Dermatologic Manifestations of Mixed Connective Tissue Disease Follow-up
- Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD more...
Deterrence/Prevention
- Deterrence and prevention of mixed connective-tissue disease (MCTD) includes photoprotection.
Complications
- Approximately 10% of patients with mixed connective-tissue disease (MCTD) have an increased risk of cancer development during the course of disease.
Prognosis
- Prognosis for patients with mixed connective-tissue disease (MCTD) is better than for patients who have only one form of overlapping disease. Many patients will later progress to scleroderma or lupus; some will remain undifferentiated. In addition, myositis of MCTD may have a better prognosis than other forms of myositis.
- In one case series, solid tumors developed in approximately 10% of patients.
- Nephritis is associated with a poor prognosis. It is considered a common cause of death.
Patient Education
- Educate mixed connective-tissue disease (MCTD) patients regarding photoprotection. Patients should avoid sun exposure, which is a triggering and aggravating factor.
- Patients with Raynaud phenomenon should be instructed to avoid exposure to cold.
Aringer M, Steiner G, Smolen JS. Does mixed connective tissue disease exist? Yes. Rheum Dis Clin North Am. Aug 2005;31(3):411-20, v. [Medline].
Kattah NH, Kattah MG, Utz PJ. The U1-snRNP complex: structural properties relating to autoimmune pathogenesis in rheumatic diseases. Immunol Rev. Jan 2010;233(1):126-45. [Medline].
Gunnarsson R, Molberg O, Gilboe IM, Gran JT. The prevalence and incidence of mixed connective tissue disease: a national multicentre survey of Norwegian patients. Ann Rheum Dis. Mar 11 2011;[Medline].
Grader-Beck T, Wigley FM. Raynaud's phenomenon in mixed connective tissue disease. Rheum Dis Clin North Am. Aug 2005;31(3):465-81, vi. [Medline].
Hall S, Hanrahan P. Muscle involvement in mixed connective tissue disease. Rheum Dis Clin North Am. Aug 2005;31(3):509-17, vii. [Medline].
Swart JF, Wulffraat NM. Diagnostic workup for mixed connective tissue disease in childhood. Isr Med Assoc J. Aug-Sep 2008;10(8-9):650-2. [Medline].
Oh YB, Jun JB, Kim CK, et al. Mixed connective tissue disease associated with skin defects of livedoid vasculitis. Clin Rheumatol. 2000;19(5):381-4. [Medline].
Weber P, Ganser G, Frosch M, Roth J, Hülskamp G, Zimmer KP. Twenty-four hour intraesophageal pH monitoring in children and adolescents with scleroderma and mixed connective tissue disease. J Rheumatol. Nov 2000;27(11):2692-5. [Medline].
Fagundes MN, Caleiro MT, Navarro-Rodriguez T, et al. Esophageal involvement and interstitial lung disease in mixed connective tissue disease. Respir Med. Jun 2009;103(6):854-60. [Medline].
Aalokken TM, Lilleby V, Soyseth V, et al. Chest abnormalities in juvenile-onset mixed connective tissue disease: assessment with high-resolution computed tomography and pulmonary function tests. Acta Radiol. May 2009;50(4):430-6. [Medline].
Colin G, Nunes H, Hatron PY, Cadranel J, Tillie I, Wallaert B. [Clinical study of interstitial lung disease in mixed connective tissue disease]. Rev Mal Respir. Mar 2010;27(3):238-46. [Medline].
Lundberg IE. Cardiac involvement in autoimmune myositis and mixed connective tissue disease. Lupus. 2005;14(9):708-12. [Medline].
Gourley I, Bell AL, Biggart D. Kikuchi's disease as a presenting feature of mixed connective tissue disease. Clin Rheumatol. Jan 1995;14(1):104-7. [Medline].
Peller JS, Gabor GT, Porter JM, Bennett RM. Angiographic findings in mixed connective tissue disease. Correlation with fingernail capillary photomicroscopy and digital photoplethysmography findings. Arthritis Rheum. Jul 1985;28(7):768-74. [Medline].
Sharp GC, Irvin WS, Tan EM, Gould RG, Holman HR. Mixed connective tissue disease--an apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA). Am J Med. Feb 1972;52(2):148-59. [Medline].
Hof D, Raats JM, Pruijn GJ. Apoptotic modifications affect the autoreactivity of the U1 snRNP autoantigen. Autoimmun Rev. Jul 2005;4(6):380-8. [Medline].
Hasegawa EM, Caleiro MT, Fuller R, Carvalho JF. The frequency of anti-beta2-glycoprotein I antibodies is low and these antibodies are associated with pulmonary hypertension in mixed connective tissue disease. Lupus. Jun 2009;18(7):618-21. [Medline].
Seguchi M, Soejima Y, Tateishi A, et al. Mixed connective tissue disease with multiple organ damage: successful treatment with plasmapheresis. Intern Med. Dec 2000;39(12):1119-22. [Medline].
Yamamura K, Takahara M, Masunaga K, Sawabe T, Kitano M, Mashino T, et al. Subcutaneous calcification of the lower legs in a patient with mixed connective tissue disease. J Dermatol. Mar 21 2011;[Medline].

