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Myeloperoxidase-antineutrophil cytoplasmic antibody-related crescentic glomerulonephritis after treatment for clinically amyopathic dermatomyositis: a coincidental combination or not?

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Abstract

A 60-year-old Japanese man exhibited rapidly progressive glomerulonephritis 10 years after receiving prednisolone therapy for clinically amyopathic dermatomyositis (CADM). Upon admission, there were no signs of dermatomyositis. Laboratory analyses revealed the presence of myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA) at 1,280 EU in the absence of anti-glomerular basement membrane antibody and anti-melanoma differentiation-associated gene 5 antibodies, which are typically expressed in CADM. A renal biopsy demonstrated that 14 of 29 glomeruli showed global sclerosis, and the remaining 15 glomeruli exhibited fibrotic and fibrocellular crescent formation without immunoglobulin and complement. Following treatment with 500 mg/day methylprednisolone pulse therapy for 3 days, the patient was started on 30 mg/day of prednisolone orally. On the third day of hospitalization, we began hemodialysis for uremia and anuria with three treatments of plasma exchange starting on the tenth hospital day. Unfortunately, the patient’s renal function did not recover, despite decreases in CRP and MPO-ANCA levels to the normal range. This case is the first English language report of MPO-ANCA-related crescentic glomerulonephritis in a patient who had recovered from CADM.

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Acknowledgments

We thank Dr. Kei Hoshino and Dr. Yoshinao Muro, Division of Connective Tissue Disease and Autoimmunity Department of Dermatology, Nagoya University Graduate School of Medicine, for their analysis of anti-MDA5 antibodies. This work was supported by a grant (to H.I.) from the Progressive Renal Diseases Research Project of the Ministry of Health, Labour and Welfare of Japan.

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None declared.

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Authors and Affiliations

  1. Division of Nephrology and Rheumatology, Department of Internal Medicine, Aichi Medical University School of Medicine, Nagakute-cyo, Aichi, 480-1195, Japan

    Hirohisa Kawai, Wataru Kitagawa, Natsuko Suzuki, Kunihiro Maeda, Keisuke Suzuki, Naoto Miura, Hiroyuki Morita, Syogo Banno, Masahiro Yamamura & Hirokazu Imai

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  1. Hirohisa Kawai
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  2. Wataru Kitagawa
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  3. Natsuko Suzuki
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  4. Kunihiro Maeda
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  5. Keisuke Suzuki
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  6. Naoto Miura
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  7. Hiroyuki Morita
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  8. Syogo Banno
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  9. Masahiro Yamamura
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  10. Hirokazu Imai
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Correspondence to Hirokazu Imai.

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Kawai, H., Kitagawa, W., Suzuki, N. et al. Myeloperoxidase-antineutrophil cytoplasmic antibody-related crescentic glomerulonephritis after treatment for clinically amyopathic dermatomyositis: a coincidental combination or not?. Clin Exp Nephrol 15, 577–581 (2011). https://doi.org/10.1007/s10157-011-0432-2

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  • DOI: https://doi.org/10.1007/s10157-011-0432-2

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