ArticleOtolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery2011
Cryopyrin-associated periodic syndromes: otolaryngologic and audiologic manifestations.
Article in Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers, 1 of them a synthesis that pooled it.
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Who cites it
39 citing papers in PubMed, 1 synthesis or guideline pooled it, 91 citations in OpenAlex.
- The 2021 EULAR/American College of Rheumatology Points to Consider for Diagnosis, Management and Monitoring of the Interleukin-1 Mediated Autoinflammatory Diseases: Cryopyrin-Associated Periodic Syndromes, Tumour Necrosis Factor Receptor-Associated Periodic Syndrome, Mevalonate Kinase Deficiency, and Deficiency of the Interleukin-1 Receptor Antagonist.Arthritis & rheumatology (Hoboken, N.J.) · 2022Pooled it
- A case of cryopyrin-associated periodic syndrome presenting with abdominal symptoms due to a novel mutation NLRP3 p.Ile257Met.Clinical journal of gastroenterology · 2026Article
- Advances in the Study of NOD-Like Receptors in Common Otological Diseases.The journal of international advanced otology · 2025Review
- Targeting the NLRP3 inflammasome for inflammatory disease therapy.Trends in pharmacological sciences · 2025Review
- Anakinra authorized to treat severe coronavirus disease 2019; Sepsis breakthrough or time to reflect?Frontiers in microbiology · 2023Article
- Mitochondrial dysfunction in hearing loss: Oxidative stress, autophagy and NLRP3 inflammasome.Frontiers in cell and developmental biology · 2023Review
- Systemic autoinflammatory diseases in pediatric population.Asia Pacific allergy · 2022Article
- Auditory Phenotype and Histopathologic Findings of a Mutant Nlrp3 Expression Mouse Model.Frontiers in neurology · 2022Article
- Hearing Loss as the Main Clinical Presentation inFrontiers in immunology · 2022Article
- Auditory and Vestibular Characteristics of NLRP3 Inflammasome Related Autoinflammatory Disorders: Monogenic Hearing Loss Can Be Improved by Anti-interleukin-1 Therapy.Frontiers in neurology · 2022Review
- The Interactions Between Autoinflammation and Type 2 Immunity: From Mechanistic Studies to Epidemiologic Associations.Frontiers in immunology · 2022Review
- NLRP3 inflammasome and NLRP3-related autoinflammatory diseases: From cryopyrin function to targeted therapies.Frontiers in immunology · 2022Review
- Construction of an MRI-based decision tree to differentiate autoimmune and autoinflammatory inner ear disease from chronic otitis media with sensorineural hearing loss.Scientific reports · 2021Article
- Article
- Diagnosis and Management of the Cryopyrin-Associated Periodic Syndromes (CAPS): What Do We Know Today?Journal of clinical medicine · 2021Review
- Allergic Aspects of IgG4-Related Disease: Implications for Pathogenesis and Therapy.Frontiers in immunology · 2021Review
- Review
- Genetic Hearing Loss Associated With Autoinflammation.Frontiers in neurology · 2020Review
- Current Therapeutic Options for the Main Monogenic Autoinflammatory Diseases and PFAPA Syndrome: Evidence-Based Approach and Proposal of a Practical Guide.Frontiers in immunology · 2020Review
- Magnetic resonance imaging findings in a patient with cryopyrin-associated periodic syndrome: A rare hereditary multi-system inflammatory disorder.The neuroradiology journal · 2019Article
Corrections and comments
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Authors and funding
9 authors at 5 institutions in 1 country.
Funding
Abstract
objectiveCryopyrin-associated periodic syndromes (CAPS) represent a spectrum of CIAS1 gene-mediated autoinflammatory diseases characterized by recurrent systemic inflammation. The clinical spectrum of CAPS varies from mild to severe and includes the syndromes historically described as familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (NOMID). This article presents the largest cohort of patients with CAPS. The objective is to describe the pathogenesis, otolaryngologic, and audiologic manifestations of CAPS. STUDY
designProspective (2003-2009).
settingNational Institutes of Health. SUBJECTS AND
methodsFifty-seven patients with a diagnosis of CAPS were identified (31 NOMID, 11 NOMID/MWS, 9 MWS, and 6 FCAS). Comprehensive data regarding clinical manifestations, audiologic phenotype, and fluid attenuation inversion recovery MRI (FLAIR-MRI) of the brain and inner ear were obtained.
resultsComplete audiologic data obtained on 70% of ears revealed conductive hearing loss in 4 (11%) NOMID ears and mixed hearing loss in 5 (13%) NOMID and 2 (14%) NOMID/MWS ears. Sensorineural hearing loss (SNHL), worse in higher frequencies, was the most common type of hearing loss and was present in 23 (61%) NOMID, 10 (71%) NOMID/MWS, and 4 (33%) MWS ears. All of the patients with FCAS had normal hearing except 2, who had SNHL from 4 to 8 kHz. On FLAIR-MRI sequence, cochlear enhancement was noted in 26 of 29 (90%) NOMID, 6 of 11 (55%) NOMID/MWS, 3 of 9 (33%) MWS, and 1 of 6 (17%) FCAS patients and was significantly associated with the presence of hearing loss. Maxillary sinus hypoplasia and mucosal thickening were found in 39% and 86% of the cohort, respectively.
conclusionCIAS1 pathway–mediated CAPS is associated with unregulated autoinflammation mediated by interleukin-1 in the cochlea and hearing loss. Timely diagnosis is crucial to initiate early treatment with interleukin-1 receptor antagonists.
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Registered trials
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