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Adult-onset Still disease

MedGen UID:
39007
Concept ID:
C0085253
Disease or Syndrome
Synonyms: Adult Still's disease; Still's disease adult onset
SNOMED CT: Adult onset Still disease (239920006); Adult onset Still's disease (239920006); Wissler-Fanconi syndrome (239920006)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019355
Orphanet: ORPHA829

Definition

A rare inflammatory multisystem disorder characterized clinically by four cardinal signs: fever of unknown origin, arthralgia or arthritis, hyperleucocytosis, and typical skin rash. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdult-onset Still disease

Professional guidelines

PubMed

Hinze CH, Foell D, Kessel C
Nat Rev Rheumatol 2023 Dec;19(12):778-789. Epub 2023 Nov 3 doi: 10.1038/s41584-023-01042-z. PMID: 37923864
Macovei LA, Burlui A, Bratoiu I, Rezus C, Cardoneanu A, Richter P, Szalontay A, Rezus E
Int J Mol Sci 2022 Oct 24;23(21) doi: 10.3390/ijms232112810. PMID: 36361602Free PMC Article
Castañeda S, Martínez-Quintanilla D, Martín-Varillas JL, García-Castañeda N, Atienza-Mateo B, González-Gay MA
Expert Opin Biol Ther 2019 Apr;19(4):273-286. doi: 10.1080/14712598.2019.1590334. PMID: 30827128

Recent clinical studies

Therapy

Hinze CH, Foell D, Kessel C
Nat Rev Rheumatol 2023 Dec;19(12):778-789. Epub 2023 Nov 3 doi: 10.1038/s41584-023-01042-z. PMID: 37923864
Macovei LA, Burlui A, Bratoiu I, Rezus C, Cardoneanu A, Richter P, Szalontay A, Rezus E
Int J Mol Sci 2022 Oct 24;23(21) doi: 10.3390/ijms232112810. PMID: 36361602Free PMC Article
Castañeda S, Martínez-Quintanilla D, Martín-Varillas JL, García-Castañeda N, Atienza-Mateo B, González-Gay MA
Expert Opin Biol Ther 2019 Apr;19(4):273-286. doi: 10.1080/14712598.2019.1590334. PMID: 30827128
Fautrel B
Best Pract Res Clin Rheumatol 2008 Oct;22(5):773-92. doi: 10.1016/j.berh.2008.08.006. PMID: 19028363
Stone JH
JAMA 2002 Oct 2;288(13):1632-9. doi: 10.1001/jama.288.13.1632. PMID: 12350194

Prognosis

Macovei LA, Burlui A, Bratoiu I, Rezus C, Cardoneanu A, Richter P, Szalontay A, Rezus E
Int J Mol Sci 2022 Oct 24;23(21) doi: 10.3390/ijms232112810. PMID: 36361602Free PMC Article
Castañeda S, Martínez-Quintanilla D, Martín-Varillas JL, García-Castañeda N, Atienza-Mateo B, González-Gay MA
Expert Opin Biol Ther 2019 Apr;19(4):273-286. doi: 10.1080/14712598.2019.1590334. PMID: 30827128
Sun NZ, Brezinski EA, Berliner J, Haemel A, Connolly MK, Gensler L, McCalmont TH, Shinkai K
J Am Acad Dermatol 2015 Aug;73(2):294-303. Epub 2015 Jun 6 doi: 10.1016/j.jaad.2015.04.063. PMID: 26054431
Gerfaud-Valentin M, Sève P, Iwaz J, Gagnard A, Broussolle C, Durieu I, Ninet J, Hot A
Medicine (Baltimore) 2014 Oct;93(17):280-289. doi: 10.1097/MD.0000000000000112. PMID: 25398063Free PMC Article
Fautrel B
Best Pract Res Clin Rheumatol 2008 Oct;22(5):773-92. doi: 10.1016/j.berh.2008.08.006. PMID: 19028363

Clinical prediction guides

Minoia F, Ravelli A
Adv Exp Med Biol 2024;1448:59-71. doi: 10.1007/978-3-031-59815-9_6. PMID: 39117808
Sami F, Manansala M, Arora S, Manadan AM
J Clin Rheumatol 2024 Aug 1;30(5):e125-e128. Epub 2024 Jun 4 doi: 10.1097/RHU.0000000000002100. PMID: 38831495
Cavalli G, Farina N, Campochiaro C, Baldissera E, Dagna L
Expert Opin Drug Saf 2020 Dec;19(12):1549-1558. Epub 2020 Oct 27 doi: 10.1080/14740338.2020.1839411. PMID: 33078630
Hong D, Yang Z, Han S, Liang X, Ma K, Zhang X
Drug Des Devel Ther 2014;8:2345-57. Epub 2014 Nov 25 doi: 10.2147/DDDT.S73428. PMID: 25473268Free PMC Article
Fautrel B
Best Pract Res Clin Rheumatol 2008 Oct;22(5):773-92. doi: 10.1016/j.berh.2008.08.006. PMID: 19028363

Recent systematic reviews

Ruscitti P, McGonagle D, Garcia VC, Rabijns H, Toennessen K, Chappell M, Edwards M, Miller P, Hansell N, Moss J, Graziadio S, Feist E
J Rheumatol 2024 May 1;51(5):442-451. doi: 10.3899/jrheum.2023-0995. PMID: 38302170
Novelli L, Motta F, De Santis M, Ansari AA, Gershwin ME, Selmi C
J Autoimmun 2021 Feb;117:102592. Epub 2020 Dec 14 doi: 10.1016/j.jaut.2020.102592. PMID: 33401171Free PMC Article
Ramos-Casals M, Brito-Zerón P, Muñoz S, Soto MJ; BIOGEAS STUDY Group
Medicine (Baltimore) 2008 Nov;87(6):345-364. doi: 10.1097/MD.0b013e318190f170. PMID: 19011506

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