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Rheumatoid arthritis, systemic juvenile

MedGen UID:
346934
Concept ID:
C1858558
Disease or Syndrome
Synonyms: Rheumatoid Arthritis, Systemic Juvenile; Systemic Juvenile Rheumatoid Arthritis
Modes of inheritance:
Multifactorial inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Sources: HPO, Orphanet
A mode of inheritance that depends on a mixture of major and minor genetic determinants possibly together with environmental factors. Diseases inherited in this manner are termed complex diseases.
unknown inheritance
MedGen UID:
831588
Concept ID:
CN227389
Intellectual Product
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
 
Genes (locations): IL6 (7p15.3); MIF (22q11.23)
OMIM®: 604302
Orphanet: ORPHA85414

Definition

Juvenile idiopathic arthritis refers to a group of conditions involving joint inflammation (arthritis) that first appears before the age of 16. This condition is an autoimmune disorder, which means that the immune system malfunctions and attacks the body's organs and tissues, in this case the joints.Researchers have described seven types of juvenile idiopathic arthritis. The types are distinguished by their signs and symptoms, the number of joints affected, the results of laboratory tests, and the family history.Systemic juvenile idiopathic arthritis causes inflammation in one or more joints. A high daily fever that lasts at least 2 weeks either precedes or accompanies the arthritis. Individuals with systemic arthritis may also have a skin rash or enlargement of the lymph nodes (lymphadenopathy), liver (hepatomegaly), or spleen (splenomegaly).Oligoarticular juvenile idiopathic arthritis (also known as oligoarthritis) is marked by the occurrence of arthritis in four or fewer joints in the first 6 months of the disease. It is divided into two subtypes depending on the course of disease. If the arthritis is confined to four or fewer joints after 6 months, then the condition is classified as persistent oligoarthritis. If more than four joints are affected after 6 months, this condition is classified as extended oligoarthritis. Individuals with oligoarthritis are at increased risk of developing inflammation of the eye (uveitis).Rheumatoid factor positive polyarticular juvenile idiopathic arthritis (also known as polyarthritis, rheumatoid factor positive) causes inflammation in five or more joints within the first 6 months of the disease. Individuals with this condition also have a positive blood test for proteins called rheumatoid factors. This type of arthritis closely resembles rheumatoid arthritis as seen in adults.Rheumatoid factor negative polyarticular juvenile idiopathic arthritis (also known as polyarthritis, rheumatoid factor negative) is also characterized by arthritis in five or more joints within the first 6 months of the disease. Individuals with this type, however, test negative for rheumatoid factor in the blood.Psoriatic juvenile idiopathic arthritis involves arthritis that usually occurs in combination with a skin disorder called psoriasis. Psoriasis is a condition characterized by patches of red, irritated skin that are often covered by flaky white scales. Some affected individuals develop psoriasis before arthritis while others first develop arthritis. Other features of psoriatic arthritis include abnormalities of the fingers and nails or eye problems.Enthesitis-related juvenile idiopathic arthritis is characterized by tenderness where the bone meets a tendon, ligament, or other connective tissue. The most commonly affected places are the hips, knees, and feet. This tenderness, known as enthesitis, accompanies the joint inflammation of arthritis. Enthesitis-related arthritis may also involve inflammation in parts of the body other than the joints.The last type of juvenile idiopathic arthritis is called undifferentiated arthritis. This classification is given to affected individuals who do not fit into any of the above types or who fulfill the criteria for more than one type of juvenile idiopathic arthritis.
[from GHR]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRheumatoid arthritis, systemic juvenile
Follow this link to review classifications for Rheumatoid arthritis, systemic juvenile in Orphanet.

Recent clinical studies

Etiology

Cader MZ, Boroviak K, Zhang Q, Assadi G, Kempster SL, Sewell GW, Saveljeva S, Ashcroft JW, Clare S, Mukhopadhyay S, Brown KP, Tschurtschenthaler M, Raine T, Doe B, Chilvers ER, Griffin JL, Kaneider NC, Floto RA, D'Amato M, Bradley A, Wakelam MJ, Dougan G, Kaser A
Nat Immunol 2016 Sep;17(9):1046-56. Epub 2016 Aug 1 doi: 10.1038/ni.3532. PMID: 27478939Free PMC Article
Woerner A, von Scheven-Gête A, Cimaz R, Hofer M
Expert Rev Clin Immunol 2015 May;11(5):575-88. Epub 2015 Apr 4 doi: 10.1586/1744666X.2015.1032257. PMID: 25843554
Schulert GS, Grom AA
Best Pract Res Clin Rheumatol 2014 Apr;28(2):277-92. doi: 10.1016/j.berh.2014.03.002. PMID: 24974063Free PMC Article
Calkoen FG, Brinkman DM, Vervat C, van Ostaijen-Ten Dam MM, Ten Cate R, van Tol MJ, Ball LM
Cytotherapy 2013 Mar;15(3):280-91. Epub 2013 Jan 9 doi: 10.1016/j.jcyt.2012.10.017. PMID: 23312448
Ruperto N, Brunner HI, Quartier P, Constantin T, Wulffraat N, Horneff G, Brik R, McCann L, Kasapcopur O, Rutkowska-Sak L, Schneider R, Berkun Y, Calvo I, Erguven M, Goffin L, Hofer M, Kallinich T, Oliveira SK, Uziel Y, Viola S, Nistala K, Wouters C, Cimaz R, Ferrandiz MA, Flato B, Gamir ML, Kone-Paut I, Grom A, Magnusson B, Ozen S, Sztajnbok F, Lheritier K, Abrams K, Kim D, Martini A, Lovell DJ; PRINTO.; PRCSG.
N Engl J Med 2012 Dec 20;367(25):2396-406. doi: 10.1056/NEJMoa1205099. PMID: 23252526

Diagnosis

Shimizu M, Inoue N, Mizuta M, Nakagishi Y, Yachie A
Clin Exp Immunol 2018 Mar;191(3):349-355. Epub 2017 Sep 15 doi: 10.1111/cei.13026. PMID: 28815559Free PMC Article
Darby JB, Liddell L, DeGuzman M, McClain KL, Rubenstein J, Chase L, Marquez L
Pediatrics 2015 May;135(5):902-8. doi: 10.1542/peds.2014-3692. PMID: 25917994
Wilson P, Genies M, Wu E, Vincent J
Clin Pediatr (Phila) 2014 Oct;53(11):1109-11. Epub 2014 May 7 doi: 10.1177/0009922814534315. PMID: 24807978
Correll CK, Binstadt BA
Pediatr Res 2014 Jan;75(1-2):176-83. Epub 2013 Nov 8 doi: 10.1038/pr.2013.187. PMID: 24213625
Saha A, Chopra Y, Theis JD, Vrana JA, Sethi S
Am J Kidney Dis 2013 Oct;62(4):834-8. Epub 2013 May 10 doi: 10.1053/j.ajkd.2013.02.377. PMID: 23664546

Therapy

Cansever M, Şahin N, Dursun I, Geyik C, Düşünsel R, Bektaş Kut F, Tahan F
J Investig Allergol Clin Immunol 2018 Dec;28(6):436-438. doi: 10.18176/jiaci.0314. PMID: 30530397
Beresford MW, Ramanan AV
Nat Rev Rheumatol 2018 Feb;14(2):69-70. Epub 2018 Jan 11 doi: 10.1038/nrrheum.2017.214. PMID: 29323342
Chighizola CB, Ong VH, Meroni PL
Clin Rev Allergy Immunol 2017 Jun;52(3):401-423. doi: 10.1007/s12016-016-8582-3. PMID: 27515671
Woerner A, von Scheven-Gête A, Cimaz R, Hofer M
Expert Rev Clin Immunol 2015 May;11(5):575-88. Epub 2015 Apr 4 doi: 10.1586/1744666X.2015.1032257. PMID: 25843554
Wilson P, Genies M, Wu E, Vincent J
Clin Pediatr (Phila) 2014 Oct;53(11):1109-11. Epub 2014 May 7 doi: 10.1177/0009922814534315. PMID: 24807978

Prognosis

Chighizola CB, Ong VH, Meroni PL
Clin Rev Allergy Immunol 2017 Jun;52(3):401-423. doi: 10.1007/s12016-016-8582-3. PMID: 27515671
Sathe K, Khubchandani RP
Indian Pediatr 2013 Feb;50(2):237-9. PMID: 23474929
Hay AD, Ilowite NT
Pediatr Ann 2012 Nov;41(11) doi: 10.3928/00904481-20121022-10. PMID: 23814932

Clinical prediction guides

Shimizu M, Ueno K, Yachie A
Clin Vaccine Immunol 2012 Oct;19(10):1700-2. Epub 2012 Aug 8 doi: 10.1128/CVI.00309-12. PMID: 22875602Free PMC Article

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