Mixed connective tissue disease natural history, complications and prognosis
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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 or second decade of life, and start 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 MCTD, prognosis depends on the type of organ involvement.[6]
- Major causes of morbidity in long-term MCTD patients include:[7]
- The presence of pulmonary involvement is associated with worst prognosis and high mortality rate.[8]
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.
- ↑ 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.