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Vasculiti facili e difficili: cosa deve sapere e saper fare un pediatra
Vasculitides: a diagnostic challenge for paediatricians
Laura De Nardi1, Maria Isabella Petrone2, Simone Benvenuto1, Valentina Natoli3, Sarah Abu-Rumeileh4, Benmario Castaldo1, Fabrizio De Benedetti5
Scuola di Specializzazione in Pediatria
1Università di Trieste, 2Università “Tor Vergata”, Roma, 3Università di Genova, 4Università di Firenze
5IRCCS Ospedale Pediatrico “Bambino Gesù”, Roma
Maggio 2023 - pagg. 286 -298 | DOI: 10.53126/MEB42286
Abstract
Vasculitis is defined by a state of inflammation of the blood vessel walls. It can result in formation of either aneurysms or stenosis with ischemia and necrosis. The heterogeneity of clinical presentation depends on the type and size of the vessels involved, which leads to different patterns of organ damage. The skin is usually involved but not necessarily as a first manifestation of disease. Indeed, significant organ damage may occur at any time in the disease course and may precede other symptoms with significant morbidity and mortality, especially in case of diagnostic delay. The most common vasculitides in childhood are IgA-associated vasculitis (Schönlein-Henoch purpura) and Kawasaki disease. Other vasculitides are much rarer, often arising with insidious symp- toms that make them life-threatening. This paper aims to review the literature on paediatric vasculitides providing a practical guide to disentangle between different diagnoses. It also provides an overview that underlines the key differences in vasculitis presentation, management and follow-up.
Riassunto
Vasculite è per definizionel'infiammazione della parete dei vasi sanguigni. Tale flogosi può portare alla formazione di aneurismi o stenosi con conseguente ischemia e/o necrosi. L’eterogeneità della presentazione clinica dipende dal tipo e dal calibro del vaso coinvolto, tali da portare a differenti pattern di coinvolgimento d’organo. La pelle è quasi sempre interessata in corso di vasculite, ma non necessariamente per prima. Il danno d’organo può infatti precedere qualsiasi altro sintomo con una morbilità e mortalità significative, soprattutto in caso di ritardo diagnostico. La vasculite a IgA (porpora di Schönlein–Henoch) e la malattia di Kawasaki sono le vasculiti più comuni. Altre vasculiti sono più rare, spesso caratterizzate da sintomatologia sfumata che le rende difficili da diagnosticare, e con alta mortalità. Questo lavoro si offre come una guida pratica per il pediatra che debba districarsi tra le vasculiti, fornendo messaggi chiave per una corretta identificazione e diagnosi precoce.
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Bibliografia
1. Pavan M, Marchetti F, Lepore L. Le vasculiti “maggiori” in età pediatrica. Medico e Bambino 2011;30:223-8.
2. Lazzarin P, Pasero G, Marson P, Cecchetto A, Zanchin G. Takayasu’s arteritis. A concise review and some observations on a putative case reported by Giovanni Battista Morgagni (1761). Reumatismo 2011;57(4):305-13. doi: 10.4081/reumatismo.2005.305.
3. Danda D, Goel R, Joseph G, Kumar ST, Nair A, Ravindran R, et al. Clinical course of 602 patients with Takayasu’s arteritis: comparison between Childhood-onset versus adult onset disease. Rheumatology 2021;60(5): 2246-55. doi: 10.1093/rheumatology/ keaa569.
4. Misra DP, Rathore U, Kopp CR, Patro P, Agarwal V, Sharma A. Presentation and clinical course of pediatric-onset versus adult-onset Takayasu arteritis - a systematic review and meta-analysis. Clin Rheumatol 2022; 41(12):3601-13. doi: 10.1007/s10067-022-06318-5.
5. Schnabel A, Hedrich CM. Childhood vasculitis. Front Pediatr 2019;6:421. doi: 10.3389/fped.2018.00421.
6. Ozen S, Ruperto N, Dillon MJ, et al. EULAR/PreS endorsed consensus criteria for the classification of childhood vasculites. Ann Rheum Dis 2006;65:936-41. doi: 10.1136/ ard.2005.046300.
7. Calligaris L, Marchetti F, Barbi E, Ventura A. La porpora di Schönlein-Henoch. Medico e Bambino 2009;28(1):19-26.
8. Saulsbury FT. Clinical update: Henoch-Schönlein purpura. Lancet 2007;369(9566): 976-8. doi: 10.1016/S0140-6736(07)60474-7.
9. Hahn D, Hodson EM, Willis NS, Craig JC. Interventions for preventing and treating kidney disease in Henoch-Schönlein Purpura (HSP). Cochrane Database Syst Rev 2015 (8):CD005128. doi: 10.1002/14651858. CD005128.pub3.
10. Jauhola O, Ronkainen J, Koskimies O, et al. Renal manifestations of Henoch-Schonlein purpura in a 6-month prospective study of 223 children. Archives of Disease in Childhood 2010;95(11):877-82. doi: 10.1136/ adc.2009.182394.
11. Marchesi A, Tarissi de Jacobis I, et al. Kawasaki disease: guidelines of the Italian Society of Pediatrics, part I - definition, epidemiology, etiopathogenesis, clinical expression and management of the acute phase. Ital J Pediatr 2018;44(1):102. doi: 10.1186/ s13052-018-0536-3.
12. Nakamura Y, Yashiro M, Uehara R, et al. Epidemiologic features of Kawasaki disease in Japan: results of the 2007-2008 Nationwide Survey. Journal of Epidemiology 2010;20(4): 302-7. doi: 10.2188/jea.je20090180.
13. Taddio A, Pellegrin MC, Centenari C, Filippeschi IP, Ventura A, Maggiore G. Acute febrile cholestatic jaundice in children: keep in mind Kawasaki disease. J Pediatr Gastroenterol Nutr 2012;55(4):380-3. doi: 10.1097/MPG.0b013e31825513de.
14. Tulloh RMR, Mayon-White R, Harnden A, et al. Kawasaki disease: a prospective population survey in the UK and Ireland from 2013 to 2015. Arch Dis Child 2019;104(7):640-6. doi: 10.1136/archdischild-2018-315087.
15. Mossberg M, Segelmark M, Kahn R, Englund M, Mohammad A. Epidemiology of primary systemic vasculitis in children: a population-based study from southern Sweden. Scandinavian Journal of Rheumatology 2018;47(4):295-302. doi: 10.1080/03009742. 2017.1412497.
16. Lyskina G, Bockeria O, Shirinsky O, et al. Cardiovascular outcomes following Kawasaki disease in Moscow, Russia: A single center experience. Glob Cardiol Sci Pract 2017;2017 (3):e201723. doi: 10.21542/gcsp.2017.23.
17. Friedman KG, Gauvreau K, Hamaoka‐ Okamoto A, et al. Coronary artery aneurysms in Kawasaki disease: risk factors for progressive disease and adverse cardiac events in the US population. J Am Heart Assoc 2016; 5(9):e003289. doi: 10.1161/JAHA.116.003289.
18. Eleftheriou D, Levin M, Shingadia D, Tulloh R, Klein N, Brogan P. Management of Kawasaki disease. Arch Dis Child 2014;99(1): 74-83. doi: 10.1136/archdischild-2012-302841.
19. Fabi M, Andreozzi L, Corinaldesi E, et al. Inability of Asian risk scoring systems to predict intravenous immunoglobulin resistance and coronary lesions in Kawasaki disease in an Italian cohort. Eur J Pediatr 2019;178(3): 315-22. doi: 10.1007/s00431-018-3297-5.
20. Marchetti F. La fine della questione sull’uso del cortisone nella malattia di Kawasaki? Medico e Bambino pagine elettroniche 2014;17(3).
21. Ventura A. Cortisone nella Kawasaki: giustizia è fatta. Medico e Bambino 2016;35(9): 555-6.
22. Ventura A. Cortisone da subito nella Kawasaki: passo dopo passo, sempre più convinti. Medico e Bambino 2020;39(10):621-2. doi: 10.53126/MEB39621.
23. Chen S, Dong Y, Kiuchi MG, et al. Coronary artery complication in Kawasaki disease and the importance of early intervention: a systematic review and meta-analysis. JAMA Pediatr 2016;170(12):1156. doi: 10.1001/jamapediatrics.2016.2055.
24. Green J, Wardle AJ, Tulloh RM. Corticosteroids for the treatment of Kawasaki disease in children. Cochrane Database Syst Rev 2022;5(5):CD011188. doi: 10.1002/14651858. CD011188.pub3.
25. Okubo Y, Michihata N, Morisaki N, et al. Association between dose of glucocorticoids and coronary artery lesions in Kawasaki disease. Arthritis Care Res (Hoboken) 2018; 70(7):1052-7. doi: 10.1002/acr.23456.
26. Jia X, Du X, Bie S, Li X, Bao Y, Jiang M. What dose of aspirin should be used in the initial treatment of Kawasaki disease? A meta-analysis. Rheumatology 2020;59(8):1826-33. doi: 10.1093/rheumatology/keaa050.
27. Kabbaha S, Milano A, Aldeyab MA, Thorlund K. Infliximab as a second-line therapy for children with refractory Kawasaki disease: A systematic review and meta-analysis of randomized controlled trials. Br J Clin Pharmacol 2023;89(1):49-60. doi: 10.1111/bcp. 15547.
28. Koné‐Paut I, Tellier S, Belot A, et al. Phase II open label study of anakinra in intravenous immunoglobulin-resistant Kawasaki disease. Arthritis Rheumatol 2021;73(1):151-61. doi: 10.1002/art.41481.
29. Lin MT, Sun LC, Wu ET, Wang JK, Lue HC, Wu MH. Acute and late coronary outcomes in 1073 patients with Kawasaki disease with and without intravenous γ-immunoglobulin therapy. Arch Dis Child 2015;100(6): 542-7. doi: 10.1136/archdischild-2014-306427.
30. Zhou Q, Yang D, Ombrello AK, et al. Early-onset stroke and vasculopathy associated with mutations in ADA2. N Engl J Med 2014;370(10):911-20. doi: 10.1056/NEJMoa1307361.
31. Meyts I, Aksentijevich I. Deficiency of adenosine deaminase 2 (DADA2): updates on the phenotype, genetics, pathogenesis, and treatment. J Clin Immunol 2018;38(5): 569-78. doi: 10.1007/s10875-018-0525-8.
32. Caorsi R, Penco F, Grossi A, et al. ADA2 deficiency (DADA2) as an unrecognised cause of early onset polyarteritis nodosa and stroke: a multicentre national study. Ann Rheum Dis 2017;76(10):1648-56. doi: 10.1136/annrheumdis-2016-210802.
33. Navon Elkan P, Pierce SB, Segel R, et al. Mutant adenosine deaminase 2 in a polyarteritis nodosa vasculopathy. N Engl J Med 2014;370(10):921-31. doi: 10.1056/NEJMoa1307362.
34. Ombrello AK, Qin J, Hoffmann PM, et al. Treatment strategies for deficiency of adenosine deaminase 2. N Engl J Med 2019;380 (16):1582-4. doi: 10.1056/NEJMc1801927.
35. Cafaro A, Pigliasco F, Barco S, et al. A novel LC-MS/MS-based method for the diagnosis of ADA2 deficiency from dried plasma spot. Molecules 2021;26(18):5707. doi: 10.3390/molecules26185707.
36. Fullerton HJ, Wu YW, Sidney S, Johnston SC. Risk of recurrent childhood arterial ischemic stroke in a population-based cohort: the importance of cerebrovascular imaging. Pediatrics 2007;119(3):495-501. doi: 10.1542/ peds.2006-2791.
37. Smitka M, Bruck N, Engellandt K, Hahn G, Knoefler R, von der Hagen M. Clinical perspective on primary angiitis of the central nervous system in childhood (cPACNS). Front Pediatr 2020;8:281. doi: 10.3389/fped. 2020.00281.
38. Gupta N, Hiremath SB, Aviv RI, Wilson N. Childhood cerebral vasculitis: a multidisciplinary approach. Clin Neuroradiol 2023;33(1):5-20. doi: 10.1007/s00062-022-01185-8.
39. Calabrese LH, Mallek JA. Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine (Baltimore) 1988;67(1):20-39. doi: 10.1097/ 00005792-198801000-00002.
40. Cellucci T, Tyrrell PN, Pullenayegum E, Benseler SM. von Willebrand factor antigen - a possible biomarker of disease activity in childhood central nervous system vasculitis? Rheumatology 2012;51(10):1838-45. doi: 10.1093/rheumatology/kes156.
41. University of Calgary. Treatment for CNS vasculitis and IBrainD. https://cumming. ucalgary.ca/research/brainworks/resources-physicians/treatment-protocols.
42. Rabusin M, Lepore L, Costantinides F, Bussani R. A child with severe asthma. Lancet 1998;351(9095):32. doi: 10.1016/S0140-6736(97)09499-3.
43. Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 33(8):1094-100. doi: 10.1002/art.1780330806.
44. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27(2):277-301.
45. Pavan M, Agrusti A., Trombetta A, et al. La granulomatosi eosinofilica con poliangioite: dalla pelle al cuore. Medico e Bambino 2020;39(9):569-74. doi: 10.53126/MEB39569.
46. Calatroni M, Oliva E, Gianfreda D, et al. ANCA-associated vasculitis in childhood: recent advances. Ital J Pediatr 2017;43(1):46. doi: 10.1186/s13052-017-0364-x.
47. Amaddeo A, Ventura A, Marchetti F, Benettoni A, Londero M. Should cardiac involvement be included in the criteria for diagnosis of Churg Strauss syndrome? J Pediatr 2012;160(4):707. doi: 10.1016/j.jpeds.2011. 09.057.
48. Wechsler ME, Akuthota P, Jayne D, et al.; EGPA Mepolizumab Study Team. Mepolizumab or placebo for eosinophilic granulomatosis with polyangiitis. N Engl J Med 2017;376(20):1921-32. doi: 10.1056/NEJMoa1702079.
49. Suppiah R, Robson JC, Grayson PC, et al. 2022 American College of Rheumatology/ European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis. Arthritis Rheumatol 2022;74 (3):400-6. doi: 10.1136/annrheumdis-2021-221796.
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