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Effect of steroid pulse therapy on mixed connective tissue disease with pulmonary arterial hypertension
  1. Y Kamata,
  2. H Nara,
  3. H Sato,
  4. J-I Masuyama,
  5. S Minota,
  6. T Yoshio
  1. Division of Rheumatology and Clinical Immunology, Jichi Medical School, 3311 Yakushiji, Minamikawachi-machi, Tochigi-ken, 329-0498, Japan
  1. Correspondence to:
    Dr T Yoshio
    takuyoshjichi.ac.jp

https://doi.org/10.1136/ard.2004.029074

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    Although 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.1 However, a reliable and consistently effective treatment for Raynaud’s phenomenon in MCTD has not been established.

    Sildenafil is reportedly effective for PAH and Raynaud’s phenomenon in systemic sclerosis (SSc) and systemic lupus erythematosus (SLE).2–,4 However, the effect of sildenafil on PAH and Raynaud’s phenomenon in MCTD has not been reported.

    CASE REPORT

    We describe a case of PAH and Raynaud’s phenomenon in a 39 year old female patient with MCTD, in …

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