A lthough the development of pulmonary arterial hypertension (PAH) in mixed connective tissue disease (MCTD) is now recognised as the most important life threatening factor, an effective treatment for PAH has not been established. The response to steroid treatment of PAH related to MCTD varies. Furthermore, Raynaud's phenomenon is the most common symptom of MCTD and one symptom of the 1996 revised criteria for MCTD in Japan.
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