Long‐term remission with ustekinumab in a patient with pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome suggests the importance of interleukin‐12/interleukin‐23 cytokines in PSTPIP1–associated inflammatory diseases
This article focuses on the clinical presentation and treatment response of a family affected by proline/serine/threonine phosphatase–interacting protein 1 (PSTPIP1)–associated inflammatory disease, specifically pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome. The mother, carrying...
| Published in: | Arthritis & Rheumatology Vol. 78; no. 4; pp. 1000 - 1005 |
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| Main Authors: | , , , , , , |
| Format: | case study letter pictorial tables/charts Journal Article |
| Published: |
Wiley-Blackwell
Apr2026
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| Online Access: | View this record in EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=192816842&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 192816842 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 23265191 H06R jtl: Arthritis & Rheumatology issn: 23265191 maglogo: N pubinfo: dt: Apr2026 vid: 78 iid: 4 pid: 480 pub: Wiley-Blackwell place: Malden, Massachusetts artinfo: ui: 192816842 191481145 192816842 192816842 10.1002/art.43399 192816842 ppf: 1000 ppct: 5 formats: tig: atl: Long‐term remission with ustekinumab in a patient with pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome suggests the importance of interleukin‐12/interleukin‐23 cytokines in PSTPIP1–associated inflammatory diseases aug: au: Wittmann, Nico Nell, Dominik Mohs, Magdalena Grude, Marie C. Mishra, Neha Meyer‐Bahlburg, Almut Bossaller, Lukas affil: Section of Pediatric Rheumatology and Immunology, Department of Pediatric and Adolescent Medicine, University Medicine Greifswald, Greifswald, Germany sug: subj: Disease Remission Antibodies, Monoclonal Therapeutic Use Arthritis, Infectious Familial and Genetic Pyoderma Gangrenosum Familial and Genetic Acne Vulgaris Familial and Genetic Interleukins Blood Cytokines Blood Autoimmune Diseases Familial and Genetic Arthritis, Infectious Drug Therapy Acne Vulgaris Drug Therapy Pyoderma Gangrenosum Drug Therapy Wound Healing Colitis, Ulcerative Cell Culture Techniques Mutation Inflammation Mediators ab: This article focuses on the clinical presentation and treatment response of a family affected by proline/serine/threonine phosphatase–interacting protein 1 (PSTPIP1)–associated inflammatory disease, specifically pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome. The mother, carrying a common PSTPIP1 mutation (A230T), exhibited refractory pyoderma gangrenosum and ulcerative colitis, achieving long-term remission with ustekinumab, an interleukin (IL)-12/23 inhibitor, after inadequate response to anakinra. Serum cytokine analysis revealed elevated IL-1β, IL-18, IL-33, interferon-α, IL-12, and IL-23 levels in patients compared to healthy donors, with IL-12/23 blockade correlating with clinical improvement despite persistent cytokine elevations. These findings suggest a potential therapeutic role for IL-12/23 inhibition in PAPA syndrome, particularly in cases complicated by ulcerative colitis or resistant to standard treatments. pubtype: Academic Journal doctype: case study letter pictorial tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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