Mixed connective tissue disease

Mixed connective tissue disease
Classification and external resources
ICD-10 M35.1
ICD-9 710.8
DiseasesDB 8312
eMedicine med/3417
MeSH D008947

In medicine, mixed connective tissue disease (also known as Sharp's syndrome[1]), commonly abbreviated as MCTD, is an autoimmune disease, in which the body's defense system attacks itself. It was characterized in 1972.[2]

It is sometimes equated with the term "Undifferentiated connective tissue disease",[1]) but some sources specifically reject such assertions of equivalence.[3] The term was introduced by Leroy[4] in 1980.[5]

Clinical features

MCTD combines features of scleroderma, myositis, systemic lupus erythematosus, and rheumatoid arthritis[6] (with some sources adding polymyositis, dermatomyositis, and inclusion body myositis)[7] and is thus considered an overlap syndrome.

MCTD commonly causes:

Distinguishing laboratory characteristics are a positive, speckled anti-nuclear antibody and an anti-U1-RNP antibody.[8]

It has been associated with HLA-DR4.[9]

The prognosis for MCTD tends to be better than other autoimmune diseases due to lack of renal disease and response to corticosteroids.

References

  1. ^ a b Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0. 
  2. ^ Sharp GC, Irvin WS, Tan EM, Gould RG, Holman HR (February 1972). "Mixed connective tissue disease--an apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA)". Am. J. Med. 52 (2): 148–59. doi:10.1016/0002-9343(72)90064-2. PMID 4621694. http://linkinghub.elsevier.com/retrieve/pii/0002-9343(72)90064-2. 
  3. ^ John Stone (1 June 2009). Pearls & Myths in Rheumatology. Springer. pp. 169–. ISBN 9781848009332. http://books.google.com/?id=dZ1aJf4DViYC&pg=PA169. Retrieved 26 June 2010. 
  4. ^ George C. Tsokos; Caroline Gordon; Josef S. Smolen (2007). Systemic lupus erythematosus: a companion to Rheumatology. Elsevier Health Sciences. pp. 429–. ISBN 9780323044349. http://books.google.com/?id=4duiwNKAdX4C&pg=PA429. Retrieved 26 June 2010. 
  5. ^ LeRoy EC, Maricq HR, Kahaleh MB (March 1980). "Undifferentiated connective tissue syndromes". Arthritis Rheum. 23 (3): 341–3. doi:10.1002/art.1780230312. PMID 7362686. 
  6. ^ "mixed connective tissue disease" at Dorland's Medical Dictionary
  7. ^ "Mixed Connective Tissue Disease (MCTD): Autoimmune Disorders of Connective Tissue: Merck Manual Home Health Handbook". http://www.merckmanuals.com/home/bone_joint_and_muscle_disorders/autoimmune_disorders_of_connective_tissue/mixed_connective_tissue_disease_mctd.html. 
  8. ^ Venables PJ (2006). "Mixed connective tissue disease". Lupus 15 (3): 132–7. doi:10.1191/0961203306lu2283rr. PMID 16634365. 
  9. ^ Aringer M, Steiner G, Smolen JS (August 2005). "Does mixed connective tissue disease exist? Yes". Rheum. Dis. Clin. North Am. 31 (3): 411–20, v. doi:10.1016/j.rdc.2005.04.007. PMID 16084315. http://linkinghub.elsevier.com/retrieve/pii/S0889-857X(05)00020-7. 

External links

General
Other hypersensitivity/autoimmune
Other

M: MUS, DF+DRCT

anat (h/n, u, t/d, a/p, l)/phys/devp/hist

noco(m, s, c)/cong(d)/tumr, sysi/epon, injr

proc, drug (M1A/3)