Mixed connective tissue disease natural history, complications and prognosis: Difference between revisions
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==Overview== | ==Overview== | ||
If the patients with mixed connective tissue disease left untreated, approximately 35% of patients may progress to develop lung [[fibrosis]]. Common [[Complication (medicine)|complications]] of MCTD include [[polyarthritis]], [[Raynaud's phenomenon]], [[interstitial lung disease]], [[Esophageal motility disorder|esophageal dysmotility]], [[sclerodactyly]], [[polymyositis]], and [[pulmonary hypertension]]. Major causes of [[morbidity]] in long-term MCTD patients include [[sclerodactyly]], diffuse [[sclerosis]], [[Pulmonary hypertension|pulmonary arterial hypertension]], and [[nervous system disease]]. The presence of [[pulmonary]] involvement is associated with worst prognosis and high [[mortality rate]]. | |||
==Natural History, Complications, and Prognosis== | ==Natural History, Complications, and Prognosis== | ||
===Natural History=== | ===Natural History=== | ||
* If left untreated, approximately 35% of patients with mixed connective tissue disease may progress to develop lung fibrosis.<ref name="pmid22550317">{{cite journal| author=Gunnarsson R, Aaløkken TM, Molberg Ø, Lund MB, Mynarek GK, Lexberg AS et al.| title=Prevalence and severity of interstitial lung disease in mixed connective tissue disease: a nationwide, cross-sectional study. | journal=Ann Rheum Dis | year= 2012 | volume= 71 | issue= 12 | pages= 1966-72 | pmid=22550317 | doi=10.1136/annrheumdis-2011-201253 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22550317 }}</ref> | * The symptoms of mixed connective tissue disease usually develop in the first decades of life, and presents with [[Symptom|symptoms]] of other [[Rheumatology|rheumatologic]] diseases such as [[SLE]], [[systemic sclerosis]], [[Polymyositis and dermatomyositis|polymyositis]], or [[rheumatoid arthritis]].<ref name="pmid224241932">{{cite journal| author=Ortega-Hernandez OD, Shoenfeld Y| title=Mixed connective tissue disease: an overview of clinical manifestations, diagnosis and treatment. | journal=Best Pract Res Clin Rheumatol | year= 2012 | volume= 26 | issue= 1 | pages= 61-72 | pmid=22424193 | doi=10.1016/j.berh.2012.01.009 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22424193 }}</ref> | ||
* If left untreated, approximately 35% of patients with mixed connective tissue disease may progress to develop lung [[fibrosis]].<ref name="pmid22550317">{{cite journal| author=Gunnarsson R, Aaløkken TM, Molberg Ø, Lund MB, Mynarek GK, Lexberg AS et al.| title=Prevalence and severity of interstitial lung disease in mixed connective tissue disease: a nationwide, cross-sectional study. | journal=Ann Rheum Dis | year= 2012 | volume= 71 | issue= 12 | pages= 1966-72 | pmid=22550317 | doi=10.1136/annrheumdis-2011-201253 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22550317 }}</ref> | |||
===Complications=== | ===Complications=== | ||
*Common complications of MCTD include:<ref name="pmid24461387">{{cite journal |vauthors=Tani C, Carli L, Vagnani S, Talarico R, Baldini C, Mosca M, Bombardieri S |title=The diagnosis and classification of mixed connective tissue disease |journal=J. Autoimmun. |volume=48-49 |issue= |pages=46–9 |date=2014 |pmid=24461387 |doi=10.1016/j.jaut.2014.01.008 |url=}}</ref><ref name="pmid27453743">{{cite journal |vauthors=Nica AE, Alexa LM, Ionescu AO, Andronic O, Păduraru DN |title=Esophageal disorders in mixed connective tissue diseases |journal=J Med Life |volume=9 |issue=2 |pages=141–3 |date=2016 |pmid=27453743 |pmc=4863503 |doi= |url=}}</ref><ref name="pmid15716315">{{cite journal |vauthors=Bodolay E, Szekanecz Z, Dévényi K, Galuska L, Csípo I, Vègh J, Garai I, Szegedi G |title=Evaluation of interstitial lung disease in mixed connective tissue disease (MCTD) |journal=Rheumatology (Oxford) |volume=44 |issue=5 |pages=656–61 |date=May 2005 |pmid=15716315 |doi=10.1093/rheumatology/keh575 |url=}}</ref> | *Common [[Complication (medicine)|complications]] of MCTD include:<ref name="pmid24461387">{{cite journal |vauthors=Tani C, Carli L, Vagnani S, Talarico R, Baldini C, Mosca M, Bombardieri S |title=The diagnosis and classification of mixed connective tissue disease |journal=J. Autoimmun. |volume=48-49 |issue= |pages=46–9 |date=2014 |pmid=24461387 |doi=10.1016/j.jaut.2014.01.008 |url=}}</ref><ref name="pmid27453743">{{cite journal |vauthors=Nica AE, Alexa LM, Ionescu AO, Andronic O, Păduraru DN |title=Esophageal disorders in mixed connective tissue diseases |journal=J Med Life |volume=9 |issue=2 |pages=141–3 |date=2016 |pmid=27453743 |pmc=4863503 |doi= |url=}}</ref><ref name="pmid15716315">{{cite journal |vauthors=Bodolay E, Szekanecz Z, Dévényi K, Galuska L, Csípo I, Vègh J, Garai I, Szegedi G |title=Evaluation of interstitial lung disease in mixed connective tissue disease (MCTD) |journal=Rheumatology (Oxford) |volume=44 |issue=5 |pages=656–61 |date=May 2005 |pmid=15716315 |doi=10.1093/rheumatology/keh575 |url=}}</ref> | ||
**[[Polyarthritis]] | **[[Polyarthritis]] | ||
**[[Raynaud's phenomenon]] | **[[Raynaud's phenomenon]] | ||
| Line 22: | Line 26: | ||
===Prognosis=== | ===Prognosis=== | ||
*In patients with MCTD the prognosis is relatively good.<ref name="pmid23756459">{{cite journal |vauthors=Dabiri G, Falanga V |title=Connective tissue ulcers |journal=J Tissue Viability |volume=22 |issue=4 |pages=92–102 |date=November 2013 |pmid=23756459 |pmc=3930159 |doi=10.1016/j.jtv.2013.04.003 |url=}}</ref> | |||
*In MCTD, [[prognosis]] depends on the type of organ involvement.<ref name="pmid24353496">{{cite journal |vauthors=Latuśkiewicz-Potemska J, Zygmunt A, Biernacka-Zielińska M, Stańczyk J, Smolewska E |title=Mixed connective tissue disease presenting with progressive scleroderma symptoms in a 10-year-old girl |journal=Postepy Dermatol Alergol |volume=30 |issue=5 |pages=329–36 |date=October 2013 |pmid=24353496 |pmc=3858664 |doi=10.5114/pdia.2013.38365 |url=}}</ref> | *In MCTD, [[prognosis]] depends on the type of organ involvement.<ref name="pmid24353496">{{cite journal |vauthors=Latuśkiewicz-Potemska J, Zygmunt A, Biernacka-Zielińska M, Stańczyk J, Smolewska E |title=Mixed connective tissue disease presenting with progressive scleroderma symptoms in a 10-year-old girl |journal=Postepy Dermatol Alergol |volume=30 |issue=5 |pages=329–36 |date=October 2013 |pmid=24353496 |pmc=3858664 |doi=10.5114/pdia.2013.38365 |url=}}</ref> | ||
*Major causes of [[morbidity]] in long-term MCTD patients include:<ref name="pmid27436003">{{cite journal |vauthors=Ciang NC, Pereira N, Isenberg DA |title=Mixed connective tissue disease-enigma variations? |journal=Rheumatology (Oxford) |volume=56 |issue=3 |pages=326–333 |date=March 2017 |pmid=27436003 |doi=10.1093/rheumatology/kew265 |url=}}</ref> | *Major causes of [[morbidity]] in long-term MCTD patients include:<ref name="pmid27436003">{{cite journal |vauthors=Ciang NC, Pereira N, Isenberg DA |title=Mixed connective tissue disease-enigma variations? |journal=Rheumatology (Oxford) |volume=56 |issue=3 |pages=326–333 |date=March 2017 |pmid=27436003 |doi=10.1093/rheumatology/kew265 |url=}}</ref> | ||
| Line 28: | Line 33: | ||
**[[Pulmonary hypertension|Pulmonary arterial hypertension]] | **[[Pulmonary hypertension|Pulmonary arterial hypertension]] | ||
**[[Nervous system disease]] | **[[Nervous system disease]] | ||
*The | *The presence of [[pulmonary]] involvement is associated with worst prognosis and high [[mortality rate]].<ref name="pmid22424193">{{cite journal |vauthors=Ortega-Hernandez OD, Shoenfeld Y |title=Mixed connective tissue disease: an overview of clinical manifestations, diagnosis and treatment |journal=Best Pract Res Clin Rheumatol |volume=26 |issue=1 |pages=61–72 |date=February 2012 |pmid=22424193 |doi=10.1016/j.berh.2012.01.009 |url=}}</ref> | ||
==References== | ==References== | ||
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{{WH}} | {{WH}} | ||
{{WS}} | {{WS}} | ||
[[Category: | [[Category:Medicine]] | ||
[[Category:Up-To-Date]] | |||
[[Category:Rheumatology]] | |||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[2]
Overview
If the patients with mixed connective tissue disease left untreated, approximately 35% of patients may progress to develop lung fibrosis. Common complications of MCTD include polyarthritis, Raynaud's phenomenon, interstitial lung disease, esophageal dysmotility, sclerodactyly, polymyositis, and pulmonary hypertension. Major causes of morbidity in long-term MCTD patients include sclerodactyly, diffuse sclerosis, pulmonary arterial hypertension, and nervous system disease. The presence of pulmonary involvement is associated with worst prognosis and high mortality rate.
Natural History, Complications, and Prognosis
Natural History
- The symptoms of mixed connective tissue disease usually develop in the first decades of life, and presents with symptoms of other rheumatologic diseases such as SLE, systemic sclerosis, polymyositis, or rheumatoid arthritis.[1]
- If left untreated, approximately 35% of patients with mixed connective tissue disease may progress to develop lung fibrosis.[2]
Complications
- Common complications of MCTD include:[3][4][5]
- Polyarthritis
- Raynaud's phenomenon
- Puffy fingers (sausage digits)
- Interstitial lung disease
- Esophageal dysmotility
- Sclerodactyly
- Polymyositis
- Pulmonary hypertension
Prognosis
- In patients with MCTD the prognosis is relatively good.[6]
- In MCTD, prognosis depends on the type of organ involvement.[7]
- Major causes of morbidity in long-term MCTD patients include:[8]
- The presence of pulmonary involvement is associated with worst prognosis and high mortality rate.[9]
References
- ↑ Ortega-Hernandez OD, Shoenfeld Y (2012). "Mixed connective tissue disease: an overview of clinical manifestations, diagnosis and treatment". Best Pract Res Clin Rheumatol. 26 (1): 61–72. doi:10.1016/j.berh.2012.01.009. PMID 22424193.
- ↑ Gunnarsson R, Aaløkken TM, Molberg Ø, Lund MB, Mynarek GK, Lexberg AS; et al. (2012). "Prevalence and severity of interstitial lung disease in mixed connective tissue disease: a nationwide, cross-sectional study". Ann Rheum Dis. 71 (12): 1966–72. doi:10.1136/annrheumdis-2011-201253. PMID 22550317.
- ↑ Tani C, Carli L, Vagnani S, Talarico R, Baldini C, Mosca M, Bombardieri S (2014). "The diagnosis and classification of mixed connective tissue disease". J. Autoimmun. 48-49: 46–9. doi:10.1016/j.jaut.2014.01.008. PMID 24461387.
- ↑ Nica AE, Alexa LM, Ionescu AO, Andronic O, Păduraru DN (2016). "Esophageal disorders in mixed connective tissue diseases". J Med Life. 9 (2): 141–3. PMC 4863503. PMID 27453743.
- ↑ Bodolay E, Szekanecz Z, Dévényi K, Galuska L, Csípo I, Vègh J, Garai I, Szegedi G (May 2005). "Evaluation of interstitial lung disease in mixed connective tissue disease (MCTD)". Rheumatology (Oxford). 44 (5): 656–61. doi:10.1093/rheumatology/keh575. PMID 15716315.
- ↑ Dabiri G, Falanga V (November 2013). "Connective tissue ulcers". J Tissue Viability. 22 (4): 92–102. doi:10.1016/j.jtv.2013.04.003. PMC 3930159. PMID 23756459.
- ↑ Latuśkiewicz-Potemska J, Zygmunt A, Biernacka-Zielińska M, Stańczyk J, Smolewska E (October 2013). "Mixed connective tissue disease presenting with progressive scleroderma symptoms in a 10-year-old girl". Postepy Dermatol Alergol. 30 (5): 329–36. doi:10.5114/pdia.2013.38365. PMC 3858664. PMID 24353496.
- ↑ Ciang NC, Pereira N, Isenberg DA (March 2017). "Mixed connective tissue disease-enigma variations?". Rheumatology (Oxford). 56 (3): 326–333. doi:10.1093/rheumatology/kew265. PMID 27436003.
- ↑ Ortega-Hernandez OD, Shoenfeld Y (February 2012). "Mixed connective tissue disease: an overview of clinical manifestations, diagnosis and treatment". Best Pract Res Clin Rheumatol. 26 (1): 61–72. doi:10.1016/j.berh.2012.01.009. PMID 22424193.