Abstract
Immunoglobulin A (IgA) vasculitis mainly affects the joints, skin, kidneys, and gastrointestinal tract; however, purpura is an essential diagnostic criterion. Here, we report an unusual case of IgA vasculitis without purpura in an elderly woman. A 76-year-old woman was admitted to our hospital complaining of diarrhea and abdominal pain. No skin rash, purpura, jaundice, or peripheral lymphadenopathy was observed. Endoscopy of the small intestine revealed severe mucosal sloughing in the duodenum, and a biopsy specimen showed severe erosive duodenitis. A decrease in coagulation factor XIII (FXIII) activity was also observed during laboratory blood tests. IgA immunostaining revealed granular IgA deposition on the walls of the interstitial small blood vessels. Although the patient showed no purpura or renal involvement, a diagnosis of IgA vasculitis was made based on the histopathology findings from biopsies. The administration of purified FXIII concentrate improved her symptoms immediately and facilitated regeneration of the duodenal villi. When gastroenterologists encounter severe erosive duodenitis or inflammation of the small intestine, IgA vasculitis should be listed as part of the differential diagnosis even without purpura and/or renal involvement. For a definitive diagnosis, measurement of FXIII and IgA immunostaining using duodenal biopsy specimens should be performed actively.
Similar content being viewed by others
Abbreviations
- FXIII:
-
Coagulation factor XIII
- IgA:
-
Immunoglobulin A
References
Jennette JC, Falk RJ, Bacon PA, et al. 2012 Revised international chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65:1–11.
Suzuki H, Kiryluk K, Novak J, et al. The pathophysiology of IgA nephropathy. J Am Soc Nephrol. 2011;22(10):1795–803.
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.
Mills JA, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Henoch-Schönlein purpura. Arthritis Rheum. 1990;33(8):1114–21.
Saulsbury FT. Henoch–Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore). 1999;78(6):395–409.
Weiss PF. Pediatric vasculitis. Pediatr Clin North Am. 2012;59(2):407–23.
Kawasaki K, Nakamura S, Esaki M, et al. Gastrointestinal involvement in patients with vasculitis: IgA vasculitis and eosinophilic granulomatosis with polyangiitis. Endosc Int Open. 2019;7(11):E1333-e1343.
Zhang Y, Huang X. Gastrointestinal involvement in Henoch-Schönlein purpura. Scand J Gastroenterol. 2008;43(9):1038–43.
Nishiyama R, Nakajima N, Ogihara A, et al. Endoscope images of Schönlein-Henoch purpura. Digestion. 2008;77(3–4):236–41.
Esaki M, Matsumoto T, Nakamura S, et al. GI involvement in Henoch-Schönlein purpura. Gastrointest Endosc. 2002;56(6):920–3.
Ivaškevičius V, Biswas A, Garly ML, et al. Comparison of F13A1 gene mutations in 73 patients treated with recombinant FXIII-A(2). Haemophilia. 2017;23(3):e194–203.
Dorgalaleh A, Tabibian S, Hosseini MS, et al. Diagnosis of factor XIII deficiency. Hematology. 2016;21(7):430–9.
Murata M, Inatomi O, Ono K, et al. A case of life-threatening small intestinal bleeding accompanied by lower coagulation factor XIII activity. Clin J Gastroenterol. 2020;13(6):1178–82.
Naderi M, Dorgalaleh A, Dorgalaleh S, et al. Clinical manifestations and management of life-threatening bleeding in the largest group of patients with severe factor XIII deficiency. Int J Hematol. 2014;100(5):443–9.
Dalens B, Travade P, Labbé A, et al. Diagnostic and prognostic value of fibrin stabilising factor in Schönlein-Henoch syndrome. Arch Dis Child. 1983;58(1):12–4.
Fukui H, Kamitsuji H, Nagao T, et al. Clinical evaluation of a pasteurized factor XIII concentrate administration in Henoch-Schönlein purpura. Japanese Pediatric Group Thromb Res. 1989;56(6):667–75.
Kawasaki Y. J Suzuki, and H Suzuki, Efficacy of methylprednisolone and urokinase pulse therapy combined with or without cyclophosphamide in severe Henoch-Schoenlein nephritis: a clinical and histopathological study. Nephrol Dial Transplant. 2004;19(4):858–64.
Hosono K, Endo H, Inamori M, et al. Severe large-intestine involvement in adult-onset Henoch-Schönlein purpura: successful treatment with factor XIII concentrate. Digestion. 2008;78(1):9.
Utani A, Ohta M, Shinya A, et al. Successful treatment of adult Henoch-Schönlein purpura with factor XIII concentrate. J Am Acad Dermatol. 1991;24(3):438–42.
Author information
Authors and Affiliations
Contributions
Study concept and design MM. Interpretation of the case all authors. Attending physicians of the patient YY, TS, YO, and KM. Drafting of the manuscript MM and SM. Final approval of the article all authors.
Corresponding author
Ethics declarations
Conflict of interest
All authors declare that they have no conflict of interest in this article.
Human rights
All procedures followed were performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.
Informed consent
Informed consent was obtained from the patient for being included in the study.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Murata, M., Yamazaki, Y., Shimogama, T. et al. Immunoglobulin A vasculitis without purpura in an elderly female patient: a case report. Clin J Gastroenterol 14, 1090–1095 (2021). https://doi.org/10.1007/s12328-021-01422-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12328-021-01422-6