A case of systemic sclerosis with crescentic glomerulonephritis associated with perinuclear-antineutrophil cytoplasmic antibody (p-ANCA)

Shin Ichi Harashima, Shigeru Yoshizawa, Takahiko Horiuchi, Hitoshi Nakashima, Yoshiyuki Niho, Tomohiro Kusaba, Isao Hayashida, Michiya Shinozaki, Ritsuko Katafuchi, Hideki Hirakata

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Abstract

A 50-year-old female with systemic sclerosis (SSc) developed rapidly progressive renal insufficiency. Laboratory findings showed rapid elevation of serum creatinine level (3.8 mg/dl) and a high titer of perinuclear-antineutrophil cytoplasmic antibody (p-ANCA) (504 EU/ml). Renal pathology revealed crescentic glomerulonephritis (CrGN) without mucoid intimai proliferation of the interlobal arteries and fibrinoid necrosis of the afferent arterioles, Immunofluorescent micrography showed focal segmental granular deposition of IgG and C 3 in the mesangium and along the capillary loop and was in agreement with pauci-immune type. Recently, a subtype of renal involvement in SSc that is associated exclusively with normotensive renal failure and recognizable by p-ANCA is suggested. On the other hand, SSc with p-ANCA-positive glomerulonephritis as this case can be considered to be overlap sydrome of SSc and microscopic polyangitis nodosa (microscopic PN) because in microscopic PN, p-ANCA is detected at the range of 50% to 80% and renal pathology reveals necrotizing glomerulonephritis. In this point, we may describe this case as a suggestive one about p-ANCA-positive glomerulonephritis.

Original languageEnglish
Pages (from-to)86-92
Number of pages7
JournalJapanese Journal of Clinical Immunology
Volume22
Issue number2
DOIs
Publication statusPublished - Jan 1 1999

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Antineutrophil Cytoplasmic Antibodies
Systemic Scleroderma
Glomerulonephritis
Kidney
Renal Insufficiency
Pathology
Arterioles
Creatinine
Necrosis
Arteries
Immunoglobulin G
Serum

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Immunology

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A case of systemic sclerosis with crescentic glomerulonephritis associated with perinuclear-antineutrophil cytoplasmic antibody (p-ANCA). / Harashima, Shin Ichi; Yoshizawa, Shigeru; Horiuchi, Takahiko; Nakashima, Hitoshi; Niho, Yoshiyuki; Kusaba, Tomohiro; Hayashida, Isao; Shinozaki, Michiya; Katafuchi, Ritsuko; Hirakata, Hideki.

In: Japanese Journal of Clinical Immunology, Vol. 22, No. 2, 01.01.1999, p. 86-92.

Research output: Contribution to journalArticle

Harashima, SI, Yoshizawa, S, Horiuchi, T, Nakashima, H, Niho, Y, Kusaba, T, Hayashida, I, Shinozaki, M, Katafuchi, R & Hirakata, H 1999, 'A case of systemic sclerosis with crescentic glomerulonephritis associated with perinuclear-antineutrophil cytoplasmic antibody (p-ANCA)', Japanese Journal of Clinical Immunology, vol. 22, no. 2, pp. 86-92. https://doi.org/10.2177/jsci.22.86
Harashima, Shin Ichi ; Yoshizawa, Shigeru ; Horiuchi, Takahiko ; Nakashima, Hitoshi ; Niho, Yoshiyuki ; Kusaba, Tomohiro ; Hayashida, Isao ; Shinozaki, Michiya ; Katafuchi, Ritsuko ; Hirakata, Hideki. / A case of systemic sclerosis with crescentic glomerulonephritis associated with perinuclear-antineutrophil cytoplasmic antibody (p-ANCA). In: Japanese Journal of Clinical Immunology. 1999 ; Vol. 22, No. 2. pp. 86-92.
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