A case of mixed connective tissue disease successfully treated for hemophagocytic syndrome with intermittent intravenous injection of cyclophosphamide

Masashi Kato, Shinji Sato, Misako Suzuki, Hiroko Oka, Yuko Kaneko, Hidekata Yasuoka, Takaki Nojima, Akira Suwa, Michito Hirakata, Yasuo Ikeda

Research output: Contribution to journalArticlepeer-review

5 Citations (Scopus)

Abstract

A 52 year-old woman noticed general fatigue, polyarthralgia, and muscle weakness of lower extremities in October 2001. in December, she felt difficulty in walking due to muscle weakness, in January 2002, she admitted another hospital because of dyspnea on exertion and edema of lower extremities. Laboratory test revealed leukocytopenia, the elevation of creatine kinase and positive anti-U1-RNP antibodies. Her chest computed tomography (CT) showed severe interstitial pneumonia. Cardiac echogram revealed that she had pericardial effusion and pulmonary hypertension. Then she was transferred to Keio University Hospital and she was diagnosed as having mixed connective tissue disease (MCTD) manifestating myositis, interstitial pneumonia, pulmonary hypertension and pericarditis. Prednisolone (PSL) 60mg daily following to methylprednisolone (mPSL) pulse therapy was begun and her symptoms were gradually improved. in middle of February, she complained of high fever over 39.0°C. Bacterial culture tests were negative and laboratory data indicated pancytopenia and a high level of serum ferritin. Bone marrow aspiration revealed hemophagocytosis in bone marrow specimens and she was diagnosed as having hemophagocytic syndrome associated with MCTD. mPSL pulse therapy was not effective and intermittent cyclophosphamide pulse therapy (IV-CY) was performed resulting in improvement of the symptoms. This case suggested the effectiveness of IV-CY therapy in patients with corticosteroid-resistant HPS associated with connective tissue diseases.

Original languageEnglish
Pages (from-to)345-349
Number of pages5
JournalJapanese Journal of Clinical Immunology
Volume27
Issue number5
DOIs
Publication statusPublished - 2004

Keywords

  • Hemophagocytic syndrome (HPS)
  • Intermittent cyclophosphamide pulse therapy (IV-CY)
  • Mixed connective tissue disease (MCTD)

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

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