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.

Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis Complicated by Periaortitis and Cranial Hypertrophic Pachymeningitis: A Report of an Autopsy Case

“…Although Chirinos et al ( 3 ) found that cases of large-vessel involvement in AAV were reported mostly from Europe and North America, the number of reports on large-vessel involvement in AAV from Japan has recently been increasing ( 6 , 8 - 11 , 13 ). Ozaki et al ( 13 ) reviewed 24 cases of GPA-associated large-vessel involvement reported in 1990-2015.…”

Optimal management of Cogan’s syndrome: a multidisciplinary approach

“… 60 , 63 The majority of the described patients receiving pulse intravenous Cyc combined with oral glucocorticoids with a clinical stabilization of CS. Nevertheless, some articles described only a partial or short-term response, 46 , 59 , 60 , 64 , 65 or a lack of response; 12 , 15 , 60 , 66 , 67 in some cases, discontinuation was warranted due to systemic side effects. 60 , 63 …”

“…CyA is a cyclic peptide, which binds to cyclophilin of T lymphocytes; thus, CyA is able to reduce the release of several cytokines. The use of CyA combined with corticosteroid and other immunosuppressive agents in CS was documented in some case reports, 59 , 63 , 65 , 76 , 80 two case series, 60 , 64 and one prospective nonrandomized clinical study including four patients with typical CS. 81 The efficacy was variable with good response for ocular manifestations, quite effective for possible systemic vascular involvement but less effective for the audio-vestibular symptoms.…”

“…Copyright © 2014 Sugimoto et al; licensee BioMed Central Ltd. Creative Commons License available at: https://creativecommons.org/licenses/by/2.0/ . 65 …”