TY - JOUR
T1 - Simultaneous development of IgA vasculitis and eosinophilic granulomatosis with polyangiitis
AU - Asano, Yosuke
AU - Matsumoto, Yoshinori
AU - Miyazaki, Tatsuhiko
AU - Ishizu, Akihiro
AU - Morizane, Shin
AU - Hayashi, Keigo
AU - Yamamura, Yuriko
AU - Hiramatsu, Sumie
AU - Miyawaki, Yoshia
AU - Morishita, Michiko
AU - Ohashi, Keiji
AU - Watanabe, Haruki
AU - Watanabe, Katsue Sunahori
AU - Kawabata, Tomoko
AU - Sada, Kenei
AU - Makino, Hirofumi
AU - Wada, Jun
N1 - Funding Information:
This work was supported by the Ministry of Health, Labour and Welfare, Japan under Research on Rare and Intractable Diseases [H29-nannti-ippan-018]; and the Japan Agency for Medical Research and Development, AMED under the study group for strategic exploration of drug seeds for ANCA-associated vasculitis and construction of clinical evidence [17ek0109104h0003 and 17ek0109121h0003].
Publisher Copyright:
© 2019, © 2019 Japan College of Rheumatology.
PY - 2020/1/2
Y1 - 2020/1/2
N2 - Immunoglobulin A (IgA) vasculitis (IgAV) is a small vessel vasculitis presenting cutaneous purpura, arthralgias and/or arthritis, acute enteritis and glomerulonephritis caused by deposition of the IgA1-mediated immune complex. Eosinophilic granulomatosis with polyangiitis (EGPA) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) characterised by eosinophil-rich and granulomatous inflammation in small to medium-sized vessels. Both IgAV and EGPA are classified as autoimmune systemic vasculitis, but the pathogenesis of immune complex-mediated IgAV and that of pauci-immune EGPA are different. Here we report a rare case of simultaneous development of IgAV and EGPA presenting palpable purpura and numbness in a patient with a history of asthma. Histological examination revealed leukocytoclastic vasculitis with deposition of IgA, IgM and C3 in the upper dermis and necrotising vasculitis with eosinophilic infiltration and granulomatous formation in the lower dermis and subcutaneous fat, indicating the existence of IgAV and EGPA. Our case provides evidence of concurrent development of two different types of vasculitis, which may affect disease-associated complications, therapeutic strategy and prognosis.
AB - Immunoglobulin A (IgA) vasculitis (IgAV) is a small vessel vasculitis presenting cutaneous purpura, arthralgias and/or arthritis, acute enteritis and glomerulonephritis caused by deposition of the IgA1-mediated immune complex. Eosinophilic granulomatosis with polyangiitis (EGPA) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) characterised by eosinophil-rich and granulomatous inflammation in small to medium-sized vessels. Both IgAV and EGPA are classified as autoimmune systemic vasculitis, but the pathogenesis of immune complex-mediated IgAV and that of pauci-immune EGPA are different. Here we report a rare case of simultaneous development of IgAV and EGPA presenting palpable purpura and numbness in a patient with a history of asthma. Histological examination revealed leukocytoclastic vasculitis with deposition of IgA, IgM and C3 in the upper dermis and necrotising vasculitis with eosinophilic infiltration and granulomatous formation in the lower dermis and subcutaneous fat, indicating the existence of IgAV and EGPA. Our case provides evidence of concurrent development of two different types of vasculitis, which may affect disease-associated complications, therapeutic strategy and prognosis.
KW - ANCA-associated vasculitis
KW - EGPA
KW - IgA vasculitis
KW - simultaneous development
KW - systemic vasculitis
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U2 - 10.1080/24725625.2019.1673528
DO - 10.1080/24725625.2019.1673528
M3 - Article
C2 - 33086980
AN - SCOPUS:85135799716
SN - 2472-5625
VL - 4
SP - 63
EP - 69
JO - Modern Rheumatology Case Reports
JF - Modern Rheumatology Case Reports
IS - 1
ER -