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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.
See: This record
Not genetically inherited (Orphanet)
 
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

Treatment of systemic juvenile idiopathic arthritis.
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
Adult-Onset Still's Disease-A Complex Disease, a Challenging Treatment.
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
Tocilizumab for the treatment of adult-onset Still's disease.
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

Etiology

Systematic Review and Metaanalysis of Pharmacological Interventions in Adult-Onset Still Disease and the Role of Biologic Disease-Modifying Antirheumatic Drugs.
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
Autoimmune and Autoinflammatory Connective Tissue Disorders Following COVID-19.
Lim SH, Ju HJ, Han JH, Lee JH, Lee WS, Bae JM, Lee S
JAMA Netw Open 2023 Oct 2;6(10):e2336120. doi: 10.1001/jamanetworkopen.2023.36120. PMID: 37801317Free PMC Article
Bibliometrics and Visual Analysis of Adult-onset Still Disease (1976-2020).
Xu B, Wang J, Meng X, Bao B
Front Public Health 2022;10:884780. Epub 2022 Jun 15 doi: 10.3389/fpubh.2022.884780. PMID: 35784223Free PMC Article
Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy.
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
Myocarditis in adult-onset still disease.
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

Diagnosis

Autoimmune and Autoinflammatory Connective Tissue Disorders Following COVID-19.
Lim SH, Ju HJ, Han JH, Lee JH, Lee WS, Bae JM, Lee S
JAMA Netw Open 2023 Oct 2;6(10):e2336120. doi: 10.1001/jamanetworkopen.2023.36120. PMID: 37801317Free PMC Article
Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis.
González García A, Fernández-Martín J, Robles Marhuenda Á
Rheumatology (Oxford) 2023 Apr 3;62(4):1426-1435. doi: 10.1093/rheumatology/keac481. PMID: 35997567Free PMC Article
Adult-Onset Still's Disease-A Complex Disease, a Challenging Treatment.
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
Adult-onset Still disease.
Fautrel B
Best Pract Res Clin Rheumatol 2008 Oct;22(5):773-92. doi: 10.1016/j.berh.2008.08.006. PMID: 19028363
Rheumatoid arthritis: a review of the cutaneous manifestations.
Sayah A, English JC 3rd
J Am Acad Dermatol 2005 Aug;53(2):191-209; quiz 210-2. doi: 10.1016/j.jaad.2004.07.023. PMID: 16021111

Therapy

Treatment of systemic juvenile idiopathic arthritis.
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
Adult-Onset Still's Disease-A Complex Disease, a Challenging Treatment.
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
Tocilizumab for the treatment of adult-onset Still's disease.
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
Adult-onset Still disease.
Fautrel B
Best Pract Res Clin Rheumatol 2008 Oct;22(5):773-92. doi: 10.1016/j.berh.2008.08.006. PMID: 19028363
Polyarteritis nodosa.
Stone JH
JAMA 2002 Oct 2;288(13):1632-9. doi: 10.1001/jama.288.13.1632. PMID: 12350194

Prognosis

Adult-Onset Still's Disease-A Complex Disease, a Challenging Treatment.
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
Tocilizumab for the treatment of adult-onset Still's disease.
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
Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy.
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
Myocarditis in adult-onset still disease.
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
Adult-onset Still disease.
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

Criteria for Cytokine Storm Syndromes.
Minoia F, Ravelli A
Adv Exp Med Biol 2024;1448:59-71. doi: 10.1007/978-3-031-59815-9_6. PMID: 39117808
Nationwide Analysis of Adult-Onset Still Disease With and Without Hemophagocytic Lymphohistiocytosis.
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
Current treatment options and safety considerations when treating adult-onset Still's disease.
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
Interleukin 1 inhibition with anakinra in adult-onset Still disease: a meta-analysis of its efficacy and safety.
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
Adult-onset Still disease.
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

Systematic Review and Metaanalysis of Pharmacological Interventions in Adult-Onset Still Disease and the Role of Biologic Disease-Modifying Antirheumatic Drugs.
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
The JANUS of chronic inflammatory and autoimmune diseases onset during COVID-19 - A systematic review of the literature.
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
A systematic review of the off-label use of biological therapies in systemic autoimmune diseases.
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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