar 

  8. Holl-Ulrich K (2010) Histopathologie systemischer Vaskulitiden (Histopathology of systemic vasculitis). Pathologe 31(1):67–76. https://doi.org/10.1007/s00292-009-1156-x

    Article  CAS  PubMed  Google Scholar 

  9. Hysa E, Sobrero A, Camellino D et al (2020) A seasonal pattern in the onset of polymyalgia rheumatica and giant cell arteritis? A systematic review and meta-analysis. Semin Arthritis Rheum 50(5):1131–1139. https://doi.org/10.1016/j.semarthrit.2020.05.023

    Article  CAS  PubMed  Google Scholar 

  10. Jennette JC, Falk RJ (2014) Pathogenesis of antineutrophil cytoplasmic autoantibody-mediated disease. Nat Rev Rheumatol 10(8):463–473. https://doi.org/10.1038/nrrheum.2014.103

    Article  CAS  PubMed  Google Scholar 

  11. Jennette JC, Falk RJ, Bacon PA et al (2013) 2012 revised international chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheum 65(1):1–11. https://doi.org/10.1002/art.37715

    Article  CAS  PubMed  Google Scholar 

  12. Khoury P, Grayson PC, Klion AD (2014) Eosinophils in vasculitis: characteristics and roles in pathogenesis. Nat Rev Rheumatol 10(8):474–483. https://doi.org/10.1038/nrrheum.2014.98

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Koster MJ, Matteson EL, Warrington KJ (2018) Large-vessel giant cell arteritis: diagnosis, monitoring and management. Rheumatology 57(suppl_2):ii32–ii42. https://doi.org/10.1093/rheumatology/kex424

    Article  CAS  PubMed  Google Scholar 

  14. Kurata A, Saito A, Hashimoto H et al (2019) Difference in immunohistochemical characteristics between Takayasu arteritis and giant cell arteritis: It may be better to distinguish them in the same age. Mod Rheumatol 29(6):992–1001. https://doi.org/10.1080/14397595.2019.1570999

    Article  CAS  PubMed  Google Scholar 

  15. Kwon OC, Lee S‑W, Park Y‑B et al (2018) Extravascular manifestations of Takayasu arteritis: focusing on the features shared with spondyloarthritis. Arthritis Res Ther. https://doi.org/10.1186/s13075-018-1643-7

    Article  PubMed  PubMed Central  Google Scholar 

  16. Lamprecht P, Kerstein A, Klapa S et al (2018) Pathogenetic and clinical aspects of anti-neutrophil cytoplasmic autoantibody-associated Vasculitides. Front Immunol 9:680. https://doi.org/10.3389/fimmu.2018.00680

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Laudien M, Gadola SD, Podschun R et al (2010) Nasal carriage of Staphylococcus aureus and endonasal activity in Wegener s granulomatosis as compared to rheumatoid arthritis and chronic Rhinosinusitis with nasal polyps. Clin Exp Rheumatol 28(1 Suppl 57):51–55

    PubMed  Google Scholar 

  18. Lyons PA, Peters JE, Alberici F et al (2018) Genetically distinct clinical subsets, and associations with asthma and eosinophil abundance, within Eosinophilic Granulomatosis with Polyangiitis. Nat Commun. 2019;10:5120. https://doi.org/10.1038/s41467-019-12515-9

  19. Millet A, Martin KR, Bonnefoy F et al (2015) Proteinase 3 on apoptotic cells disrupts immune silencing in autoimmune vasculitis. J Clin Invest 125(11):4107–4121. https://doi.org/10.1172/JCI78182

    Article  PubMed  PubMed Central  Google Scholar 

  20. Mohammad AJ (2020) An update on the epidemiology of ANCA-associated vasculitis. Rheumatology. https://doi.org/10.1093/rheumatology/keaa089

    Article  PubMed  PubMed Central  Google Scholar 

  21. Müller A, Krause B, Kerstein-Stähle A et al (2021) Granulomatous inflammation in ANCA-associated vasculitis. IJMS. https://doi.org/10.3390/ijms22126474

    Article  PubMed  PubMed Central  Google Scholar 

  22. Onen F, Akkoc N (2017) Epidemiology of Takayasu arteritis. Presse Med 46(7–8 Pt 2):e197–e203. https://doi.org/10.1016/j.lpm.2017.05.034

    Article  PubMed  Google Scholar 

  23. Prieto-Peña D, Martínez-Rodríguez I, Loricera J et al (2019) Predictors of positive 18F-FDG PET/CT-scan for large vessel vasculitis in patients with persistent polymyalgia rheumatica. Semin Arthritis Rheum 48(4):720–727. https://doi.org/10.1016/j.semarthrit.2018.05.007

    Article  PubMed  Google Scholar 

  24. Robinette ML, Rao DA, Monach PA (2021) The immunopathology of giant cell arteritis across disease spectra. Front Immunol 12:623716. https://doi.org/10.3389/fimmu.2021.623716

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Schirmer JH, Aries PM, de Groot K et al (2017) S1-Leitlinie Diagnostik und Therapie der ANCA-assoziierten Vaskulitiden (S1 guidelines Diagnostics and treatment of ANCA-associated vasculitis). Z Rheumatol 76(Suppl 3):77–104. https://doi.org/10.1007/s00393-017-0394-1

    Article  PubMed  Google Scholar 

  26. Tamura N, Maejima Y, Tezuka D et al (2017) Profiles of serum cytokine levels in Takayasu arteritis patients: potential utility as biomarkers for monitoring disease activity. J Cardiol 70(3):278–285. https://doi.org/10.1016/j.jjcc.2016.10.016

    Article  PubMed  Google Scholar 

  27. Wang L, Ai Z, Khoyratty T et al (2020) ROS-producing immature neutrophils in giant cell arteritis are linked to vascular pathologies. JCI Insight. https://doi.org/10.1172/jci.insight.139163

    Article  PubMed  PubMed Central  Google Scholar 

  28. Watanabe R, Ishii T, Nakamura K et al (2013) Ulcerative colitis is not a rare complication of Takayasu arteritis. Mod Rheumatol. https://doi.org/10.1007/s10165-012-0827-2

    Article  PubMed  PubMed Central  Google Scholar 

  29. Wechsler ME, Akuthota P, Jayne D et al (2017) Mepolizumab or placebo for eosinophilic granulomatosis with polyangiitis. N Engl J Med 376(20):1921–1932. https://doi.org/10.1056/NEJMoa1702079

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  30. Weppner G, Ohlei O, Hammers CM et al (2018) In situ detection of PR3-ANCA+ B cells and alterations in the variable region of immunoglobulin genes support a role of inflamed tissue in the emergence of auto-reactivity in granulomatosis with polyangiitis. J Autoimmun 93:89–103. https://doi.org/10.1016/j.jaut.2018.07.004

    Article  CAS  PubMed  Google Scholar 

  31. Weyand CM, Berry GJ, Goronzy JJ (2018) The immunoinhibitory PD-1/PD-L1 pathway in inflammatory blood vessel disease. J Leukoc Biol 103(3):565–575. https://doi.org/10.1189/jlb.3MA0717-283

    Article  CAS  PubMed  Google Scholar 

  32. Zaldivar Villon MLF, La de Rocha JAL, Espinoza LR (2019) Takayasu Arteritis: Recent Developments. Curr Rheumatol Rep 21(9):45. https://doi.org/10.1007/s11926-019-0848-3

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Klinik für Rheumatologie und klinische Immunologie, Universität zu Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland

    Sabrina Arnold, Sebastian Klapa &  Peter Lamprecht

  2. Pathologie – Hamburg, MVZ Labor Lademannbogen GmbH, Hamburg, Deutschland

    Konstanze Holl-Ulrich

  3. Forschungslabor, Klinik für Rheumatologie und klinische Immunologie, Universität zu Lübeck, Lübeck, Deutschland

    Antje Müller & Anja Kerstein-Stähle

Authors
  1. Sabrina Arnold
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sebastian Klapa
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Konstanze Holl-Ulrich
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Antje Müller
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Anja Kerstein-Stähle
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Peter Lamprecht
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Peter Lamprecht.

Ethics declarations

Interessenkonflikt

S. Arnold, S. Klapa, K. Holl-Ulrich, A. Müller, A. Kerstein-Stähle und P. Lamprecht geben an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autor/-innen keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

Additional information

Redaktion

Ina Kötter, Hamburg

figure qr

QR-Code scannen & Beitrag online lesen

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Arnold, S., Klapa, S., Holl-Ulrich, K. et al. Granulomatöse Vaskulitiden und Vaskulitiden mit extravaskulärer Granulomatose. Z Rheumatol 81, 558–566 (2022). https://doi.org/10.1007/s00393-022-01249-7

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00393-022-01249-7

Schlüsselwörter

Keywords

Search

Navigation