ReviewSeminars in immunopathology2015
CAPS--pathogenesis, presentation and treatment of an autoinflammatory disease.
Review in Seminars in immunopathology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 71 papers, 2 of them syntheses that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
71 citing papers in PubMed, 2 syntheses or guidelines pooled it, 137 citations in OpenAlex.
- Low-dose corticosteroids in severe pulmonary infection: a meta-analysis of randomised controlled trials.BMJ open respiratory research · 2026Pooled it
- Emerging treatments for dermatologic diseases in infants, children, and adolescents: a systematic review of clinical trials on biologics and small molecule inhibitors.Inflammopharmacology · 2025Pooled it
- Rapid and Sustained Long-Term Efficacy and Safety of Canakinumab in Patients With Cryopyrin-Associated Periodic Syndrome Ages Five Years and Younger.Arthritis & rheumatology (Hoboken, N.J.) · 2019Trial
- Bilateral globus pallidus internus deep brain stimulation in medication-refractory dystonia secondary to infantile acute disseminated encephalomyelitis: illustrative case.Journal of neurosurgery. Case lessons · 2026Article
- The Immunopathogenesis of uveitis.Frontiers in medicine · 2026Review
- NLRP3 inflammasome and hearing loss: from mechanisms to therapies.Journal of neuroinflammation · 2025Review
- Schnitzler syndrome.Dermatologie (Heidelberg, Germany) · 2025Review
- IL1RAP Expression in Human Atherosclerosis: A Target of Novel Antibodies to Reduce Vascular Inflammation and Adhesion.Journal of the American Heart Association · 2025Article
- Neutrophilic Urticarial Dermatosis: A Window into Systemic Inflammation and Autoimmune Disorders.Clinical reviews in allergy & immunology · 2025Review
- From fat to fire: The lipid-inflammasome connection.Immunological reviews · 2025Review
- Supersulfide donors and their therapeutic targets in inflammatory diseases.Frontiers in immunology · 2025Review
- Case Report: A heterozygous mutation of NLRP3 in a Chinese child with NLRP3-AID.Frontiers in pediatrics · 2025Article
- Adult onset cryopyrin-associated periodic syndrome due to germline missense mutation inFrontiers in immunology · 2025Article
- Management of a Patient with Cardiovascular Disease Should Include Assessment of Primary and Secondary Immunodeficiencies: Part 1-Primary Immunodeficiencies.Healthcare (Basel, Switzerland) · 2024Review
- Adult patient diagnosed with Muckle-Wells syndrome, antiphospholipid syndrome and glomerular haematuria.BMJ case reports · 2024Article
- IL-1 Family Blockade in Cytokine Storm Syndromes.Advances in experimental medicine and biology · 2024Review
- Clinical and Genetic Spectrum of Nine Cases of NLRP3-Associated Autoinflammatory Disease (NLRP3-AID) and Identification of One Novel NLRP3 Mutation by Genetic Variation Analyses.Journal of immunology research · 2024Article
- Uveitis Associated with Monogenic Autoinflammatory Syndromes in Children.Ocular immunology and inflammation · 2023Review
- The phenotype and genotype of Chinese adult patients with NLRP3-associated autoinflammatory disease.Clinical rheumatology · 2023Article
- NLRP3 Inflammasome in Atherosclerosis: Putting Out the Fire of Inflammation.Inflammation · 2023Review
11 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
1 author at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The cryopyrin-associated periodic syndrome (CAPS) is a severity spectrum of rare diseases. CAPS comprises the three conditions previously described as familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disorder (NOMID), also known as chronic infantile neurologic, cutaneous, and articular (CINCA) syndrome. The clinical phenotype of CAPS is characterized by systemic inflammation. General symptoms are fatigue and fever. Local manifestations affect multiple tissues such as skin, joints, muscles, eyes, and the central nervous system. Distinct clinical features are characteristic for each subphenotype. In FCAS, these are cold-induced urticaria and fever, in MWS systemic amyloidosis and hearing loss and in NOMID/CINCA central nervous system inflammation and bone deformities. CAPS is caused by single heterozygous germline or somatic gain of function mutations in the NLRP3 gene encoding the protein cryopyrin. Cryopyrin nucleates an NLRP3 inflammasome, which regulates the activation and cleavage of caspase-1 that cleaves the pro-inflammatory cytokines, IL-1β and IL-18. IL-1β plays the key role in the induction of inflammation in CAPS. This has been confirmed by the application of IL-1 blocking agents, which lead not only to a rapid and sustained reversal of daily symptoms but also to some extent of long-term disease sequelae. To prevent CAPS-induced organ damage, early diagnosis and swift initiation of effective treatment are mandatory.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.