ArticleOrphanet journal of rare diseases2025
Undifferentiated autoinflammatory disease in adults: a prospective study in 61 patients.
Article in Orphanet journal of rare diseases, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers, 1 of them a synthesis that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
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.
The trial behind it
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
4 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Undifferentiated autoinflammatory diseases: a systematic review.World journal of pediatrics : WJP · 2026Pooled it
- Before calling it FMF: MEFV variants of uncertain significance in autoinflammatory disease.Rheumatology (Oxford, England) · 2026Article
- Recurrent Sterile Pleuropericarditis After Withdrawal of Renal Transplant Immunosuppression in Anuric ESRD: A Case of Suspected IL-1-Mediated Autoinflammation.Case reports in rheumatology · 2026Article
- Syndrome of undifferentiated recurrent fever (SURF): a multicenter real-world experience from Türkiye.Clinical rheumatology · 2025Observational
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
3 authors.
Funding
Abstract
backgroundsUndifferentiated or undefined systemic autoinflammatory disease (uSAID) encompasses a group of rare, heterogeneous diseases characterized by the features of well-defined systemic autoinflammatory diseases (SAIDs), but lacking diagnostic phenotypes or genetic confirmation. We aimed to describe the phenotypes, genotypes and treatment responses of Chinese adult patients with uSAID.
methodsThe final diagnosis of uSAID was reached in 61 patients, whose organ-specific inflammation was compared and three subgroups were identified based on phenotypic similarities to well-defined SAIDs. Phenotypes, genotypes and treatment responses were analyzed in these subgroups.
resultsAmong the 61 uSAID patients, 17 had disease-onset during childhood, and 44 had adult-onset. Compared to those without pulmonary manifestations, patients with pulmonary involvement exhibited higher frequencies of myalgia, skin lesions, cardiac involvement, gastrointestinal involvement, urinary involvement, lymphadenopathy, headache, and intellectual impairments. Twenty-four patients exhibited monogenic SAID-like phenotypes, 12 had polygenic SAID-like phenotypes, and the remaining 25 were categorized as having atypical phenotypes. Among the 53 patients followed, 25% (13/53) improved spontaneously with complete or partial recovery independent of therapy. Patients with atypical phenotypes had the highest spontaneous remission rate (10/23, 43%).
conclusionThis study is the first to describe the clinical and genetic features of a cohort of Chinese adult patients with uSAID. Patients with pulmonary manifestations may be more prone to developing complex phenotypes, while those with atypical phenotypes have a high rate of spontaneous remission, indicating a favorable prognosis.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
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.