title: Rapidly progressive cryoglobulinemic glomerulonephritis.
abstract: The clinical course of cryoglobulinemic glomerulonephritis (CGGN) is usually slowly progressive, and only a minority of these patients progress to end-stage renal failure. This report describes an atypical case of a patient with CGGN who demonstrated a rapidly progressive and irreversible renal deterioration. After presenting with symptoms of an upper respiratory infection, the patient exhibited oliguric acute renal failure, which was followed by systemic efflorescence accompanied by a fever. The laboratory analyses of his serum revealed the patient to be cryoglobulin-positive with markedly decreased serum C4 levels and an increased anti-streptolysin O titer. A serological test for the hepatitis C virus was negative. A renal biopsy showed that the patient had diffuse endocapillary and extracapillary proliferation together with marked endoluminal thrombi and subendothelial deposits in glomeruli. An electron microscopic examination demonstrated the presence of electron-dense subendothelial, subepithelial and huge intraluminal deposits with a specific annular and cylindrical structure. These features were consistent with active and severe CGGN. Despite aggressive treatment with corticosteroid pulses and a plasma exchange, a second renal biopsy demonstrated further advanced renal injury and revealed no signs of recovery.
authors.affiliation: Division of Kidney and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, 3-25-8 Nishi-Shinbashi, Minato-Ku, 105-8461, Japan. nobuotsuboi@aol.com
authors.forename: Nobuo
authors.initials: N
authors.lastname: Tsuboi
authors.forename: Mayuri
authors.initials: M
authors.lastname: Ichinose
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Kawamura
authors.forename: Kensuke
authors.initials: K
authors.lastname: Joh
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Utsunomiya
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Hosoya
mesh_terms: D001426:Bacterial Proteins / Q000276:immunology; D003449:Cryoglobulinemia / Q000473:pathology / Q000503:physiopathology; D003450:Cryoglobulins / Q000378:metabolism; D005921:Glomerulonephritis / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D007676:Kidney Failure, Chronic / Q000473:pathology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D013301:Streptolysins / Q000276:immunology
chemical_list: D001426:Bacterial Proteins; D003450:Cryoglobulins; D013301:Streptolysins; C025964:streptolysin O