title: Childhood Cogan syndrome with aortitis and anti-neutrophil cytoplasmic antibody-associated glomerulonephritis.
abstract: Cogan syndrome is a systemic disease manifesting interstitial keratitis, sensorineural hearing loss, tinnitus, and rotatory vertigo. Renal complications of this syndrome are very rare. We encountered an adolescent with Cogan syndrome complicated by aortitis and anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis. At the age of 14, the patient showed proteinuria in a screening urinalysis at school and was found to lack a right radial pulse. Magnetic resonance angiography disclosed right subclavian artery stenosis. Examination of a renal biopsy specimen showed ANCA-positive crescentic glomerulonephritis. Steroid and immunosuppressant treatment improved renal function and histopathology, but repeated recurrences followed. At 18, the patient developed rotatory vertigo, a sense of ear fullness, and sensorineural hearing loss. The patient was diagnosed with Cogan syndrome. We know of no previous description of ANCA-positive crescentic glomerulonephritis in children with Cogan syndrome. Accordingly, evaluation of aortitis in childhood should include not only otolaryngologic and ophthalmologic examinations, but also periodic urine examination and renal function tests.
authors.affiliation: Department of Pediatrics, Kinki University Faculty of Medicine, 377-2 Ohno-higashi, Osaka-Sayama 589-8511, Japan.
authors.forename: Keisuke
authors.initials: K
authors.lastname: Sugimoto
authors.affiliation: Department of Pediatrics, Kinki University Faculty of Medicine, 377-2 Ohno-higashi, Osaka-Sayama 589-8511, Japan.
authors.forename: Tomoki
authors.initials: T
authors.lastname: Miyazawa
authors.affiliation: Department of Pediatrics, Kinki University Faculty of Medicine, 377-2 Ohno-higashi, Osaka-Sayama 589-8511, Japan.
authors.forename: Hitomi
authors.initials: H
authors.lastname: Nishi
authors.affiliation: Department of Pediatrics, Kinki University Faculty of Medicine, 377-2 Ohno-higashi, Osaka-Sayama 589-8511, Japan.
authors.forename: Akane
authors.initials: A
authors.lastname: Izu
authors.affiliation: Department of Pediatrics, Kinki University Faculty of Medicine, 377-2 Ohno-higashi, Osaka-Sayama 589-8511, Japan.
authors.forename: Takuji
authors.initials: T
authors.lastname: Enya
authors.affiliation: Department of Pediatrics, Kinki University Faculty of Medicine, 377-2 Ohno-higashi, Osaka-Sayama 589-8511, Japan.
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Okada
authors.affiliation: Department of Pediatrics, Kinki University Faculty of Medicine, 377-2 Ohno-higashi, Osaka-Sayama 589-8511, Japan.
authors.forename: Tsukasa
authors.initials: T
authors.lastname: Takemura
mesh_terms: D000293:Adolescent; D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D018501:Antirheumatic Agents / Q000008:administration & dosage; D001025:Aortitis / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D001241:Aspirin / Q000008:administration & dosage*; D002386:Cataract / Q000175:diagnosis*; D055952:Cogan Syndrome / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D016572:Cyclosporine / Q000008:administration & dosage*; D018450:Disease Progression; D005921:Glomerulonephritis / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis*; D006801:Humans; D007668:Kidney / Q000473:pathology; D007677:Kidney Function Tests; D018810:Magnetic Resonance Angiography; D008297:Male; D011239:Prednisolone / Q000008:administration & dosage*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D018501:Antirheumatic Agents; D016572:Cyclosporine; D011239:Prednisolone; D001241:Aspirin
keywords: Childhood onset; Proteinuria; Sensorineural hearing loss; Steroid therapy; Tubulointerstitial nephropathy; Vertigo