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Vasculitis
Other namesVasculitides[1]
Petechia and purpura on the lower limb due to infection-associated vasculitis.
Pronunciation

Vasculitis refers to the inflammation and destruction of blood vessels. Vasculitides are an uncommon and diverse group of disorders.[2]

Signs and symptoms

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The clinical presentation of the various vasculitides on the skin and internal organs is mostly determined by the diameter or size of the vessels mainly affected.[3] Non-specific symptoms are common and include fever, headache, fatigue, myalgia, weight loss, and arthralgia.[4][5]

All forms of vasculitis, even large vessel vasculitides, may cause skin manifestations. The most common skin manifestations include purpura, nodules, livedo reticularis, skin ulcers, and purpuric urticaria.[6]

Type Name Main symptoms
Primary large vessel vasculitis[7] Takayasu arteritis Diminished or absent pulses, vascular bruits, hypertension, Takayasu retinopathy, and aortic regurgitation.[8]
Giant cell arteritis Headache, scalp tenderness, jaw claudication, and blindness.[9]
Primary medium vessel vasculitis[7] Polyarteritis nodosa Mononeuritis multiplex, nodules, purpura, livedo, and hypertension.[10]
Kawasaki disease Fever, conjunctivitis, exanthema, palmoplantar erythema, cervical lymphadenopathy, and mucosal enanthema.[11][12]
Primary small vessel antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis[7] Microscopic polyangiitis Focal segmental rapidly progressive glomerulonephritis, proteinuria, hemoptysis, palpable purpura, abdominal pain, and peripheral neuropathy.[13]
Granulomatosis with polyangiitis Crusting rhinorrhea, sinusitis, chronic otitis

media, nasal obstruction, shortness of breath, and chronic cough.[14][15]

Eosinophilic granulomatosis with polyangiitis Asthma, allergic rhinitis, sinusitis, nasal polyps, peripheral neuropathy, pulmonary infiltrates, and abdominal pain.[16][17]
Primary immune complex small vessel vasculitis[7] Anti-glomerular basement membrane disease Glomerulonephritis, lung hemorrhage, hematuria, hemoptysis, cough, and dyspnea.[18]
Cryoglobulinemic vasculitis Palpable purpura, Raynaud's phenomenon, joint pain,[19] and peripheral neuropathy.
IgA vasculitis Palpable purpura, arthralgia, abdominal pain, nephritis, and haematuria.[20]
Hypocomplementemic urticarial vasculitis Hives, arthralgia, membranoproliferative glomerulonephritis, and chronic obstructive pulmonary disease.[21]
Primary variable vessel vasculitis[7] Behcet’s disease Oral ulcers, genital ulcers, papulopustular lesions, uveitis, superficial venous thrombosis and deep vein thrombosis.[22]
Cogan’s syndrome Interstitial keratitis, ocular redness, vertigo, and tinnitus.[23]
Single-organ vasculitis[24][7] Cutaneous small-vessel vasculitis Palpable purpura, necrosis, ulceration, bullae, and nodules.[25]
Cutaneous arteritis Nodules, livedo reticularis, ulcers, and gangrene.[26]
Primary central nervous system vasculitis Headache, cognitive impairment, stroke, encephalopathy, and seizures.[27]
Retinal vasculitis Visual impairments, floaters, and macular edema.[28]
Secondary vasculitis[7] Lupus vasculitis Palpable purpura, petechiae, papulonodular lesions, urticaria lesions, and mononeuritis multiplex.[29]
Rheumatoid vasculitis Purpura, focal digital lesions, ulcers, digital necrosis, pyoderma, distal sensory or motor neuropathy, and mononeuritis multiplex.[30]


Causes

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Risk factors

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Triggers

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Genetics

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Virology

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Mechanism

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Diagnosis

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Treatment

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Outlook

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Epidemiology

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Type Age Sex Ethnic differences Incidence Prevalence
Takayasu arteritis 25 ± 12.5[31] 88.3% female, 11.6 % male[31] More common in Southeast Asia[32] Annual 0.4 - 3.4 per million[32] 8.4 - 40 per million[32]
Giant cell arteritis 71-80 years[33] Female to male ratio of 3:1[33] More common in Caucasians.[34] Annual 15–35 per 100 000 individuals 50< years[34] 84 - 250 per 100,000[32]
Polyarteritis nodosa 40–60 years[32] male-to-female ratio of 1.5:1[32] None[32] Annual incidence of 0.9–8.0 per million[32] 31 per million[32]
Kawasaki disease <5 years[32] Male to female ratio of 1.5:1[35] More common in Japan[32] 5 - 22 per 100,000 children < 5 years old in North America, Europe, and Australia. > 50 per 100,000 children < 5 years old in Japan, South Korea and Taiwan.[36]
Microscopic polyangiitis 66.98 ±13.12[37] No difference.[37] More common in Southern Europe.[38] 2.4 per million (Turkey) - 16 per million (USA).[38] 19.3 per million (Turkey) - 184 per million (USA).[38]
Granulomatosis with polyangiitis 55–69 years old.[39] Incidence higher in men.[39] Incidence is higher amongst white people.[39] 11.8/million person-years.[39] 134.9 per million.[39]
Eosinophilic granulomatosis with polyangiitis 57.4±14.2[40] 1.2 per 1,000,000 person-years.[40] 2.0–38.0 per 1,000,000 people.[40]
Anti-glomerular basement membrane disease
Cryoglobulinemic vasculitis
IgA vasculitis <16 years[32]
Hypocomplementemic urticarial vasculitis 40–60 years[32]
Behcet’s disease More common during the second and third decades[32]
Cogan’s syndrome
Cutaneous small-vessel vasculitis
Cutaneous arteritis
Primary central nervous system vasculitis
Retinal vasculitis
Lupus vasculitis
Rheumatoid vasculitis

History

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Society and culture

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Special populations

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Geriatrics

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Pregnancy

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Children

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See also

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References

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  1. ^ "Vasculitis — Definition". Merriam-Webster Online Dictionary. Archived from the original on 2016-07-01. Retrieved 2009-01-08.
  2. ^ Micheletti, Robert G.; Merkel, Peter A. (2022). "Vasculitis". Interdisciplinary Approaches to Overlap Disorders in Dermatology & Rheumatology. Cham: Springer International Publishing. p. 179–198. doi:10.1007/978-3-319-18446-3_8. ISBN 978-3-319-18445-6.
  3. ^ Sunderkötter, Cord (2022). "Vasculitis and Vasculopathies". Braun-Falco´s Dermatology. Berlin, Heidelberg: Springer Berlin Heidelberg. p. 1125–1169. doi:10.1007/978-3-662-63709-8_64. ISBN 978-3-662-63708-1.
  4. ^ Luqmani, Raashid; Robson, Joanna; Suppiah, Ravi (August 2018). "272: Vasculitis". In Davey, Patrick; Sprigings, David (eds.). Diagnosis and Treatment in Internal Medicine. Oxford University Press. pp. 927–931. ISBN 978-0-19-956874-1. Retrieved 8 August 2024.
  5. ^ Jayne, David (2009). "The diagnosis of vasculitis". Best Practice & Research Clinical Rheumatology. 23 (3): 445–453. doi:10.1016/j.berh.2009.03.001.
  6. ^ Shavit, Eran; Alavi, Afsaneh; Sibbald, R. Gary (2018). "Vasculitis—What Do We Have to Know? A Review of Literature". The International Journal of Lower Extremity Wounds. 17 (4): 218–226. doi:10.1177/1534734618804982. ISSN 1534-7346.
  7. ^ a b c d e f g Jennette, J. Charles (27 September 2013). "Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides". Clinical and Experimental Nephrology. 17 (5). Springer Science and Business Media LLC: 603–606. doi:10.1007/s10157-013-0869-6. ISSN 1342-1751. PMC 4029362. PMID 24072416.
  8. ^ Johnston, S L; Lock, R J; Gompels, M M (2024-03-14). "Takayasu arteritis: a review". Journal of Clinical Pathology. 55 (7). BMJ Publishing Group. doi:10.1136/jcp.55.7.481. PMID 12101189. Retrieved 2024-08-18.
  9. ^ Hoffman, Gary S. (2016-11-01). "Giant Cell Arteritis". Annals of Internal Medicine. 165 (9): ITC67. doi:10.7326/AITC201611010. ISSN 0003-4819.
  10. ^ Forbess, Lindsy; Bannykh, Serguei (2015). "Polyarteritis Nodosa". Rheumatic Disease Clinics of North America. 41 (1): 33–46. doi:10.1016/j.rdc.2014.09.005.
  11. ^ Son, M. B. F.; Newburger, J. W. (2013-04-01). "Kawasaki Disease". Pediatrics in Review. 34 (4): 151–162. doi:10.1542/pir.34-4-151. ISSN 0191-9601.
  12. ^ Hedrich, Christian M.; Schnabel, Anja; Hospach, Toni (2018-07-10). "Kawasaki Disease". Frontiers in Pediatrics. 6. doi:10.3389/fped.2018.00198. ISSN 2296-2360. PMC 6048561. PMID 30042935.
  13. ^ Chung, Sharon A.; Seo, Philip (2010). "Microscopic Polyangiitis". Rheumatic Disease Clinics of North America. 36 (3): 545–558. doi:10.1016/j.rdc.2010.04.003. PMC 2917831. PMID 20688249.
  14. ^ Comarmond, Cloé; Cacoub, Patrice (2014). "Granulomatosis with polyangiitis (Wegener): Clinical aspects and treatment". Autoimmunity Reviews. 13 (11): 1121–1125. doi:10.1016/j.autrev.2014.08.017.
  15. ^ Grygiel-Górniak, Bogna; Limphaibool, Nattakarn; Perkowska, Katarzyna; Puszczewicz, Mariusz (2018-10-03). "Clinical manifestations of granulomatosis with polyangiitis: key considerations and major features". Postgraduate Medicine. 130 (7): 581–596. doi:10.1080/00325481.2018.1503920. ISSN 0032-5481.
  16. ^ Vaglio, A.; Buzio, C.; Zwerina, J. (2013). "Eosinophilic granulomatosis with polyangiitis (Churg–Strauss): state of the art". Allergy. 68 (3): 261–273. doi:10.1111/all.12088. ISSN 0105-4538.
  17. ^ White, Jpe; Dubey, S. (2023). "Eosinophilic granulomatosis with polyangiitis: A review". Autoimmunity Reviews. 22 (1): 103219. doi:10.1016/j.autrev.2022.103219.
  18. ^ Bharati, Joyita; Jhaveri, Kenar D.; Salama, Alan D.; Oni, Louise (2024). "Anti–Glomerular Basement Membrane Disease: Recent Updates". Advances in Kidney Disease and Health. 31 (3): 206–215. doi:10.1053/j.akdh.2024.04.007.
  19. ^ Silva, Filipa; Pinto, Claudemira; Barbosa, Arsénio; Borges, Tiago; Dias, Carlos; Almeida, Jorge (2019). "New insights in cryoglobulinemic vasculitis". Journal of Autoimmunity. 105: 102313. doi:10.1016/j.jaut.2019.102313.
  20. ^ Pillebout, Evangéline; Sunderkötter, Cord (2021). "IgA vasculitis". Seminars in Immunopathology. 43 (5): 729–738. doi:10.1007/s00281-021-00874-9. ISSN 1863-2297.
  21. ^ Gu, Stephanie L.; Jorizzo, Joseph L. (2021). "Urticarial vasculitis". International Journal of Women's Dermatology. 7 (3): 290–297. doi:10.1016/j.ijwd.2021.01.021. PMC 8243153. PMID 34222586.
  22. ^ Bettiol, Alessandra; Prisco, Domenico; Emmi, Giacomo (2020-05-01). "Behçet: the syndrome". Rheumatology. 59 (Supplement_3): iii101–iii107. doi:10.1093/rheumatology/kez626. ISSN 1462-0324.
  23. ^ Iliescu, Daniela Adriana; Timaru, Cristina Mihaela; Batras, Mehdi; Simone, Algerino De; Stefan, Cornel (2024-03-14). "COGAN'S SYNDROME". Romanian Journal of Ophthalmology. 59 (1). Romanian Society of Ophthalmology. PMID 27373108. Retrieved 2024-08-19.
  24. ^ Martins-Martinho, Joana; Dourado, Eduardo; Khmelinskii, Nikita; Espinosa, Pablo; Ponte, Cristina (2021). "Localized Forms of Vasculitis". Current Rheumatology Reports. 23 (7). doi:10.1007/s11926-021-01012-y. ISSN 1523-3774. PMC 8247627. PMID 34196889.
  25. ^ Russell, James P.; Gibson, Lawrence E. (2006). "Primary cutaneous small vessel vasculitis: approach to diagnosis and treatment". International Journal of Dermatology. 45 (1): 3–13. doi:10.1111/j.1365-4632.2005.02898.x. ISSN 0011-9059.
  26. ^ Furukawa, Fukumi (2012). "Cutaneous Polyarteritis Nodosa: An Update". Annals of Vascular Diseases. 5 (3). Editorial Committee of Annals of Vascular Diseases. doi:10.3400/avd.ra.12.00061. PMID 23555526. Retrieved 2024-08-19.
  27. ^ Junek, Mats; Perera, Kanjana S; Kiczek, Matthew; Hajj-Ali, Rula A (2023-08-26). "Current and future advances in practice: a practical approach to the diagnosis and management of primary central nervous system vasculitis". Rheumatology Advances in Practice. 7 (3). doi:10.1093/rap/rkad080. ISSN 2514-1775. PMC 10712448. PMID 38091383.
  28. ^ Abu El-Asrar, Ahmed M.; Herbort, Carl P.; Tabbara, Khalid F. (2005). "Retinal Vasculitis". Ocular Immunology and Inflammation. 13 (6): 415–433. doi:10.1080/09273940591003828. ISSN 0927-3948.
  29. ^ Leone, Patrizia; Prete, Marcella; Malerba, Eleonora; Bray, Antonella; Susca, Nicola; Ingravallo, Giuseppe; Racanelli, Vito (2021-11-05). "Lupus Vasculitis: An Overview". Biomedicines. 9 (11). MDPI AG: 1626. doi:10.3390/biomedicines9111626. ISSN 2227-9059.
  30. ^ Bartels, Christie M.; Bridges, Alan J. (2010-09-15). "Rheumatoid Vasculitis: Vanishing Menace or Target for New Treatments?". Current Rheumatology Reports. 12 (6). Springer Science and Business Media LLC: 414–419. doi:10.1007/s11926-010-0130-1. ISSN 1523-3774.
  31. ^ a b Alnabwani, Dina; Patel, Palak; Kata, Priyaranjan; Patel, Vraj; Okere, Arthur; Cheriyath, Pramil (2021-09-15). "The Epidemiology and Clinical Manifestations of Takayasu Arteritis: A Descriptive Study of Case Reports". Cureus. Springer Science and Business Media LLC. doi:10.7759/cureus.17998. ISSN 2168-8184.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  32. ^ a b c d e f g h i j k l m n Watts, Richard A.; Hatemi, Gulen; Burns, Jane C.; Mohammad, Aladdin J. (2021-12-01). "Global epidemiology of vasculitis". Nature Reviews Rheumatology. 18 (1). Springer Science and Business Media LLC: 22–34. doi:10.1038/s41584-021-00718-8. ISSN 1759-4790.
  33. ^ a b Sharma, Ankita; Mohammad, Aladdin J.; Turesson, Carl (2020). "Incidence and prevalence of giant cell arteritis and polymyalgia rheumatica: A systematic literature review". Seminars in Arthritis and Rheumatism. 50 (5). Elsevier BV: 1040–1048. doi:10.1016/j.semarthrit.2020.07.005. ISSN 0049-0172.
  34. ^ a b Watts, Richard A.; Scott, David G.I. (2014). "Epidemiology of vasculitis". Oxford Textbook of Vasculitis. Oxford University Press. p. 6–25. doi:10.1093/med/9780199659869.003.0002. ISBN 978-0-19-965986-9.
  35. ^ Rowley, Anne H.; Shulman, Stanford T. (2018-12-11). "The Epidemiology and Pathogenesis of Kawasaki Disease". Frontiers in Pediatrics. 6. doi:10.3389/fped.2018.00374. ISSN 2296-2360. PMC 6298241. PMID 30619784.
  36. ^ Elakabawi, Karim; Lin, Jing; Jiao, Fuyong; Guo, Ning; Yuan, Zuyi (2020). "Kawasaki Disease: Global Burden and Genetic Background". Cardiology Research. 11 (1). Elmer Press, Inc.: 9–14. doi:10.14740/cr993. ISSN 1923-2829.
  37. ^ a b Bataille, Pierre M.; Durel, Cécile-Audrey; Chauveau, Dominique; Panes, Arnaud; Thervet, Éric Simon; Terrier, Benjamin (2022). "Epidemiology of granulomatosis with polyangiitis and microscopic polyangiitis in adults in France". Journal of Autoimmunity. 133. Elsevier BV: 102910. doi:10.1016/j.jaut.2022.102910. ISSN 0896-8411.
  38. ^ a b c Mohammad, Aladdin J (2020-05-01). "An update on the epidemiology of ANCA-associated vasculitis". Rheumatology. 59 (Supplement_3): iii42–iii50. doi:10.1093/rheumatology/keaa089. ISSN 1462-0324.
  39. ^ a b c d e Pearce, Fiona A.; Grainge, Matthew J.; Lanyon, Peter C.; Watts, Richard A.; Hubbard, Richard B. (2016-12-24). "The incidence, prevalence and mortality of granulomatosis with polyangiitis in the UK Clinical Practice Research Datalink". Rheumatology: kew413. doi:10.1093/rheumatology/kew413. ISSN 1462-0324.
  40. ^ a b c Hwee, Jeremiah; Harper, Lorraine; Fu, Qinggong; Nirantharakumar, Krishnarajah; Mu, George; Jakes, Rupert W. (2024). "Prevalence, incidence and healthcare burden of eosinophilic granulomatosis with polyangiitis in the UK". ERJ Open Research. 10 (3): 00430–2023. doi:10.1183/23120541.00430-2023. ISSN 2312-0541. PMC 11089387. PMID 38746859.

Further reading

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