A case of mixed connective tissue disease with lupus-like manifestations of the central nervous system, successfully treated with cyclophosphamide combined with prednisolone

M. Kuroki, A. Okayama, G. Kuwata, M. Okamoto, G. Tanaka, M. Iga, Y. Kai, M. Matsuyama, K. Murai, H. Tsubouchi

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

A 29-year-old woman experienced Raynaud's phenomenon, swelling of her fingers, eruptions on her face, and muscle weakness in 1990. She was diagnosed as having mixed connective tissue disease (MCTD) and was treated with prednisolone (PSL) for 3 years. Most of her complaints disappeared after the treatment. In March 1997, she experienced fever, erythema, and lymphadenopathy. Although she was treated with PSL (20 mg/day) again, muscle weakness, mental disturbance, and recto-urinary disturbance appeared. When she was re-admitted to our hospital, increased levels of muscle-derived enzymes and positivity of anti-RNP antibody were found. High signal areas in her cerebrum were shown by magnetic resonance imaging, and slow and spike pattern was shown by electroencephalography. Hypoperfusion of the cerebral blood flow was suggested on single photon emission computed tomography. The number of mononuclear cells, amount of protein and level of interleukin-6 were found to be elevated in her cerebrospinal fluid (CSF). Her neurological manifestations were diagnosed as being due to MCTD, and showed characteristics similar to those of systemic lupus erythematosus. She was treated with PSL (60 mg/day) followed by steroid pulse therapy. Because the response to this treatment was partial, oral administration of cyclophosphamide (CPM) (100 mg/day) was added. Muscle weakness and neurological abnormalities as well as abnormal laboratory findings gradually improved over the following two months. We conclude that the treatment with CPM combined with PSL may be useful, when neurological manifestations of MCTD are serious and resistant to conventional therapy.

Original languageEnglish
Pages (from-to)627-632
Number of pages6
JournalRyumachi
Volume40
Issue number3
Publication statusPublished - Aug 7 2000

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Mixed Connective Tissue Disease
Prednisolone
Cyclophosphamide
Central Nervous System
Muscle Weakness
Neurologic Manifestations
Cerebrovascular Circulation
Therapeutics
Raynaud Disease
Cerebrum
Erythema
Single-Photon Emission-Computed Tomography
Systemic Lupus Erythematosus
Fingers
Oral Administration
Cerebrospinal Fluid
Electroencephalography
Anti-Idiotypic Antibodies
Interleukin-6
Fever

All Science Journal Classification (ASJC) codes

  • Rheumatology

Cite this

A case of mixed connective tissue disease with lupus-like manifestations of the central nervous system, successfully treated with cyclophosphamide combined with prednisolone. / Kuroki, M.; Okayama, A.; Kuwata, G.; Okamoto, M.; Tanaka, G.; Iga, M.; Kai, Y.; Matsuyama, M.; Murai, K.; Tsubouchi, H.

In: Ryumachi, Vol. 40, No. 3, 07.08.2000, p. 627-632.

Research output: Contribution to journalArticle

Kuroki, M, Okayama, A, Kuwata, G, Okamoto, M, Tanaka, G, Iga, M, Kai, Y, Matsuyama, M, Murai, K & Tsubouchi, H 2000, 'A case of mixed connective tissue disease with lupus-like manifestations of the central nervous system, successfully treated with cyclophosphamide combined with prednisolone', Ryumachi, vol. 40, no. 3, pp. 627-632.
Kuroki, M. ; Okayama, A. ; Kuwata, G. ; Okamoto, M. ; Tanaka, G. ; Iga, M. ; Kai, Y. ; Matsuyama, M. ; Murai, K. ; Tsubouchi, H. / A case of mixed connective tissue disease with lupus-like manifestations of the central nervous system, successfully treated with cyclophosphamide combined with prednisolone. In: Ryumachi. 2000 ; Vol. 40, No. 3. pp. 627-632.
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AU - Okamoto, M.

AU - Tanaka, G.

AU - Iga, M.

AU - Kai, Y.

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AU - Tsubouchi, H.

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