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'''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 doesn't meet the criteria for any specific autoimmune disease, such as [[systemic lupus erythematosus]] or [[scleroderma]].<ref name=uctd /> Latent lupus and incomplete lupus erythematosus are alternative terms that have been used to describe this condition.<ref name=uctdreview />
'''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 doesn't meet the criteria for any specific autoimmune disease, such as [[systemic lupus erythematosus]] or [[scleroderma]].<ref name=uctd /> '''Latent lupus''' and '''incomplete lupus''' are alternative terms that have been used to describe this condition.<ref name=uctdreview />


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 RNP antibodies.<ref name=mctd />
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 RNP antibodies.<ref name=mctd />

It is estimated that up to 25 percent of people with systemic autoimmune disease could be considered to have UCTD.<ref name=prevalentild />


==Signs and symptoms==
==Signs and symptoms==
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<ref name=uctd>{{cite journal | last=Bodolay | first=E. | last2=Szegedi | first2=G. | date=May 2009 | title=Undifferentiated connective tissue disease | journal=Orvosi Hetilap | language=Hungarian | publisher=Akadémiai Kiadó | volume=150 | issue=19 | pages=867-872 | doi=10.1556/OH.2009.28610 | issn=1788-6120 | pmid=19403430 | authorformat=vanc | author-separator=, | author-name-separator=&#32; }}</ref>
<ref name=uctd>{{cite journal | last=Bodolay | first=E. | last2=Szegedi | first2=G. | date=May 2009 | title=Undifferentiated connective tissue disease | journal=Orvosi Hetilap | language=Hungarian | publisher=Akadémiai Kiadó | volume=150 | issue=19 | pages=867-872 | doi=10.1556/OH.2009.28610 | issn=1788-6120 | pmid=19403430 | authorformat=vanc | author-separator=, | author-name-separator=&#32; }}</ref>
<ref name=uctdreview>{{cite journal | last=Mosca | first=M. | last2=Neri | first2=R. | last3=Bombardieri | first3=S. | year=1999 | title=Undifferentiated connective tissue diseases (UCTD): a review of the literature and a proposal for preliminary classification criteria | url=http://www.clinexprheumatol.org/article.asp?a=1936 | format=PDF | journal=Clinical and Experimental Rheumatology | volume=17 | issue=5 | pages=615 | pmid=10544849 | authorformat=vanc | author-separator=, | author-name-separator=&#32; }}</ref>
<ref name=uctdreview>{{cite journal | last=Mosca | first=M. | last2=Neri | first2=R. | last3=Bombardieri | first3=S. | year=1999 | title=Undifferentiated connective tissue diseases (UCTD): a review of the literature and a proposal for preliminary classification criteria | url=http://www.clinexprheumatol.org/article.asp?a=1936 | format=PDF | journal=Clinical and Experimental Rheumatology | volume=17 | issue=5 | pages=615 | pmid=10544849 | authorformat=vanc | author-separator=, | author-name-separator=&#32; }}</ref>
<ref name=prevalentild>{{cite journal | last=Lunardi | first=Francesca | last2=Balestro | first2=Elisabetta | last3=Nordio | first3=Beatrice | last4=Cozzi | first4=Franco | last5=Polverosi | first5=Roberta | last6=Sfriso | first6=Paolo | last7=Braccioni | first7=Fausto | last8=Calabrese | first8=Fiorella | display-authors=5 | date=June 2011 | title=Undifferentiated connective tissue disease presenting with prevalent interstitial lung disease: Case report and review of literature | url=http://www.diagnosticpathology.org/content/6/1/50/ | journal=Diagnostic Pathology | volume=6 | issue=50 | pages=1-4 | doi=10.1186/1746-1596-6-50 | pmc=3126759 | pmid=21645423 | authorformat=vanc | author-separator=, | author-name-separator=&#32; }}</ref>
<ref name=mctd>{{vcite book | author=Robert W. Hoffman; Eric L. Greidinger | chapter=Mixed connective tissue disease | editor=George C. Tsokos | title=Modern Therapeutics in Rheumatic Diseases | publisher=Humana Press | year=2002 | doi=10.1007/978-1-59259-239-5_23 | isbn=978-1-59259-239-5 | chapterid=Chapter 23 | pages=347-357}}</ref>
<ref name=mctd>{{vcite book | author=Robert W. Hoffman; Eric L. Greidinger | chapter=Mixed connective tissue disease | editor=George C. Tsokos | title=Modern Therapeutics in Rheumatic Diseases | publisher=Humana Press | year=2002 | doi=10.1007/978-1-59259-239-5_23 | isbn=978-1-59259-239-5 | chapterid=Chapter 23 | pages=347-357}}</ref>
<ref name=defininguctd>{{cite journal | last=Doria | first=A. | last2=Mosca | first2=M. | last3=Gambari | first3=P.F. | last4=Bombardieri | first4=S. | date=February 2005 | title=Defining unclassifiable connective tissue diseases: incomplete, undifferentiated, or both? | url=http://www.jrheum.org/content/32/2/213.long | format=PDF | journal=The Journal of Rheumatology | volume=32 | issue=2 | pages=213-215 | pmid=15693073 | authorformat=vanc | author-separator=, | author-name-separator=&#32; }}</ref>
<ref name=defininguctd>{{cite journal | last=Doria | first=A. | last2=Mosca | first2=M. | last3=Gambari | first3=P.F. | last4=Bombardieri | first4=S. | date=February 2005 | title=Defining unclassifiable connective tissue diseases: incomplete, undifferentiated, or both? | url=http://www.jrheum.org/content/32/2/213.long | format=PDF | journal=The Journal of Rheumatology | volume=32 | issue=2 | pages=213-215 | pmid=15693073 | authorformat=vanc | author-separator=, | author-name-separator=&#32; }}</ref>
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<ref name=petroleumrisk>{{cite journal | last=Lacey | first=JV Jr | last2=Garabrant | first2=DH | last3=Laing | first3=TJ | last4=Gillespie | first4=BW | last5=Mayes | first5=MD | last6=Cooper | first6=BC | last7=Schottenfeld | first7=D | display-authors=5 | date=April 1999 | title=Petroleum distillate solvents as risk factors for undifferentiated connective tissue disease (UCTD) | url=http://aje.oxfordjournals.org/content/149/8/761.full.pdf+html | journal=American Journal of Epidemiology | publisher=Oxford University Press | volume=149 | issue=8 | pages=761-770 | doi=10.1093/oxfordjournals.aje.a009885 | pmid=10206626 | authorformat=vanc | author-separator=, | author-name-separator=&#32; }}</ref>
<ref name=petroleumrisk>{{cite journal | last=Lacey | first=JV Jr | last2=Garabrant | first2=DH | last3=Laing | first3=TJ | last4=Gillespie | first4=BW | last5=Mayes | first5=MD | last6=Cooper | first6=BC | last7=Schottenfeld | first7=D | display-authors=5 | date=April 1999 | title=Petroleum distillate solvents as risk factors for undifferentiated connective tissue disease (UCTD) | url=http://aje.oxfordjournals.org/content/149/8/761.full.pdf+html | journal=American Journal of Epidemiology | publisher=Oxford University Press | volume=149 | issue=8 | pages=761-770 | doi=10.1093/oxfordjournals.aje.a009885 | pmid=10206626 | authorformat=vanc | author-separator=, | author-name-separator=&#32; }}</ref>
<ref name=meddevicesrisk>{{cite journal | last=Laing | first=TJ | last2=Schottenfeld | first2=D | last3=Lacey | first3=JV Jr | last4=Gillespie | first4=BW | last5=Garabrant | first5=DH | last6=Cooper | first6=BC | last7=Heeringa | first7=SG | last8=Alcser | first8=KH | last9=Mayes | first9=MD | display-authors=5 | date=October 2001 | title=Potential risk factors for undifferentiated connective tissue disease among women: implanted medical devices | url=http://aje.oxfordjournals.org/content/154/7/610.full | journal=American Journal of Epidemiology | publisher=Oxford University Press | volume=154 | issue=7 | pages=610-617 | doi=10.1093/aje/154.7.610 | pmid=11581094 | authorformat=vanc | author-separator=, | author-name-separator=&#32; }}</ref>
<ref name=meddevicesrisk>{{cite journal | last=Laing | first=TJ | last2=Schottenfeld | first2=D | last3=Lacey | first3=JV Jr | last4=Gillespie | first4=BW | last5=Garabrant | first5=DH | last6=Cooper | first6=BC | last7=Heeringa | first7=SG | last8=Alcser | first8=KH | last9=Mayes | first9=MD | display-authors=5 | date=October 2001 | title=Potential risk factors for undifferentiated connective tissue disease among women: implanted medical devices | url=http://aje.oxfordjournals.org/content/154/7/610.full | journal=American Journal of Epidemiology | publisher=Oxford University Press | volume=154 | issue=7 | pages=610-617 | doi=10.1093/aje/154.7.610 | pmid=11581094 | authorformat=vanc | author-separator=, | author-name-separator=&#32; }}</ref>
<ref name=uctd2004>{{cite journal | last=Mosca | first=M | last2=Baldini | first2=C | last3=Bombardieri | first3=S | year=2004 | title=Undifferentiated connective tissue diseases in 2004 | url=http://www.clinexprheumatol.org/article.asp?a=2414 | format=PDF | journal=Clinical and Experimental Rheumatology | publisher= | volume=22 | issue=3 Suppl 33 | pages=S17 | pmid=15344591 | authorformat=vanc | author-separator=, | author-name-separator=&#32; }}</ref>
<ref name=uctd2004>{{cite journal | last=Mosca | first=M | last2=Baldini | first2=C | last3=Bombardieri | first3=S | year=2004 | title=Undifferentiated connective tissue diseases in 2004 | url=http://www.clinexprheumatol.org/article.asp?a=2414 | format=PDF | journal=Clinical and Experimental Rheumatology | publisher= | volume=22 | issue=3 Suppl 33 | pages=S14-S18 | pmid=15344591 | authorformat=vanc | author-separator=, | author-name-separator=&#32; }}</ref>
<ref name=fiveyearfollowup>{{cite journal | last=Bodolay | first=E | last2=Csiki | first2=Z | last3=Szekanecz | first3=Z | last4=Ben | first4=T | last5=Kiss | first5=E | last6=Zeher | first6=M | last7=Szucs | first7=G | last8=Danko | first8=K | last9=Szegedi | first9=G | display-authors=5 | year=2003 | title=Five-year follow-up of 665 Hungarian patients with undifferentiated connective tissue disease (UCTD) | journal=Clinical and Experimental Rheumatology | volume=21 | issue=3 | pages=313-320 | pmid=12846049 | authorformat=vanc | author-separator=, | author-name-separator=&#32; }}</ref>
<ref name=fiveyearfollowup>{{cite journal | last=Bodolay | first=E | last2=Csiki | first2=Z | last3=Szekanecz | first3=Z | last4=Ben | first4=T | last5=Kiss | first5=E | last6=Zeher | first6=M | last7=Szucs | first7=G | last8=Danko | first8=K | last9=Szegedi | first9=G | display-authors=5 | year=2003 | title=Five-year follow-up of 665 Hungarian patients with undifferentiated connective tissue disease (UCTD) | journal=Clinical and Experimental Rheumatology | volume=21 | issue=3 | pages=313-320 | pmid=12846049 | authorformat=vanc | author-separator=, | author-name-separator=&#32; }}</ref>
<ref name=vitamind>{{cite journal | last=Zold | first=Eva | last2=Szodoray | first2=Peter | last3=Gaal | first3=Janos | last4=Kappelmayer | first4=Janos | last5=Csathy | first5=Laszlo | last6=Gyimesi | first6=Edit | last7=Zeher | first7=Margit | last8=Szegedi | first8=Gyula | last9=Bodolay | first9=Edit | display-authors=5 | year=2008 | title=Vitamin D deficiency in undifferentiated connective tissue disease | url=http://arthritis-research.com/content/10/5/R123 | journal=Arthritis Research and Therapy | volume=10 | issue=5 | pages=R123 | doi=10.1186/ar2533 | pmc=2592813 | pmid=18928561 | authorformat=vanc | author-separator=, | author-name-separator=&#32; }}</ref>}}
<ref name=vitamind>{{cite journal | last=Zold | first=Eva | last2=Szodoray | first2=Peter | last3=Gaal | first3=Janos | last4=Kappelmayer | first4=Janos | last5=Csathy | first5=Laszlo | last6=Gyimesi | first6=Edit | last7=Zeher | first7=Margit | last8=Szegedi | first8=Gyula | last9=Bodolay | first9=Edit | display-authors=5 | year=2008 | title=Vitamin D deficiency in undifferentiated connective tissue disease | url=http://arthritis-research.com/content/10/5/R123 | journal=Arthritis Research and Therapy | volume=10 | issue=5 | pages=R123 | doi=10.1186/ar2533 | pmc=2592813 | pmid=18928561 | authorformat=vanc | author-separator=, | author-name-separator=&#32; }}</ref>}}

Revision as of 18:27, 7 December 2014

Undifferentiated connective tissue disease
SpecialtyImmunology, rheumatology Edit this on Wikidata

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 doesn't 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 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, malaise, and fever.[6] Other symptoms associated with UCTD include:[7]

Risk Factors

One study has shown a link between exposure to petroleum distillates, and increased risk of UCTD.[8] Another study suggests that implanted medical devices are a possible risk factor, as well.[9]

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. [10]

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.[11]

Severe vitamin D deficiency has been associated with the progression of UCTD into defined connective tissue diseases.[12] 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.[10]

References

  1. ^ Bodolay, E.; Szegedi, G. (May 2009). "Undifferentiated connective tissue disease". Orvosi Hetilap (in Hungarian). 150 (19). Akadémiai Kiadó: 867–872. doi:10.1556/OH.2009.28610. ISSN 1788-6120. PMID 19403430. {{cite journal}}: Unknown parameter |author-name-separator= ignored (help); Unknown parameter |author-separator= ignored (help); Unknown parameter |authorformat= ignored (help)
  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. {{cite journal}}: Unknown parameter |author-name-separator= ignored (help); Unknown parameter |author-separator= ignored (help); Unknown parameter |authorformat= ignored (help)
  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. ^ Lunardi, Francesca; Balestro, Elisabetta; Nordio, Beatrice; Cozzi, Franco; Polverosi, Roberta; 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 3126759. PMID 21645423. {{cite journal}}: Unknown parameter |author-name-separator= ignored (help); Unknown parameter |author-separator= ignored (help); Unknown parameter |authorformat= ignored (help)CS1 maint: unflagged free DOI (link)
  5. ^ Owlia, Mohammad Bagher (2006). "Clinical spectrum of connective tissue disorders" (PDF). Journal, Indian Academy of Clinical Medicine. 7 (3): 218. {{cite journal}}: Unknown parameter |author-name-separator= ignored (help); Unknown parameter |author-separator= ignored (help); Unknown parameter |authorformat= ignored (help)
  6. ^ a b Doria, A.; Mosca, M.; Gambari, P.F.; Bombardieri, S. (February 2005). "Defining unclassifiable connective tissue diseases: incomplete, undifferentiated, or both?" (PDF). The Journal of Rheumatology. 32 (2): 213–215. PMID 15693073. {{cite journal}}: Unknown parameter |author-name-separator= ignored (help); Unknown parameter |author-separator= ignored (help); Unknown parameter |authorformat= ignored (help)
  7. ^ Vaz, C.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. {{cite journal}}: Cite journal requires |journal= (help); Unknown parameter |author-name-separator= ignored (help); Unknown parameter |author-separator= ignored (help); Unknown parameter |authorformat= ignored (help)
  8. ^ Lacey, JV Jr; Garabrant, DH; Laing, TJ; Gillespie, BW; Mayes, MD; et al. (April 1999). "Petroleum distillate solvents as risk factors for undifferentiated connective tissue disease (UCTD)". American Journal of Epidemiology. 149 (8). Oxford University Press: 761–770. doi:10.1093/oxfordjournals.aje.a009885. PMID 10206626. {{cite journal}}: Unknown parameter |author-name-separator= ignored (help); Unknown parameter |author-separator= ignored (help); Unknown parameter |authorformat= ignored (help)
  9. ^ Laing, TJ; Schottenfeld, D; Lacey, JV Jr; Gillespie, BW; Garabrant, DH; et al. (October 2001). "Potential risk factors for undifferentiated connective tissue disease among women: implanted medical devices". American Journal of Epidemiology. 154 (7). Oxford University Press: 610–617. doi:10.1093/aje/154.7.610. PMID 11581094. {{cite journal}}: Unknown parameter |author-name-separator= ignored (help); Unknown parameter |author-separator= ignored (help); Unknown parameter |authorformat= ignored (help)
  10. ^ a b 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. {{cite journal}}: Unknown parameter |author-name-separator= ignored (help); Unknown parameter |author-separator= ignored (help); Unknown parameter |authorformat= ignored (help)
  11. ^ 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. {{cite journal}}: Unknown parameter |author-name-separator= ignored (help); Unknown parameter |author-separator= ignored (help); Unknown parameter |authorformat= ignored (help)
  12. ^ Zold, Eva; Szodoray, Peter; Gaal, Janos; Kappelmayer, Janos; Csathy, Laszlo; et al. (2008). "Vitamin D deficiency in undifferentiated connective tissue disease". Arthritis Research and Therapy. 10 (5): R123. doi:10.1186/ar2533. PMC 2592813. PMID 18928561. {{cite journal}}: Unknown parameter |author-name-separator= ignored (help); Unknown parameter |author-separator= ignored (help); Unknown parameter |authorformat= ignored (help)CS1 maint: unflagged free DOI (link)
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