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Juvenile rheumatoid arthritis

MedGen UID:
811462
Concept ID:
C3714757
Disease or Syndrome
Synonym: Rheumatoid arthritis, juvenile
 
HPO: HP:0005681

Definition

An older, deprecated term that encompassed three major types of autoimmune or autoinflammatory arthritis in children: systemic-onset, pauciarticular, or polyarticular arthritis. The juvenile rheumatoid arthritis classification system has been replaced by the International League of Associations for Rheumatology (ILAR) juvenile idiopathic arthritis classification system. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Juvenile rheumatoid arthritis

Conditions with this feature

Ramon syndrome
MedGen UID:
208669
Concept ID:
C0796133
Disease or Syndrome
A rare, genetic, primary bone dysplasia syndrome characterized by bilateral, painless swelling of the face extending from the mandible to the inferior orbital margins (cherubism), epilepsy, gingival fibromatosis (possibly obscuring teeth), and intellectual disability. Other associated variable features include hypertrichosis, stunted growth, juvenile rheumatoid arthritis, and development of ocular abnormalities (e.g. pigmentary retinopathy, optic disc pallor, Axenfeld anomaly). Radiological images typically show bilateral multifocal radiolucency involving the body, angle and ramus of the mandible and coronoid process.
Spondyloenchondrodysplasia with immune dysregulation
MedGen UID:
375009
Concept ID:
C1842763
Disease or Syndrome
Spondyloenchondrodysplasia with immune dysregulation (SPENCDI) is an immunoosseous dysplasia combining the typical metaphyseal and vertebral bone lesions of spondyloenchondrodysplasia (SPENCD) with immune dysfunction and neurologic involvement. The skeletal dysplasia is characterized by radiolucent and irregular spondylar and metaphyseal lesions that represent islands of chondroid tissue within bone. The vertebral bodies show dorsally accentuated platyspondyly with disturbance of ossification. Clinical abnormalities such as short stature, rhizomelic micromelia, increased lumbar lordosis, barrel chest, facial anomalies, and clumsy movements may be present (Menger et al., 1989). Central nervous system involvement includes spasticity, mental retardation, and cerebral calcifications, and immune dysregulation ranges from autoimmunity to immunodeficiency. Neurologic and autoimmune manifestations have been observed in different combinations within a single family, suggesting that this disorder may be defined by specific radiographic features but has remarkably pleiotropic manifestations (Renella et al., 2006). Briggs et al. (2016) also noted variability in skeletal, neurologic, and immune phenotypes, which was sometimes marked between members of the same family. Classification of the Enchondromatoses In their classification of the enchondromatoses, Spranger et al. (1978) called Ollier disease and Maffucci syndrome types I and II enchondromatosis, respectively; metachondromatosis (156250), type III; and spondyloenchondrodysplasia (SPENCD), also called spondyloenchondromatosis, type IV; enchondromatosis with irregular vertebral lesions, type V; and generalized enchondromatosis, type VI. Halal and Azouz (1991) added 3 tentative categories to the 6 in the classification of Spranger et al. (1978). Pansuriya et al. (2010) suggested a new classification of enchondromatosis (multiple enchondromas).
Chromosome 15q11.2 deletion syndrome
MedGen UID:
467404
Concept ID:
C3180937
Disease or Syndrome
A heterozygous deletion of chromosome 15q11.2 may increase the susceptibility to neuropsychiatric or neurodevelopmental problems, including delayed psychomotor development, speech delay, autism spectrum disorder, attention deficit-hyperactivity disorder, obsessive-compulsive disorder, and possibly seizures (summary by Doornbos et al., 2009 and Burnside et al., 2011). See also chromosome 15q11.2 duplication syndrome (608636).

Professional guidelines

PubMed

Kastrati K, Aletaha D, Burmester GR, Chwala E, Dejaco C, Dougados M, McInnes IB, Ravelli A, Sattar N, Stamm TA, Takeuchi T, Trauner M, van der Heijde D, Voshaar MJH, Winthrop K, Smolen JS, Kerschbaumer A
RMD Open 2022 Sep;8(2) doi: 10.1136/rmdopen-2022-002359. PMID: 36260501Free PMC Article
Abramowicz S, Kim S, Prahalad S, Chouinard AF, Kaban LB
Int J Oral Maxillofac Surg 2016 Jul;45(7):801-12. Epub 2016 May 6 doi: 10.1016/j.ijom.2016.03.013. PMID: 27160609
Abdel MP, Figgie MP
Orthop Clin North Am 2014 Oct;45(4):435-42. Epub 2014 Jul 24 doi: 10.1016/j.ocl.2014.06.002. PMID: 25199416

Recent clinical studies

Etiology

Naz S, Mushtaq A, Rehman S, Bari A, Maqsud A, Khan MZ, Ahmad TM
J Coll Physicians Surg Pak 2013 Jun;23(6):409-12. PMID: 23763801
Gallagher KT, Bernstein B
Curr Opin Rheumatol 1999 Sep;11(5):372-6. doi: 10.1097/00002281-199909000-00008. PMID: 10503657
Lindsley CB
Curr Opin Rheumatol 1995 Sep;7(5):425-9. doi: 10.1097/00002281-199509000-00011. PMID: 8519616
Ansell BM
Curr Opin Rheumatol 1992 Oct;4(5):706-12. PMID: 1419506
Schaller J
Postgrad Med 1977 Jan;61(1):177-84. doi: 10.1080/00325481.1977.11714517. PMID: 299941

Diagnosis

Prock-Gibbs H, Marshall BJ, Werntz JR
Pediatr Rev 2022 Apr 1;43(4):191-197. doi: 10.1542/pir.2020-000331. PMID: 35362033
Bovid KM, Moore MD
Orthop Clin North Am 2019 Oct;50(4):471-488. Epub 2019 Aug 6 doi: 10.1016/j.ocl.2019.06.003. PMID: 31466663
Naz S, Mushtaq A, Rehman S, Bari A, Maqsud A, Khan MZ, Ahmad TM
J Coll Physicians Surg Pak 2013 Jun;23(6):409-12. PMID: 23763801
Gallagher KT, Bernstein B
Curr Opin Rheumatol 1999 Sep;11(5):372-6. doi: 10.1097/00002281-199909000-00008. PMID: 10503657
Pertusi RM, Rubin BR, Blackwell D
J Am Osteopath Assoc 1996 May;96(5):298-302. doi: 10.7556/jaoa.1996.96.5.298. PMID: 8936447

Therapy

Naz S, Mushtaq A, Rehman S, Bari A, Maqsud A, Khan MZ, Ahmad TM
J Coll Physicians Surg Pak 2013 Jun;23(6):409-12. PMID: 23763801
Papp KA
Expert Opin Pharmacother 2004 Oct;5(10):2139-46. doi: 10.1517/14656566.5.10.2139. PMID: 15461549
Gallagher KT, Bernstein B
Curr Opin Rheumatol 1999 Sep;11(5):372-6. doi: 10.1097/00002281-199909000-00008. PMID: 10503657
Pertusi RM, Rubin BR, Blackwell D
J Am Osteopath Assoc 1996 May;96(5):298-302. doi: 10.7556/jaoa.1996.96.5.298. PMID: 8936447
Calabro JJ, Marchesano JM
N Engl J Med 1967 Oct 5;277(14):746-9 concl. doi: 10.1056/NEJM196710052771406. PMID: 5342857

Prognosis

Schneider R, Passo MH
Rheum Dis Clin North Am 2002 Aug;28(3):503-30. doi: 10.1016/s0889-857x(02)00016-9. PMID: 12380368
Ansell BM
Adv Exp Med Biol 1999;455:27-33. doi: 10.1007/978-1-4615-4857-7_5. PMID: 10599320
Graham TB, Giannini EH
Indian J Pediatr 1996 May-Jun;63(3):283-91. doi: 10.1007/BF02751520. PMID: 10830003
Pertusi RM, Rubin BR, Blackwell D
J Am Osteopath Assoc 1996 May;96(5):298-302. doi: 10.7556/jaoa.1996.96.5.298. PMID: 8936447
Tucker LB
Curr Opin Rheumatol 1993 Sep;5(5):619-28. doi: 10.1097/00002281-199305050-00010. PMID: 7691139

Clinical prediction guides

An Q, Jin MW, An XJ, Xu SM, Wang L
Eur Rev Med Pharmacol Sci 2017 Oct;21(19):4322-4326. PMID: 29077164
Schneider R, Passo MH
Rheum Dis Clin North Am 2002 Aug;28(3):503-30. doi: 10.1016/s0889-857x(02)00016-9. PMID: 12380368
Ansell BM
Adv Exp Med Biol 1999;455:27-33. doi: 10.1007/978-1-4615-4857-7_5. PMID: 10599320
Lindsley CB
Curr Opin Rheumatol 1995 Sep;7(5):425-9. doi: 10.1097/00002281-199509000-00011. PMID: 8519616
Tucker LB
Curr Opin Rheumatol 1993 Sep;5(5):619-28. doi: 10.1097/00002281-199305050-00010. PMID: 7691139

Recent systematic reviews

Jandhyala R
BMC Med Res Methodol 2023 May 20;23(1):121. doi: 10.1186/s12874-023-01947-z. PMID: 37210484Free PMC Article
Barik S, Jain A, Chanakya PV, Raj V, Goyal T
Eur J Orthop Surg Traumatol 2023 Oct;33(7):2737-2748. Epub 2023 Mar 22 doi: 10.1007/s00590-023-03525-x. PMID: 36947313
Konopitski A, Okafor C, Smith B, Baldwin K, Sheth NP
Arch Orthop Trauma Surg 2023 Feb;143(2):1081-1094. Epub 2022 Feb 7 doi: 10.1007/s00402-022-04357-w. PMID: 35129653
Woolnough L, Pomputius A, Vincent HK
Gait Posture 2021 Mar;85:38-54. Epub 2020 Dec 31 doi: 10.1016/j.gaitpost.2020.12.010. PMID: 33513531
Xiao Y, Liu H, Chen L, Wang Y, Yao X, Jiang X
Biosci Rep 2019 Jul 31;39(7) Epub 2019 Jul 18 doi: 10.1042/BSR20190298. PMID: 31235484Free PMC Article

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