ArticleAnnals of the rheumatic diseases2024
Interrupting an IFN-γ-dependent feedback loop in the syndrome of pyogenic arthritis with pyoderma gangrenosum and acne.
Article in Annals of the rheumatic diseases, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
What it found
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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.
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Who cites it
12 citing papers in PubMed, 14 citations in OpenAlex.
- Acne: epidemiology, risk factors, pathogenesis, management and cutting-edge research.Signal transduction and targeted therapy · 2026Review
- Biologic-biologic and biologic-JAK inhibitor combination therapy in refractory systemic autoinflammatory diseases.Seminars in arthritis and rheumatism · 2026Observational
- Decoding variants of uncertain significance in systemic autoinflammatory diseases.Nature reviews. Rheumatology · 2026Review
- The use of JAK inhibitors and tocilizumab in the management of TRAPS: a sibling case study.Pediatric rheumatology online journal · 2026Article
- Autoinflammatory disease and severe neutropenia due toJournal of human immunity · 2026Article
- A novel pathogenic variant in PSTPIP1 highlights the diversity of PSTPIP1-associated disorders.Rheumatology (Oxford, England) · 2026Article
- A protracted diagnostic journey of pediatric PAPA syndrome and subsequent response to tofacitinib therapy: a case report and literature review.Frontiers in immunology · 2026Review
- NLRP3-inflammasome Related Genes as Emerging Biomarkers and Therapeutic Targets in Psoriasis.Inflammation · 2025Article
- Targeting pyroptosis in myocardial inflammation and fibrosis: molecular mechanisms and therapeutic strategies.Apoptosis : an international journal on programmed cell death · 2025Review
- Allogeneic hematopoietic cell transplantation for autoinflammatory disorders.International journal of hematology · 2025Review
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Corrections and comments
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Authors and funding
17 authors at 5 institutions in 2 countries.
Funding
Abstract
objectivesTo study the molecular pathogenesis of PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) syndrome, a debilitating hereditary autoinflammatory disease caused by dominant mutation in
methodsGene knock-out and knock-in mice were generated to develop an animal model. THP1 and retrovirally transduced U937 human myeloid leukaemia cell lines, peripheral blood mononuclear cells, small interfering RNA (siRNA) knock-down, site-directed mutagenesis, cytokine immunoassays, coimmunoprecipitation and immunoblotting were used to study inflammasome activation. Cytokine levels in the skin were evaluated by immunohistochemistry. Responsiveness to Janus kinase (JAK) inhibitors was evaluated ex vivo with peripheral blood mononuclear cells and in vivo in five treatment-refractory PAPA patients.
resultsThe knock-in mouse model of PAPA did not recapitulate the human disease. In a human myeloid cell line model, PAPA-associated
conclusionPAPA-associated
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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.