Abstract
IgA vasculitis, formerly known as Henoch–Schonlein purpura (HSP), is the most common form of systemic vasculitis in children and is characterized by inflammation of the small vessels with typical deposition of IgA immune complexes. It is a leukocytoclastic type of vasculitis and is characterized by a tetrad of clinical manifestations: non-thrombocytopenia or coagulopathy-induced palpable purpura, arthritis, or arthralgia, gastrointestinal, and renal involvement. The exact cause of IgA vasculitis is not known yet, although infections, vaccinations and insect bites have been implicated in the appearance of the disease. The main risk factors for Clostridioides difficile infection (CDI) are previous CDI, age > 65 years old, pharmacologic agents (antibiotics, PPIs, histamine-2 receptor antagonists, glucocorticoids, and chemotherapy), prior hospitalization, the presence of co-morbidities, especially inflammatory bowel diseases and chronic kidney disease (CKD) and immunosuppression. Oral vancomycin or fidaxomicin are the gold standard of the therapy, with metronidazole being an alternative choice. The purpose of this study was to describe a case of IgA vasculitis and Clostridioides difficile infection to see whether there is any association between the two distinct clinical entities. Herein, we describe a 17-year old patient with IgA vasculitis and bloody diarrhea due to Clostridioides difficile infection and we discuss the co-existence of these two pathological conditions. The patient presented to the hospital with diffuse abdominal pain, nausea, vomiting, and two episodes of bloody diarrhea. Stools tested positive for Clostridioides difficile toxins, while he remained afebrile with hs-CRP = 1.5 mg/dL (normal range < 0.5 mg/dL). Direct immunofluorescence from the extremities’ purplish eruption showed leukocytoclastic vasculitis with IgA deposition. Whether co-existence of the two above-mentioned distinct clinical entities is just a co-incidence or CDI is a triggering factor for IgA vasculitis remains to be elucidated in future large-scale studies.
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Abbreviations
- CDI:
-
Clostridioides difficile infection
- HSP:
-
Henoch–Schonlein purpura
- CKD:
-
Chronic kidney disease
- ESR:
-
Erythrocyte sedimentation rate
- WBC:
-
White Blood count
References
Levy M, Broyer M, Arsan A, Levy-Bentolila D, Habib R (1976) Anaphylactoid purpura nephritis in childhood: natural history and immunopathology. Adv Nephrol Necker Hosp 6:183–184
Wang JJ, Xu Y, Liu FF, Wu Y, Samadli S, Wu YF, Luo HH, Zhang DD, Hu P (2020) Association of the infectious triggers with childhood Henoch-Schonlein purpura in Anhui province, China. J Infect Public Health 13(1):110–117. https://doi.org/10.1016/j.jiph.2019.07.004
Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V, Garcia-Fuentes M, Gonzalez-Gay MA (1997) Henoch-Schonlein purpura in adulthood and childhood: two different expressions of the same syndrome. Arthritis Rheum 40(5):859–864
Crobach MJT, Vernon JJ, Loo VG, Kong LY, Pechine S, Wilcox MH, Kuijper EJ (2018) Understanding Clostridium difficile colonization. Clin Microbiol Rev. https://doi.org/10.1128/CMR.00021-17
Jarmo O, Veli-Jukka A, Eero M (2019) Treatment of Clostridioides (Clostridium) infection. Ann Med 13:1–9. https://doi.org/10.1080/07853890.2019.1701703
Boey CC, Ramanujam TM, Looi LM (1997) Clostridium difficile-related necrotizing pseudomembranous enteritis in association with Henoch-Schonlein purpura. J Pediatr Gastroenterol Nutr 24(4):426–429
Cojocariu C, Stanciu C, Ancuta C, Danciu M, Chiriac S, Trifan A (2016) Immunoglobulin A vasculitis complicated with Clostridium difficile infection: a rare case report and brief review of the literature. J Gastrointest Liver Dis 25(2):235–238. https://doi.org/10.15403/jgld.2014.1121.252.csd
Narchi H, Beattle TJ, Taylor RG, Evans TJ, Azmy AF (1988) Pseudomembranous colitis in association with Henoch–Schonlein purpura. Scott Med J 33:308–309
Alsaied T, Weber J, George A, Villegas M, Vossmeyer TM (2014) An adolescent with abdominal pain, rash, joint swelling, severe bloody diarrhea and impressive leukocytosis. Clin Pediatr 53:1206–1208. https://doi.org/10.1177/0009922814536266
Hayakawa T, Imaeda H, Nakamura M, Komoto S, Maruta K, Shiozu H, Ogata H, Iwao Y, Ishii H, Hibi T (2005) Association of pseudomembranous colitis with Henoch–Schonlein purpura. J Gastroenterol 40:641–645
Feasel P, Billings SD, Bergfeld WF, Piliang MP, Fernandez AP, Ko JS (2018) Direct immunofluorescence testing in vasculitis: a single institution experience with Henoch–Schonlein purpura. J Cutan Pathol 45(1):16–22. https://doi.org/10.1111/cup.13054
Hung SP, Yang YH, Lin YT, Wang LC, Lee JH, Chiang BL (2009) Clinical manifestations and outcomes of Henoch–Schonlein purpura: comparison between adults and children. Pediatr Neonatol 50(4):162–168. https://doi.org/10.1016/S1875-9572(09)60056-5
Nakamura A, Fuchigami T, Inamo Y (2010) Protein-loosing enteropathy associated with Henoch–Schonlein purpura. Pediatr Rep 2(2):e20. https://doi.org/10.4081/pr.2010.e20
Trygstad CW, Stiehm ER (1971) Elevated serum IgA globulin in anaphylactoid purpura. Pediatrics 47(6):1023–1028
Saulsbury FT (1999) Henoch–Schonlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore) 78(6):395–409
Tang C, Scaramangas-Plumley D, Nast CC, Mosenifar Z, Edelstein MA, Weisman M (2017) A case of Henoch–Schonlein Purpura associated with rotavirus infection in an elderly Asian male and review of the literature. Am J Case Rep 8(18):136–142
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DK, FK, and FP wrote the whole manuscript; MV and NGV supervised the manuscript; CK and DS were responsible for the figures and the histopathology findings, while AS was responsible for the references’ section.
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Kounatidis, D., Vadiaka, M., Kouvidou, C. et al. Clostridioides difficile infection in a patient with immunoglobulin A vasculitis: a triggering factor or a rare complication of the disease? A case-based review. Rheumatol Int 40, 997–1000 (2020). https://doi.org/10.1007/s00296-020-04586-5
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DOI: https://doi.org/10.1007/s00296-020-04586-5