- Mixed connective tissue disease
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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:
- joint pain/swelling,
- malaise,
- Raynaud phenomenon,
- Sjögren's syndrome,
- muscle inflammation, and
- sclerodactyly (thickening of the skin of the pads of the fingers)
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
- ^ a b Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ "mixed connective tissue disease" at Dorland's Medical Dictionary
- ^ "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.
- ^ Venables PJ (2006). "Mixed connective tissue disease". Lupus 15 (3): 132–7. doi:10.1191/0961203306lu2283rr. PMID 16634365.
- ^ 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
Systemic CT disorders (M32–M36, 710) General Inflammatory myopathy/Myositis: Dermatopolymyositis (Dermatomyositis/Juvenile dermatomyositis, Polymyositis) · Inclusion body myositisOverlap syndrome / Mixed connective tissue diseaseOther hypersensitivity/autoimmune Other Categories:- Connective tissue diseases
- Diseases involving the fasciae
- Systemic connective tissue disorders
- Disease stubs
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