Prognosis
Mixed connective tissue disease (MCTD) and the other overlap syndromes are chronic diseases that have highly variable courses. The overall outlook is defined by the severity of individual organ involvement. Some patients will have minor symptoms easily controlled with few pharmacologic interventions. Others will have progressive internal organ dysfunction, with the development of life-threatening complications that may or may not be responsive to immunosuppressive therapy. In these patients the onset of pulmonary hypertension, cardiac involvement, or interstitial lung disease each portends a poorer prognosis, and they are indications for aggressive immunosuppressive therapy. Pulmonary hypertension is the commonest disease-related cause of death in patients with MCTD.[6]
Although overall prognosis is largely determined by the internal organ involvement and the response to treatment, morbidity due to complications of therapy takes on progressively more importance as the need for aggressive immunosuppressive regimens increases.
Patients with antisynthetase syndrome are generally considered to have a poor prognosis compared to myositis without antisynthetase syndrome, likely related to the progression of interstitial lung disease in this group.[21]
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