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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.
AuthorsKeisuke Sugimoto, Tomoki Miyazawa, Hitomi Nishi, Akane Izu, Takuji Enya, Mitsuru Okada, Tsukasa Takemura
JournalPediatric rheumatology online journal (Pediatr Rheumatol Online J) Vol. 12 Pg. 15 ( 2014) ISSN: 1546-0096 [Print] England
PMID24803850 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Antibodies, Antineutrophil Cytoplasmic
  • Antirheumatic Agents
  • Cyclosporine
  • Prednisolone
  • Aspirin
Topics
  • Adolescent
  • Adult
  • Antibodies, Antineutrophil Cytoplasmic (blood)
  • Antirheumatic Agents (administration & dosage)
  • Aortitis (diagnosis, drug therapy, physiopathology)
  • Aspirin (administration & dosage)
  • Cataract (diagnosis)
  • Cogan Syndrome (blood, diagnosis, drug therapy, physiopathology)
  • Cyclosporine (administration & dosage)
  • Disease Progression
  • Glomerulonephritis (diagnosis, drug therapy, physiopathology)
  • Hearing Loss, Sensorineural (diagnosis)
  • Humans
  • Kidney (pathology)
  • Kidney Function Tests
  • Magnetic Resonance Angiography
  • Male
  • Prednisolone (administration & dosage)

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