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Undifferentiated connective tissue syndrome

MedGen UID:
592754
•Concept ID:
C0409999
•
Disease or Syndrome
Synonyms: Undifferentiated Connective Tissue Disease; Undifferentiated Connective Tissue Diseases
SNOMED CT: Undifferentiated connective tissue disease (239918008)
 
Monarch Initiative: MONDO:0019527
Orphanet: ORPHA90002

Definition

A rare systemic autoimmune disease characterized by the presence of signs and symptoms suggestive of a systemic autoimmune disease that do not fulfil the existing classification criteria. The main clinical manifestations are arthritis with arthralgia, Raynaud's phenomenon, xerostomia, xerophthalmia, and leukopenia, while neurologic or renal involvement are virtually absent. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUndifferentiated connective tissue syndrome
Follow this link to review classifications for Undifferentiated connective tissue syndrome in Orphanet.

Professional guidelines

PubMed

A systematic review of the incidence, management and prognosis of new-onset autoimmune connective tissue diseases after COVID-19.
Kouranloo K, Dey M, Elwell H, Nune A
Rheumatol Int 2023 Jul;43(7):1221-1243. Epub 2023 Feb 14 doi: 10.1007/s00296-023-05283-9. PMID: 36786873Free PMC Article
European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes.
Knobler R, Moinzadeh P, Hunzelmann N, Kreuter A, Cozzio A, Mouthon L, Cutolo M, Rongioletti F, Denton CP, Rudnicka L, Frasin LA, Smith V, Gabrielli A, Aberer E, Bagot M, Bali G, Bouaziz J, Braae Olesen A, Foeldvari I, Frances C, Jalili A, Just U, Kähäri V, Kárpáti S, Kofoed K, Krasowska D, Olszewska M, Orteu C, Panelius J, Parodi A, Petit A, Quaglino P, Ranki A, Sanchez Schmidt JM, Seneschal J, Skrok A, Sticherling M, Sunderkötter C, Taieb A, Tanew A, Wolf P, Worm M, Wutte NJ, Krieg T
J Eur Acad Dermatol Venereol 2017 Sep;31(9):1401-1424. Epub 2017 Aug 9 doi: 10.1111/jdv.14458. PMID: 28792092
Undifferentiated connective tissue diseases (UCTD): a review of the literature and a proposal for preliminary classification criteria.
Mosca M, Neri R, Bombardieri S
Clin Exp Rheumatol 1999 Sep-Oct;17(5):615-20. PMID: 10544849

Recent clinical studies

Etiology

Frequency of factors associated with habitual abortion in 197 couples.
Stephenson MD
Fertil Steril 1996 Jul;66(1):24-9. PMID: 8752606
Clinical and prognostic significance of vasculitis as an early manifestation of connective tissue disease syndromes.
Lakhanpal S, Conn DL, Lie JT
Ann Intern Med 1984 Dec;101(6):743-8. doi: 10.7326/0003-4819-101-6-743. PMID: 6149715
A prospective study of Raynaud phenomenon and early connective tissue disease. A five-year report.
Harper FE, Maricq HR, Turner RE, Lidman RW, Leroy EC
Am J Med 1982 Jun;72(6):883-8. doi: 10.1016/0002-9343(82)90846-4. PMID: 7091158

Diagnosis

Frequency of factors associated with habitual abortion in 197 couples.
Stephenson MD
Fertil Steril 1996 Jul;66(1):24-9. PMID: 8752606
Image analysis of nailfold capillaries in patients with undifferentiated connective tissue syndromes.
Ohtsuka T, Yamakage A, Tamura T
J Dermatol 1995 Dec;22(12):921-5. doi: 10.1111/j.1346-8138.1995.tb03946.x. PMID: 8647998
Raynaud's phenomenon with digital necrosis as the first manifestation of undifferentiated connective tissue syndrome.
Sabán J, Rodríguez-García JL, País JR, Mellado N, Muñoz E
Dermatologica 1991;182(2):121-3. doi: 10.1159/000247759. PMID: 2050232
A prospective study of Raynaud phenomenon and early connective tissue disease. A five-year report.
Harper FE, Maricq HR, Turner RE, Lidman RW, Leroy EC
Am J Med 1982 Jun;72(6):883-8. doi: 10.1016/0002-9343(82)90846-4. PMID: 7091158

Prognosis

Raynaud's phenomenon with digital necrosis as the first manifestation of undifferentiated connective tissue syndrome.
Sabán J, Rodríguez-García JL, País JR, Mellado N, Muñoz E
Dermatologica 1991;182(2):121-3. doi: 10.1159/000247759. PMID: 2050232
Clinical and prognostic significance of vasculitis as an early manifestation of connective tissue disease syndromes.
Lakhanpal S, Conn DL, Lie JT
Ann Intern Med 1984 Dec;101(6):743-8. doi: 10.7326/0003-4819-101-6-743. PMID: 6149715
A prospective study of Raynaud phenomenon and early connective tissue disease. A five-year report.
Harper FE, Maricq HR, Turner RE, Lidman RW, Leroy EC
Am J Med 1982 Jun;72(6):883-8. doi: 10.1016/0002-9343(82)90846-4. PMID: 7091158

Clinical prediction guides

A prospective study of Raynaud phenomenon and early connective tissue disease. A five-year report.
Harper FE, Maricq HR, Turner RE, Lidman RW, Leroy EC
Am J Med 1982 Jun;72(6):883-8. doi: 10.1016/0002-9343(82)90846-4. PMID: 7091158

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