title: Anti-RNA polymerase III antibody-associated scleroderma renal crisis in a patient with limited cutaneous systemic sclerosis: A case report.
abstract: A 69-year-old Japanese man was presented with hypertensive crisis. Renal histology revealed malignant nephrosclerosis, including an onion skin pattern with fibrinoid necrosis of the small arteries from arterioles up to interlobular arteries. Immunological investigation clarified positive anti-RNA polymerase (RNAP) III antibody, and limited cutaneous systemic sclerosis (Lc SSc) was diagnosed by skin biopsy as the underlying disease causing scleroderma renal crisis (SRC). Angiotensin covering enzyme (ACE) inhibitor therapy and calcium antagonist were effective for his renal condition. Although an association between SRC and anti-RNAP III antibody has already been reported in patients with diffuse cutaneous SSc (Dc SSc), this case indicates that SRC with hypetensive emergency with malignant nephrosclerosis can also be diagnosed on patients with Lc SSc patients by the examination of anti-RNAP III antibody.
authors.affiliation: a Nephrology Center, Toranomon Hospital , Japan.
authors.forename: Daisuke
authors.initials: D
authors.lastname: Takada
authors.affiliation: a Nephrology Center, Toranomon Hospital , Japan.
authors.forename: Junichi
authors.initials: J
authors.lastname: Hoshino
authors.affiliation: a Nephrology Center, Toranomon Hospital , Japan.
authors.forename: Koichi
authors.initials: K
authors.lastname: Kikuchi
authors.affiliation: a Nephrology Center, Toranomon Hospital , Japan.
authors.forename: Junko
authors.initials: J
authors.lastname: Yabuuchi
authors.affiliation: a Nephrology Center, Toranomon Hospital , Japan.
authors.forename: Yuta
authors.initials: Y
authors.lastname: Kogure
authors.affiliation: a Nephrology Center, Toranomon Hospital , Japan.
authors.forename: Toshiharu
authors.initials: T
authors.lastname: Ueno
authors.affiliation: a Nephrology Center, Toranomon Hospital , Japan.
authors.forename: Akinari
authors.initials: A
authors.lastname: Sekine
authors.affiliation: a Nephrology Center, Toranomon Hospital , Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Yamanouchi
authors.affiliation: a Nephrology Center, Toranomon Hospital , Japan.
authors.forename: Keiichi
authors.initials: K
authors.lastname: Sumida
authors.affiliation: a Nephrology Center, Toranomon Hospital , Japan.
authors.forename: Koki
authors.initials: K
authors.lastname: Mise
authors.affiliation: a Nephrology Center, Toranomon Hospital , Japan.
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Suwabe
authors.affiliation: a Nephrology Center, Toranomon Hospital , Japan.
authors.forename: Noriko
authors.initials: N
authors.lastname: Hayami
authors.affiliation: a Nephrology Center, Toranomon Hospital , Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Sawa
authors.affiliation: a Nephrology Center, Toranomon Hospital , Japan.|b The Okinaka Memorial Institute for Medical Research, Toranomon Hospital , Japan.
authors.forename: Kenmei
authors.initials: K
authors.lastname: Takaichi
authors.affiliation: c Department of Dermatology , Toranomon Hospital , Japan , and.
authors.forename: Nobukazu
authors.initials: N
authors.lastname: Hayasi
authors.affiliation: d Department of Pathology , Toranomon Hospital , Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Fujii
authors.affiliation: d Department of Pathology , Toranomon Hospital , Japan.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Ohashi
authors.affiliation: a Nephrology Center, Toranomon Hospital , Japan.|b The Okinaka Memorial Institute for Medical Research, Toranomon Hospital , Japan.
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Ubara
mesh_terms: D000368:Aged; D000906:Antibodies / Q000276:immunology; D006801:Humans; D008297:Male; D009400:Nephrosclerosis / Q000209:etiology* / Q000276:immunology; D009401:Nephrosis / Q000209:etiology* / Q000276:immunology; D012320:RNA Polymerase III / Q000276:immunology*; D012595:Scleroderma, Systemic / Q000150:complications* / Q000276:immunology / Q000473:pathology; D012867:Skin / Q000473:pathology
chemical_list: D000906:Antibodies; D012320:RNA Polymerase III
keywords: Anti-RNA polymerase III antibody; Diffuse cutaneous systemic sclerosis; Hypertensive emergency; Limited cutaneous systemic sclerosis; Malignant nephrosclerosis; Modified Rodnan TSS score; Scleroderma renal crisis