Vasculitis and Cogan’s Syndrome

Vollertsen, Randall S.

doi:10.1016/s0889-857x(21)01068-1

Cited by 65 publications

(3 citation statements)

References 21 publications

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“… 10 Very few of patients may have positive rheumatoid factor, anti-neutrophil cytoplasmic antibodies, and anti-nuclear antibody spectra. 11 Currently, there are only 300 literatures related to CS, most of which are case reports or case series. The diagnosis of CS mainly relies on its characteristic clinical manifestations and the exclusion of other diseases, such as viral infection, tuberculosis, Wegener’s granulomatosis with polyangiitis, Behçet, Vogt–Koyanagi–Harada and malignant disease.…”

Section: Discussionmentioning

confidence: 99%

Cogan’s Syndrome Combined with Hypertrophic Pachymeningitis: A Case Report

Lu,

Lv,

Zhu

et al. 2024

JIR

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Cogan’s syndrome (CS) is a rare chronic inflammatory disease, characterized by interstitial keratitis and vestibular auditory dysfunction. Hypertrophic pachymeningitis (HP) is a rare chronic aseptic inflammatory disease of the central nervous system. This article reports a patient with CS coexisting with HP. The patient was a 66-year-old male with fever, headache, red eyes, hearing loss, and significantly elevated inflammatory markers. Cerebrospinal fluid examination, blood culture, and tests for autoantibodies such as antinuclear antibodies were negative. Pure tone audiology (PTA) indicated bilateral sensorineural deafness. Both Positron emission tomography–computed tomography (PET/CT) and vascular color Doppler ultrasound suggest the presence of vasculitis. Considering Cogan’s syndrome, the patient received 40 mg of methylprednisolone intravenously once daily. The brain’s magnetic resonance imaging (MRI) revealed slightly thickened and enhanced dura mater, suggesting HP. The dose of methylprednisolone was increased to 40 mg intravenously every 8 hours, leading to the patient’s improved symptoms and decreased inflammatory markers. Both CS and HP are rare chronic inflammatory diseases, and their coexistence is even rarer, with only two reported cases in literature up to date. The coexistence of CS and HP should be considered when the CS patients with headaches do not respond well to treatment.

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Section: Discussionmentioning

confidence: 99%

Cogan’s Syndrome Combined with Hypertrophic Pachymeningitis: A Case Report

Lu,

Lv,

Zhu

et al. 2024

JIR

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“…The ocular condition is characterized by interstitial keratitis which causes vision impairment, conjunctivitis and inflammation around the eye, as well as an increase in light sensitivity. Interstitial keratitis can coexist with syphilitic disease, which is often caused by a combination of bacteria and viruses associated with systemic vasculitides (46,47). Viruses such as Herpes simplex virus (HSV), Epstein Barr virus (EBV) and Rubulavirus cause viral infections presented in corneal disease.…”

Section: Cogan's Syndromementioning

confidence: 99%

Association of human gut microbiota with rare diseases: A close peep through

Roy

Nag

Saini

et al. 2022

IRDR

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disorders, human gut microbiome, personalized therapeutics, rare diseasesThe human body harbors approximately 10 14 cells belonging to a diverse group of microorganisms. Bacteria outnumbers protozoa, fungi and viruses inhabiting our gastrointestinal tract (GIT), commonly referred to as the "human gut microbiome". Dysbiosis occurs when the balanced relationship between the host and the gut microbiota is disrupted, altering the usual microbial population there. This increases the susceptibility of the host to pathogens, and chances of its morbidity. It is due to the fact that the gut microbiome plays an important role in human health; it influences the progression of conditions varying from colorectal cancer to GIT disorders linked with the nervous system, autoimmunity, metabolism and inheritance. A rare disease is a lethal and persistent condition affecting 2-3 people per 5,000 populaces. This review article intends to discuss such rare neurological, autoimmune, cardio-metabolic and genetic disorders of man, focusing on the fundamental mechanism that links them with their gut microbiome. Ten rare diseases, including Pediatric Crohn's disease (PCD), Lichen planus (LP), Hypophosphatasia (HPP), Discitis, Cogan's syndrome, Chancroid disease, Sennetsu fever, Acute cholecystitis (AC), Grave's disease (GD) and Tropical sprue (TS) stands to highlight as key examples, along with personalized therapeutics meant for them. This medicinal approach addresses the individual's genetic and genomic pathography, and tackles the illness with specific and effective treatments.

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“…The auditory symptoms in Cogan's syndrome are classically similar to those observed in cases of Meniere's syndrome. Without treatment, Cogan's syndrome could become severe, resulting in sensorineural hearing loss in over 50% of the patients [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

Atypical Cogan’s Syndrome with Choroidal Effusion: A Case Report

Oh¹,

Lee²,

Hwang³

2022

Case Rep Ophthalmol

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Cogan’s syndrome is a rare form of autoimmune vasculitis that manifests with ocular and audiovestibular symptoms. The clinical presentations of Cogan’s syndrome can be classified as typical and atypical, and the auditory symptoms are classically similar to those seen in cases of Meniere’s syndrome. Without treatment, Cogan’s syndrome could become severe, resulting in sensorineural hearing loss in over 50% of patients. This report describes a rare case of uncontrolled intraocular pressure and choroidal effusion in a patient with atypical Cogan’s syndrome. A 51-year-old woman was referred to our clinic on account of bilateral distortion of visual acuity and ocular pain following admission to the internal medicine department for bilateral hearing loss, acute renal failure, and fever. Upon ocular examination, we observed bilateral 2–3+ cells in the anterior chamber and closed-angle glaucoma, and choroidal effusion in the right eye. The intraocular pressure could not be controlled with topical and systemic medications, and a bilateral valve implant surgery was performed subsequently. Two weeks later, after systemic corticosteroid therapy, the choroidal effusion resolved. Cogan’s syndrome can provoke angle-closure glaucoma with choroidal effusion in patients with a short axial length and shallow anterior chamber.

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Vasculitis and Cogan’s Syndrome

Cited by 65 publications

References 21 publications

Cogan’s Syndrome Combined with Hypertrophic Pachymeningitis: A Case Report

Cogan’s Syndrome Combined with Hypertrophic Pachymeningitis: A Case Report

Association of human gut microbiota with rare diseases: A close peep through

Atypical Cogan’s Syndrome with Choroidal Effusion: A Case Report

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