Prognosis of Mixed Connective Tissue Disease

What is the course and prognosis of MCTD?

Over a 10-year period, 58% of patients are still classified as MCTD. The remaining patients evolve into SSc (17%), SLE (9%), or RA (2.5%). There is a low incidence of life-threatening renal disease and neurologic disease in MCTD. The major mortality results from progressive PAH and its cardiac sequelae. The development of end-organ involvement dictates the morbidity and mortality.

As a general rule, the SLE-like features of arthritis and pleurisy are treated with NSAIDs, antimalarials, low-dose prednisone (<20 mg/day), and occasionally methotrexate. Inflammatory myositis is treated with high doses of prednisone (60 mg/day) and sometimes methotrexate or azathioprine. SSc-like features of Raynaud’s phenomenon, dysphagia, and reflux esophagitis are treated , Systemic Sclerosis. Serious end-organ involvement such as myocarditis and ILD may require aggressive treatment with corticosteroids alongside cyclophosphamide or mycophenolate. Some evidence suggests that PAH secondary to MCTD or SLE (but not SSc) may benefit from immunosuppressive medication such as cyclophosphamide (controversial given limited data, and should not replace standard therapy for PAH).

15585

Sign up to receive the trending updates and tons of Health Tips

Join SeekhealthZ and never miss the latest health information

15856