Atypical presentation of immunoglobulin A vasculitis mimicking acute appendicitis in a pediatric patient: a case report
Status PubMed-not-MEDLINE Language English Country Great Britain, England Media print-electronic
Document type Journal Article
PubMed
40961422
PubMed Central
PMC12452480
DOI
10.1080/20565623.2025.2558311
Knihovny.cz E-resources
- Keywords
- Acute appendicitis, IgA vasculitis, abdominal surgery, diagnostic challenges, gastrointestinal involvement, pediatric abdominal pain, purpura, vasculitis,
- Publication type
- Journal Article MeSH
Acute appendicitis is a common pediatric surgical emergency, but its clinical presentation can overlap with other conditions, complicating diagnosis. We present a case of a 4-year-old female who initially presented with symptoms suggestive of appendicitis, including right iliac fossa pain, vomiting, and fever. Imaging studies were inconclusive, and a laparoscopic appendectomy was performed. No signs of appendicitis were found, but vasculitic changes were noted in a segment of the ileum. Postoperatively, the patient developed a mild purpuric rash, and elevated C-reactive protein (CRP) levels prompted further investigation. Subsequent laboratory tests and clinical findings led to a diagnosis of Immunoglobulin A (IgA) vasculitis, characterized by a small-vessel vasculitis affecting the gastrointestinal tract. This case highlights the diagnostic challenges when presenting symptoms mimic appendicitis but are ultimately attributed to an atypical presentation of IgA vasculitis. The importance of considering systemic inflammatory conditions like IgA vasculitis in pediatric abdominal pain is emphasized, especially when initial diagnoses do not align with imaging or surgical findings. A multidisciplinary approach, including rheumatological evaluation, was crucial in securing the correct diagnosis and managing the patient's care.
IgA vasculitis (IgAV) is a condition that causes inflammation of small blood vessels. It can often affect the skin, joints, kidneys, and digestive system. This condition usually involves the upper part of the small intestine, but in rare cases, it can affect the very end of the small intestine (terminal ileum). In our case, a patient came to the hospital with sudden, severe abdominal pain and imaging that suggested a localized bowel problem. Surgeons operated urgently, and only later (when a skin rash appeared) IgAV was diagnosed. The patient recovered quickly after starting steroid treatment, without needing further bowel procedures. This case shows that IgAV can sometimes look like a surgical emergency. By being aware of this possibility, we can prevent unnecessary surgery and guide treatment decisions.
Department of Surgery Masaryk University Brno Czech Republic
Faculty of Medical Sciences Lebanese University Beirut Lebanon
See more in PubMed
Andersson RE. The natural history and traditional management of appendicitis. World J Surg. 2021;45(7):1829–1836. PubMed
Sulu B, Gunerhan Y, Palanci Y, et al. Unusual findings in appendectomy specimens: evaluation of 2458 cases and review of the literature. Indian J Surg. 2011;73(4):261–266.
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. doi: 10.1002/art.37715 PubMed DOI
Heineke MH, Ballering AV, Jamin A, et al. New insights in the pathogenesis of immunoglobulin A vasculitis (Henoch-Schönlein purpura). Autoimmun Rev. 2017;16(12):1246–1253. doi: 10.1016/j.autrev.2017.10.009 PubMed DOI
Xu L, Li Y, Wu X.. IgA vasculitis update: epidemiology, pathogenesis, and biomarkers. Front Immunol. 2022;13:921864. doi: 10.3389/fimmu.2022.921864 PubMed DOI PMC
Ruperto N, Ozen S, Pistorio A, et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis, and childhood Takayasu arteritis: Ankara 2008. Part I: overall methodology and clinical characterization. Ann Rheum Dis. 2010;69(5):790–797. doi: 10.1136/ard.2009.116624 PubMed DOI
Pillebout E, Sunderkötter C.. IgA vasculitis (Henoch-Schönlein purpura). J Am Soc Nephrol. 2021;32(10):2791–2802.
Trapani S, Micheli A, Grisolia F, et al. Henoch-Schönlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of the literature. Semin Arthritis Rheum. 2005;35(3):143–153. doi: 10.1016/j.semarthrit.2005.08.007 PubMed DOI
Pillebout E, Thervet E, Hill G, et al. Henoch-Schönlein purpura in adults: outcome and prognostic factors. J Am Soc Nephrol. 2002;13(5):1271–1278. doi: 10.1097/01.asn.0000013883.99976.22 PubMed DOI
Narchi H. Risk of long-term renal impairment and duration of follow up recommended for Henoch-Schönlein purpura with normal or minimal urinary findings: a systematic review. Arch Dis Child. 2005;90(9):916–920. doi: 10.1136/adc.2005.074641 PubMed DOI PMC
Agarwal P, Sharma A.. Diagnostic challenges in pediatric abdominal pain. J Pediatr Surg. 2018;53(7):1420–1426.
Jiao Y, Zhang S.. Gastrointestinal involvement in IgA vasculitis: a review. Pediatr Rheumatol. 2017;15(1):31.
Zhou L, Li L.. Clinical spectrum of IgA vasculitis in children. Lancet Rheumatol. 2019;1(4):217–227.
Chen Y, Wang X.. Small bowel vasculitis in IgA vasculitis: pathophysiological insights. Int J Rheumatol. 2020;2020:9845296.
Sampson M, O’Neill A.. Diagnosis of IgA vasculitis in pediatric patients. Pediatr Rheumatol Int Trials Org J. 2018;19(1):16–22.
Hunter SE, Malik A.. Use of corticosteroids in autoimmune diseases. BMJ. 2017;358:j3580.
Kim J, Park S.. Masking of symptoms by corticosteroids in pediatric rheumatic diseases. Rheumatol Rep. 2016;8(2):43–48.
Murray BW, Peters E.. Delayed rash in IgA vasculitis. Rheumatol J. 2020;29(3):272–279.
Eisen SA, Russo A.. Corticosteroid therapy and delayed symptom onset in autoimmune diseases. Autoimmun Rev. 2021;20(10):102799.
Gong YQ, Han L, Zhang JY, et al. Abdominal imaging and endoscopic characteristics of adult abdominal IgA vasculitis: a multicenter retrospective study. Ann Med. 2024;56(1):2408467. doi: 10.1080/07853890.2024.2408467 PubMed DOI PMC
Ozen S, Pistorio A, Iusan SM, et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: final classification criteria. Ann Rheum Dis. 2010;69(5):798–806. doi: 10.1136/ard.2009.116657 PubMed DOI
Cai ZP, Wu HY, Han DG, et al. Clinical features and risk factors of abdominal IgA vasculitis in adults. Clin Rheumatol. 2025;44(9):3639–3648. doi: 10.1007/s10067-025-07569-8 PubMed DOI
Rubino C, Monacelli C, Marrani E, et al. Gastrointestinal involvement in IgA vasculitis: a single-center 11-year study on a cohort of 118 children. Clin Rheumatol. 2021;40(12):5041–5046. doi: 10.1007/s10067-021-05863-9 PubMed DOI
Cojocariu C, Singeap AM, Chiriac S, et al. IgA vasculitis-Henoch-Schönlein purpura. Dig Syst Recent Adv. 2020;35:35–49.
Sağlam MK, Yıldırım S, Ergüven M, et al. Gastrointestinal features of pediatric IgA vasculitis and their association with renal complications: an observational study. Eur J Pediatr. 2025;184(5):320–328. doi: 10.1007/s00431-025-06157-x PubMed DOI PMC
Levine DM, Schumacher A.. Systemic lupus erythematosus and gastrointestinal symptoms: a comprehensive review. Clin Rev Allergy Immunol. 2019;56(3):242–253.
Gonzalez S, Khanna S.. Diagnostic criteria for inflammatory bowel disease in children. Pediatr Gastroenterol. 2018;56(1):56–63.
Sulu B, Gunerhan Y, Palanci Y, et al. Unusual findings in appendectomy specimens: evaluation of 2458 cases and review of the literature. Indian J Surg. 2011;73(4):261–266.
Xu L, Li Y, Wu X.. IgA vasculitis update: epidemiology, pathogenesis, and biomarkers. Front Immunol. 2022;13:921864. PubMed PMC
Gong YQ, Han L, Zhang JY, et al. Abdominal imaging and endoscopic characteristics of adult abdominal IgA vasculitis: a multicenter retrospective study. Ann Med. 2024;56(1):2408467. PubMed PMC
Cai ZP, Wu HY, Han DG, et al. Clinical features and risk factors of abdominal IgA vasculitis in adults. Clin Rheumatol. 2025;44(9):3639–3648. PubMed
Rubino C, Monacelli C, Marrani E, et al. Gastrointestinal involvement in IgA vasculitis: a single-center 11-year study on a cohort of 118 children. Clin Rheumatol. 2021;40(12):5041–5046. PubMed