ArticleJCI insight2016
Anakinra as a diagnostic challenge and treatment option for systemic autoinflammatory disorders of undefined etiology.
Article in JCI insight, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers, 1 of them a synthesis that pooled it.
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Who cites it
33 citing papers in PubMed, 1 synthesis or guideline pooled it, 68 citations in OpenAlex.
- Undifferentiated autoinflammatory diseases: a systematic review.World journal of pediatrics : WJP · 2026Pooled it
- Adult patients with autoinflammation of unknown origin partially phenocopy the immune presentation of Still's disease.Nature communications · 2026Article
- Long-term anakinra therapy in Schnitzler syndrome: real-world evidence on remission of neutrophilic dermatitis and systemic inflammation, safety, biomarker insights and dose optimization.Frontiers in immunology · 2026Observational
- Advances in mechanistic investigation and treatment of steroid-refractory ICI-induced liver injury.Clinical and experimental medicine · 2025Review
- Undifferentiated autoinflammatory disease in adults: a prospective study in 61 patients.Orphanet journal of rare diseases · 2025Article
- Anti-Inflammatory Drugs for Alcoholic Liver Disease: A Systematic Review on Survival and Adverse Events.International journal of hepatology · 2025Review
- Case Report: Efficacy, safety, and favorable long-term outcome of early treatment with IL-1 inhibitors in a patient with chronic infantile neurological cutaneous articular (CINCA) syndrome caused by NLRP3 mosaicism.Frontiers in pediatrics · 2024Article
- Using Combination therapy to overcome diverse challenges of Immune Checkpoint Inhibitors treatment.International journal of biological sciences · 2024Review
- Safety and efficacy of canakinumab treatment for undifferentiated autoinflammatory diseases: the data of a retrospective cohort two-centered study.Frontiers in medicine · 2023Article
- Systematic genetic analysis of pediatric patients with autoinflammatory diseases.Frontiers in genetics · 2023Article
- Features predicting colchicine efficacy in treatment of children with undefined systemic autoinflammatory disease: A retrospective cohort study.European journal of rheumatology · 2022Article
- Improving Diagnosis and Clinical Management of Acquired Systemic Autoinflammatory Diseases.Journal of inflammation research · 2022Review
- Anakinra in Paediatric Rheumatology and Periodic Fever Clinics: Is the Higher Dose Safe?Frontiers in pediatrics · 2022Article
- Development and Implementation of the AIDA International Registry for Patients With Undifferentiated Systemic AutoInflammatory Diseases.Frontiers in medicine · 2022Article
- Biologic Therapy for Refractory Immune Checkpoint Inhibitor Colitis.Biologics : targets & therapy · 2022Review
- USAID Associated with Myeloid Neoplasm and VEXAS Syndrome: Two Differential Diagnoses of Suspected Adult Onset Still's Disease in Elderly Patients.Journal of clinical medicine · 2021Article
- Syndrome of Undifferentiated Recurrent Fever (SURF): An Emerging Group of Autoinflammatory Recurrent Fevers.Journal of clinical medicine · 2021Review
- Persistent inflammatory and non-inflammatory mechanisms in refractory rheumatoid arthritis.Nature reviews. Rheumatology · 2021Review
- Genetic panel screening in patients with clinically unclassified systemic autoinflammatory diseases.Clinical rheumatology · 2020Article
- Familial Mediterranean fever, from pathogenesis to treatment: a contemporary reviewTurkish journal of medical sciences · 2020Review
Corrections and comments
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Authors and funding
7 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundSome adult patients presenting with unexplained pyrexia, serositis, skin rashes, arthralgia, myalgia, and other symptoms commonly found in autoinflammatory disorders may not fit a specific diagnosis, either because their clinical phenotype is nondiagnostic or genetic tests are negative. We used the term undifferentiated systemic autoinflammatory disorder (uSAID) to describe such cases. Given that well-defined autoinflammatory diseases show responses to IL-1 blockade, we evaluated whether anakinra was useful for both diagnosing and treating uSAID patients.
methodsWe performed a retrospective analysis of consecutive patients presenting with uSAID between 2012-2015 who were treated with the recombinant IL-1 receptor antagonist anakinra. uSAID was diagnosed after excluding malignancy, infection, and pathogenic mutations in known hereditary fever syndromes (HFS) genes and where clinical criteria for adult onset Still's disease (AOSD) were not met.
resultsA total of 11 patients presented with uSAID (5 males and 6 females), with a mean time to diagnosis of 3.5 years (1-8 years). Patients were unresponsive or only partially controlled on disease-modifying antirheumatic drug (DMARD)/steroid treatment. Anakinra controlled symptoms within 4-6 weeks of starting treatment in 9 of 11 cases. Two patients discontinued therapy - one due to incomplete response and another due to severe injection-site reactions.
conclusionThis retrospective case series demonstrates that the spectrum of poorly defined autoinflammatory disorders that show responsiveness to anakinra is considerable. Anakinra seems a viable treatment option for these patients, who are unresponsive to standard steroid/DMARD treatments. Moreover, given the mechanisms of action, response to anakinra implicates underlying IL-1 dysregulation in the disease pathogenesis of responding uSAIDs patients.
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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.