Undifferentiated connective tissue disease

Undifferentiated connective tissue disease
Classification and external resources
ICD-10 M35.9
ICD-9-CM 710.9

Undifferentiated connective tissue disease (UCTD) is a disease in which the body mistakenly attacks its own tissues. It is diagnosed when there is evidence of an existing autoimmune condition which does not meet the criteria for any specific autoimmune disease, such as systemic lupus erythematosus or scleroderma.[1] Latent lupus and incomplete lupus are alternative terms that have been used to describe this condition.[2]

The term is sometimes used interchangeably with mixed connective tissue disease, an overlap syndrome. However, MCTD is thought by some researchers to be a clinically distinct entity and is strongly associated with the presence of high titers of ribonucleoprotein (RNP) antibodies.[3]

It is estimated that up to 25 percent of people with systemic autoimmune disease could be considered to have UCTD.[4]

Signs and symptoms

Disease presentation varies widely from patient to patient, as UCTD is by definition nonspecific.[5] Symptoms typically include constitutional complaints that are common to connective tissue diseases such as fatigue, a general sense of feeling unwell, and fever.[6] Other symptoms associated with UCTD include:[7]

Lung involvement, such as nonspecific interstitial pneumonia, is a possible disease complication.[4]

Diagnosis

There is no official diagnostic criteria for UCTD. Diagnostic testing generally aims to determine whether a patient has a definite or undifferentiated connective tissue disease.[6]

Treatment

Treatment largely depends upon individual disease progression and the nature of presenting symptoms. Antimalarials, corticosteroids, and other drugs may be prescribed, if deemed appropriate by the treating physician.[8]

Prognosis

Most patients will maintain a diagnosis of undifferentiated connective tissue disease. However, about one third of UCTD patients will differentiate to a specific autoimmune disease, like rheumatoid arthritis or systemic sclerosis. About 12 percent of patients will go into remission.[9]

Severe vitamin D deficiency has been associated with the progression of UCTD into defined connective tissue diseases.[10] The presence of the autoantibodies anti-dsDNA, anti-Sm, and anti-cardiolipin has been shown to correlate with the development of systemic lupus erythematosus, specifically.[8]

References

  1. ↑ Bodolay E, Szegedi G (May 2009). "Undifferentiated connective tissue disease". Orvosi Hetilap (in Hungarian). Akadémiai Kiadó. 150 (19): 867–872. doi:10.1556/OH.2009.28610. ISSN 1788-6120. PMID 19403430.
  2. ↑ Mosca M, Neri R, Bombardieri S (1999). "Undifferentiated connective tissue diseases (UCTD): a review of the literature and a proposal for preliminary classification criteria" (PDF). Clinical and Experimental Rheumatology. 17 (5): 615. PMID 10544849.
  3. ↑ Robert W. Hoffman; Eric L. Greidinger. Mixed connective tissue disease. In: George C. Tsokos. Modern Therapeutics in Rheumatic Diseases. Humana Press; 2002. doi:10.1007/978-1-59259-239-5_23. ISBN 978-1-59259-239-5. Chapter 23, p. 347-357.
  4. 1 2 Lunardi F, Balestro E, Nordio B, Cozzi F, Polverosi R, et al. (June 2011). "Undifferentiated connective tissue disease presenting with prevalent interstitial lung disease: Case report and review of literature". Diagnostic Pathology. 6 (50): 1–4. doi:10.1186/1746-1596-6-50. PMC 3126759Freely accessible. PMID 21645423.
  5. ↑ Owlia MB (2006). "Clinical spectrum of connective tissue disorders" (PDF). Journal, Indian Academy of Clinical Medicine. 7 (3): 218.
  6. 1 2 Doria A, Mosca M, Gambari P, Bombardieri S (February 2005). "Defining unclassifiable connective tissue diseases: incomplete, undifferentiated, or both?" (PDF). The Journal of Rheumatology. 32 (2): 213–215. PMID 15693073.
  7. ↑ Vaz C, Couto M, Medeiros D, Miranda L, Costa J, et al. (August 2009). "Undifferentiated connective tissue disease: a seven-center cross-sectional study of 184 patients". 28 (8). Springer-Verlag: 915–921. doi:10.1007/s10067-009-1175-2. ISSN 1434-9949. PMID 19390908.
  8. 1 2 Mosca M, Baldini C, Bombardieri S (2004). "Undifferentiated connective tissue diseases in 2004" (PDF). Clinical and Experimental Rheumatology. 22 (3 Suppl 33): S14–S18. PMID 15344591.
  9. ↑ Bodolay E, Csiki Z, Szekanecz Z, Ben T, Kiss E, et al. (2003). "Five-year follow-up of 665 Hungarian patients with undifferentiated connective tissue disease (UCTD)". Clinical and Experimental Rheumatology. 21 (3): 313–320. PMID 12846049.
  10. ↑ Zold E, Szodoray P, Gaal J, Kappelmayer J, Csathy L, et al. (2008). "Vitamin D deficiency in undifferentiated connective tissue disease". Arthritis Research & Therapy. 10 (5): R123. doi:10.1186/ar2533. PMC 2592813Freely accessible. PMID 18928561.
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