Cogan’s Syndrome: Clinical Presentations and Update on Treatment

Wheeler, J. Gary; Temprano, Katherine K; Keller, Angela Prost

doi:10.1007/s11882-020-00945-1

Cited by 34 publications

(25 citation statements)

References 26 publications

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“…Although our patient was diagnosed with CS after typical oculovestibular symptoms of uveitis and hearing loss, he had already progressed to have systemic illness in the form of inflammatory arthritis. Reports of systemic manifestations in CS vary but can be as high 66% to 80% of patients [ 1 , 3 ]. Cardiovascular, neurologic, and gastrointestinal involvement have all been reported in such cases.…”

Section: Discussionmentioning

confidence: 99%

“…Cogan syndrome (CS) is a rare autoimmune disorder, with approximately 300 cases reported in the current literature [ 1 ]. Although patients can present at any age, this disease is most frequently seen in younger adults from 30 to 40 years of age [ 1 ]. It is associated most frequently with ocular, vestibular, and auditory involvement from presumed small-vessel vasculitis.…”

Section: Introductionmentioning

confidence: 99%

“…Vogt-Koyanagi-Harada syndrome (uveitis, alopecia, poliosis, plus vitiligo), Susac syndrome (retinocochleocerebral vasculopathy), and congenital syphilis are possible differential diagnostic considerations for CS [ 2 ]. Various autoimmune diseases, however, have also been implicated in a minority (8–10%) of patients with CS, including Wegener’s granulomatosis, sarcoidosis, Takayasu arteritis, polyarteritis nodosa, rheumatoid arthritis, and inflammatory bowel disease [ 1 ]. Large vessel vasculitis, specifically aortitis, has been observed in up to 10% of patients with CS [ 1 ], but acute limb ischemia in the setting of this illness has been reported in only a few case reports.…”

Section: Introductionmentioning

confidence: 99%

“…Various autoimmune diseases, however, have also been implicated in a minority (8–10%) of patients with CS, including Wegener’s granulomatosis, sarcoidosis, Takayasu arteritis, polyarteritis nodosa, rheumatoid arthritis, and inflammatory bowel disease [ 1 ]. Large vessel vasculitis, specifically aortitis, has been observed in up to 10% of patients with CS [ 1 ], but acute limb ischemia in the setting of this illness has been reported in only a few case reports. Treatment of patients presenting with serious systemic vasculitis typically requires a multi-disciplinary approach with considerations for medical and surgical therapies; aortic valve replacement, for example, has been described in severe aortitis and aortic insufficiency [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

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Acute Limb Ischemia in Cogan Syndrome

Mohseni

2022

Am J Case Rep

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Patient: Male, 50-year-old Final Diagnosis: Cogan’s Syndrome • vascular ischemia • vasculitis Symptoms: Change in extremity temperature • extremity pain • pulselessness Medication: — Clinical Procedure: — Specialty: General and Internal Medicine • Rheumatology Objective: Rare disease Background: Cogan syndrome is a rare autoimmune disorder associated most frequently with ocular, vestibular, and auditory involvement from presumed small vessel vasculitis. Cogan syndrome, in a significant proportion of patients, can progress to systemic symptoms, including gastrointestinal, neurologic, and musculoskeletal manifestations. Large-vessel involvement has also been described in some cases (eg, aortitis), but acute limb ischemia in the setting of this illness has been infrequently reported. Case Report: We present a rare case of Cogan syndrome complicated by acute vascular ischemia of the left upper extremity. A 50-year-old man presented with symptoms of severe acute pain and weakness of the left arm. The patient endorsed a diagnosis of Cogan syndrome 4 years prior in the setting of unilateral left-sided hearing loss and bilateral uveitis. A physical examination revealed pallor of the left forearm and pulselessness at the wrist. Computed tomography angiography was suggestive of vasculitis and concerns for embolic occlusion of several arterial structures of the left upper limb. After consultation with various specialists, the patient was treated with high-dose steroids, anticoagulants, and topical nitroglycerin and experienced significant clinical improvement. Conclusions: Treatment of Cogan syndrome with severe systemic manifestations depends on the organ involvement and degree of extension. Our patient’s presentation serves as an impressive example of systemic vasculitis with subsequent acute ischemia in the setting of this rare autoimmune disorder. In such a case, given the potential for life- or limb-threatening systemic vascular catastrophes, emergent interventions (including imaging, anticoagulation, and specialist involvement) are required to prevent untoward outcomes.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Acute Limb Ischemia in Cogan Syndrome

Mohseni

2022

Am J Case Rep

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“…The hallmark of CS is the presence of ocular and audiovestibular symptoms. 10 , 32 , 33 These findings may be accompanied by evidence of systemic vasculitis. 10 , 32 The most common ocular symptoms include interstitial keratitis, uveitis, and episcleritis.…”

Section: Differential Diagnosismentioning

confidence: 99%

Immune-Mediated Inner Ear Disease Associated with Type 1 Autoimmune Hepatitis: A Challenging Coexistence

Zarachi

Pelechas

Tsikou

et al. 2022

MJR

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Autoimmune hepatitis (AIH) is characterized by elevated serum transaminase, increased immunoglobulin G levels, presence of autoantibodies, and hepatocellular damage. Coexistence with other autoimmune diseases has been reported in almost half of patients with AIH. Here, we report a 60-year-old man who developed rapidly progressive, bilateral, asymmetrical, and asynchronous sensorineural hearing loss that was consistent with immune-mediated inner ear disease (IMIED). This devastating presentation evolved as a late manifestation in the context of a six-month systemic illness that had previously resulted in type 1 AIH. A biochemical remission with normalization of aminotransferases achieved within two months after the initiation of corticosteroids with azathioprine. Further, an acceptable response has also been achieved at the patient regarding the right ear-hearing impairment; though, treatment could not reverse the substantial decrement in hearing capability of the left ear. To our knowledge, this is the first case report of the concurrent development of type 1 AIH and IMIED.

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