Structure of the vasculitides observed in the Clinic of Rheumatology
PDF EN
PDF BG (Български)

Keywords

Vasculitis, ANCA-associated vasculitides (AAV), vasculitis with necrosis, classification

How to Cite

[1]
Yoneva, T., Zdravkova, Y., Kotov, G., Rashkov, R. and Sheytanov, I. 2020. Structure of the vasculitides observed in the Clinic of Rheumatology. Rheumatology (Bulgaria). 28, 3 (Nov. 2020), 7-24. DOI:https://doi.org/10.35465/28.3.2020.pp7-24.

Abstract

Systemic vasculitides are a heterogenic group of disorders characterized by destructive inflammation and fibrinoid necrosis of the vascular wall, vessel occlusion and tissue ischemia. Vasculitides presenting with necrosis are included in neither of the contemporary classifications, even though this type of vascular pathology is the one with the most dramatic manifestations in rheumatology. There have been no analyses of the nosological association, clinical features and therapeutic management of the vasculitides with necrosis in the pertinent literature. The aim of the present study was to analyze vasculitides in the Bulgarian population in terms of their nosological association; to examine the portion that ANCA-associated vasculitides represent out of all vasculitic syndromes on the one hand, and to make an analysis of the vasculitides with necrosis according to their nosological association on the other. In the present study, we included 388 patients with vasculitis, 251 of whom were female and 137 male. We conducted a prospective and retrospective analysis which covered the patients with vasculitis who were admitted to the Clinic of Rheumatology over the period 2009-2018. ANCA-associated vasculitides were the most often diagnosed vasculitides in the Clinic of Rheumatology. Vasculitic manifestations over the course of connective tissue diseases (most often systemic lupus erythematosus and systemic sclerosis) were the second most common group. Life-threatening cases of vasculitis with necrosis were mainly the result of flares of different connective tissue diseases. The major necrotizing vasculitides (Wegener’s granulomatosis and microscopic polyangiitis) were responsible for 12.6% of the cases of vasculitis with necrosis. In order to establish the correct diagnosis and start the suitable treatment, it is of vital importance to recognize the different vasculitic syndromes and their wide differential diagnosis. Most of them respond well to the currently available therapeutic options, especially if the correct diagnosis has been established early.

https://doi.org/10.35465/28.3.2020.pp7-24
PDF EN
PDF BG (Български)

References

  1. Larbi T, Hamzaoui S, Abdallah M, et al. Current Classifications. In: Systemic Vasculitis. 1st Ed. L. Hendaoui, A. Stanson, M. Habib Bouhaouala, F. Joffre (Eds.). Berlin, Heidelberg, Springer-Verlag, 2012, 7-13.
  2. Gross WL, Trabandt A, Reinhold-Keller E. Diagnosis and evaluation of vasculitis. Rheumatology (Oxford), 2000, 39(3), 245-252. https://academic.oup.com/rheumatology/article/39/3/245/1783711
  3. Abdgawad M. History, Classification and Pathophysiology of Small Vessel Vasculitis, Updates in the Diagnosis and Treatment of Vasculitis, Lazaros I. Sakkas and Christina Katsiari, IntechOpen. https://www.intechopen.com/books/updates-in-the-diagnosis-and-treatment-of-vasculitis/history-classification-and-pathophysiology-of-small-vessel-vasculitis
  4. Dunogué B, Pagnoux C, Guillevin L. Churg-Strauss syndrome: clinical symptoms, complementary investigations, prognosis and outcome, and treatment. Semin Respir Crit Care Med, 2011, 32(3), 298-309. https://www.thieme-connect.com/products/ejournals/abstract/10.1055/s-0031-1279826
  5. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum, 2013, 65(1), 1-11. https://onlinelibrary.wiley.com/doi/pdf/10.1002/art.37715
  6. Sada KE, Yamamura M, Harigai M, et al. Classification and characteristics of Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study. Arthritis Res Ther, 2014, 16(2), R101. https://arthritis-research.biomedcentral.com/articles/10.1186/ar4550
  7. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum, 1994, 37(2), 187-192. https://onlinelibrary.wiley.com/doi/epdf/10.1002/art.1780370206
  8. Basu N, Watts R, Bajema I, et al. EULAR points to consider in the development of classification and diagnostic criteria in systemic vasculitis. Ann Rheum Dis, 2010, 69(10), 1744-1750. https://ard.bmj.com/content/69/10/1744
  9. Fries JF, Hunder GG, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Summary. Arthritis Rheum, 1990, 33(8), 1135-1136. https://onlinelibrary.wiley.com/doi/epdf/10.1002/art.1780330812
  10. Bruce IN, Bell AL. A comparison of two nomenclature systems for primary system vasculitis. Br J Rheumatol 1997, 36, 453–458. https://academic.oup.com/rheumatology/article/36/4/453/1783341
  11. Louis Bridges S, Ball GV. Vasculitis, Oxford, Oxford University Press (P), 2008, 3-21.
  12. Koldingsnes W, Nossent H. Epidemiology of ANCA associated vasculitis. Norsk Epidemiologi, 2009, 18(1), 37-48. https://www.ntnu.no/ojs/index.php/norepid/article/view/62
  13. Gibson A, Stamp LK, Chapman PT, et al. The epidemiology of Wegener's granulomatosis and microscopic polyangiitis in a Southern Hemisphere region. Rheumatology (Oxford), 2006, 45(5), 624-628. https://academic.oup.com/rheumatology/article/45/5/624/1788733
  14. Hauer HA, Bajema IM, van Houwelingen HC, et al. Renal histology in ANCA-associated vasculitis: differences between diagnostic and serologic subgroups. Kidney Int, 2002, 61(1), 80-89. https://www.kidney-international.org/article/S0085-2538(15)48154-2/fulltext
  15. Ball GV, Fressler BJ, Louis Bridges S. Oxford Textbook of Vasculitis, Oxford, Oxford University Press (P), 2014.
  16. Cardinali C, Caproni M, Bernacchi E, et al. The spectrum of cutaneous manifestations in lupus erythematosus--the Italian experience. Lupus, 2000, 9(6), 417-423. https://journals.sagepub.com/doi/10.1191/096120300678828569?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200pubmed
  17. Cieślik P, Hrycek A, Kłuciński P. Vasculopathy and vasculitis in systemic lupus erythematosus. Pol Arch Med Wewn, 2008, 118(1-2), 57-63. https://www.mp.pl/paim/en/node/306/pdf
  18. Drenkard C, Villa AR, Reyes E, et al. Vasculitis in systemic lupus erythematosus. Lupus, 1997, 6(3), 235-242. https://journals.sagepub.com/doi/10.1177/096120339700600304?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200pubmed
  19. González-Gay MA, García-Porrúa C. Systemic vasculitis in adults in northwestern Spain, 1988-1997. Clinical and epidemiologic aspects. Medicine (Baltimore), 1999, 78(5), 292-308. https://journals.lww.com/md-journal/Abstract/1999/09000/Systemic_Vasculitis_in_Adults_in_Northwestern.2.aspx
  20. Ramos-Casals M, Nardi N, Lagrutta M, et al. Vasculitis in systemic lupus erythematosus: prevalence and clinical characteristics in 670 patients. Medicine (Baltimore), 2006, 85(2), 95-104. https://journals.lww.com/md-journal/Fulltext/2006/03000/Vasculitis_in_Systemic_Lupus_Erythematosus_.4.aspx
  21. Appel GB, Pirani CL, D'Agati V. Renal vascular complications of systemic lupus erythematosus. J Am Soc Nephrol, 1994, 4(8), 1499-1515. https://jasn.asnjournals.org/content/4/8/1499.long
  22. Carlson JA, Chen KR. Cutaneous vasculitis update: neutrophilic muscular vessel and eosinophilic, granulomatous, and lymphocytic vasculitis syndromes. Am J Dermatopathol, 2007, 29(1), 32-43. https://journals.lww.com/amjdermatopathology/Abstract/2007/02000/Cutaneous_Vasculitis_Update__Neutrophilic_Muscular.7.aspx
  23. Oddis CV, Eisenbeis CH Jr, Reidbord HE, et al. Vasculitis in systemic sclerosis: association with Sjögren's syndrome and the CREST syndrome variant. J Rheumatol, 1987, 14(5), 942-948. https://pubmed.ncbi.nlm.nih.gov/3430523/
  24. Quéméneur T, Mouthon L, Cacoub P, et al. Systemic vasculitis during the course of systemic sclerosis: report of 12 cases and review of the literature. Medicine (Baltimore), 2013, 92(1), 1-9. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5370746/pdf/medi-92-01.pdf
  25. Feldman D, Hochberg MC, Zizic TM, et al. Cutaneous vasculitis in adult polymyositis/dermatomyositis. J Rheumatol, 1983, 10(1), 85-89. https://jhu.pure.elsevier.com/en/publications/cutaneous-vasculitis-in-adult-polymyositisdermatomyositis-3
  26. Kadoya A, Akahoshi T, Sekiyama N, et al. Cutaneous vasculitis in a patient with dermatomyositis without muscle involvement. Intern Med, 1994, 33(12), 809-812. https://www.jstage.jst.go.jp/article/internalmedicine1992/33/12/33_12_809/_pdf/-char/en
  27. Panush RS, Katz P, Longley S, et al. Rheumatoid vasculitis: diagnostic and therapeutic decisions. Clin Rheumatol, 1983, 2(4), 321-330. https://link.springer.com/article/10.1007/BF02041550
  28. Watts RA, Carruthers DM, Symmons DP, et al. The incidence of rheumatoid vasculitis in the Norwich Health Authority. Br J Rheumatol, 1994, 33(9), 832-833. https://academic.oup.com/rheumatology/article-abstract/33/9/832/1782118?redirectedFrom=fulltext
  29. Nyhäll-Wåhlin BM, Petersson IF, Jacobsson C, et al. Extra-articular manifestations in a community-based sample of patients with rheumatoid arthritis: incidence and relationship to treatment with TNF inhibitors. Scand J Rheumatol, 2012, 41(6), 434-437. https://www.tandfonline.com/doi/full/10.3109/03009742.2012.695803
  30. Hervier B, Hamidou M, Haroche J, et al. Systemic lupus erythematosus associated with ANCA-associated vasculitis: an overlapping syndrome?. Rheumatol Int, 2012, 32(10), 3285-3290. https://link.springer.com/article/10.1007%2Fs00296-011-2055-z
  31. Curtiss P, Liebman T, Khorolsky C, et al. Systemic lupus erythematosus and antineutrophil cytoplasmic antibody-associated vasculitis: An emerging overlap syndrome with cutaneous manifestations. JAAD Case Rep, 2018, 4(5), 493-496. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6031565/pdf/main.pdf
  32. Draibe J, Salama AD. Association of ANCA associated vasculitis and rheumatoid arthritis: a lesser recognized overlap syndrome. Springerplus, 2015, 4, 50. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4315802/pdf/40064_2015_Article_835.pdf
  33. Martín-Nares E, Zuñiga-Tamayo D, Hinojosa-Azaola A. Prevalence of overlap of antineutrophil cytoplasmic antibody associated vasculitis with systemic autoimmune diseases: an unrecognized example of poliautoimmunity. Clin Rheumatol, 2019, 38(1), 97-106. https://link.springer.com/article/10.1007%2Fs10067-018-4212-1
  34. Lee LA, Norris DA. Mechanisms of cutaneous tissue damage in lupus erythematosus. Immunol Ser, 1989, 46, 359-386. https://europepmc.org/article/med/2488864
  35. Herrick AL, Oogarah PK, Freemont AJ, et al. Vasculitis in patients with systemic sclerosis and severe digital ischaemia requiring amputation. Ann Rheum Dis, 1994, 53(5), 323-326. https://ard.bmj.com/content/53/5/323.long
Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.