title: A case of slowly progressive scleroderma kidney.
abstract: A rapidly progressive renal deterioration accompanied by acute-onset/uncontrolled hypertension characterizes scleroderma renal crisis (SRC), a life-threatening complication that occurs in patients with systemic sclerosis (SSc). To date, however, SSc with advanced renal failure has only rarely been reported in the absence of SRC. We report here an atypical case of diffuse cutaneous SSc where renal insufficiency progressed slowly to end-stage renal failure over a 14-year follow-up period after the diagnosis of SSc. In the renal biopsy, which was obtained at a relatively early stage of renal impairment, we found histological findings consistent with those of scleroderma kidneys. Unlike typical SRC, however, the larger renal arteries seemed to be unaffected. These histological findings were probably responsible for the "slowly progressive" renal impairment over the years without causing typical SRC.
authors.affiliation: Division of Kidney and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, 3-25-8 Nishi-Shimbashi, Minato-Ku, Tokyo, 105-8461, Japan. mokabe@jikei.ac.jp.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Okabe
authors.affiliation: Division of Kidney and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, 3-25-8 Nishi-Shimbashi, Minato-Ku, Tokyo, 105-8461, Japan.
authors.forename: Nobuo
authors.initials: N
authors.lastname: Tsuboi
authors.affiliation: Division of Kidney and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, 3-25-8 Nishi-Shimbashi, Minato-Ku, Tokyo, 105-8461, Japan.
authors.forename: Takahide
authors.initials: T
authors.lastname: Suzuki
authors.affiliation: Division of Kidney and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, 3-25-8 Nishi-Shimbashi, Minato-Ku, Tokyo, 105-8461, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Yokoo
authors.affiliation: Division of Kidney and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, 3-25-8 Nishi-Shimbashi, Minato-Ku, Tokyo, 105-8461, Japan.
authors.forename: Yoichi
authors.initials: Y
authors.lastname: Miyazaki
authors.affiliation: Division of Kidney and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, 3-25-8 Nishi-Shimbashi, Minato-Ku, Tokyo, 105-8461, Japan.
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Utsunomiya
authors.affiliation: Division of Kidney and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, 3-25-8 Nishi-Shimbashi, Minato-Ku, Tokyo, 105-8461, Japan.
authors.forename: Iwao
authors.initials: I
authors.lastname: Ohno
authors.affiliation: Division of Kidney and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, 3-25-8 Nishi-Shimbashi, Minato-Ku, Tokyo, 105-8461, Japan.
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Hosoya
mesh_terms: D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D018450:Disease Progression; D005260:Female; D006801:Humans; D007668:Kidney / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D008875:Middle Aged; D011507:Proteinuria / Q000209:etiology*; D012595:Scleroderma, Systemic / Q000150:complications* / Q000473:pathology; D012859:Sjogren's Syndrome / Q000150:complications
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors