European Journal of Pediatrics

, Volume 149, Issue 6, pp 441–442 | Cite as

Efficacy of cyclosporin A for idiopathic pulmonary fibrosis

  • M. Fukazawa
  • M. Kawano
  • S. Hisano
  • K. Ueda
  • K. Matsuba
Pneumology

Abstract

In an 11-year-old girl with severe dyspnoea and cyanosis, idiopathic pulmonary fibrosis (IPF) was diagnosed. The PaO2 of blood could be maintained between 50 mm Hg and 70 mm Hg with a high dose pulse corticosteroid therapy, although this dose could not be reduced. Azathioprine in addition to prednisolone provoked deterioration. Twelve weeks after admission, cyclosporin A (Cs A), a known immunosuppressant, was started together with prednisolone. The PaO2 reverted to normal within 1 month and prednisolone could be tapered off within 6 months. Two years after admission, the patient is free of symptoms on continuous Cs A therapy. This case seems to be the first case successfully treated with Cs A.

Key words

Idiopathic pulmonary fibrosis Cyclosporin A Corticosteroid 

Abbreviations

Cs A

cyclosporin A

FEV1

forced expiratory volume in 1 s

FVC

forced vital capacity

IPF

idiopathic pulmonary fibrosis

References

  1. 1.
    Crystal RG, Gadek JE, Ferrans VJ, Fulmer JD, Line BR, Hunninghake GW (1981) Interstitial lung disease: current concepts of pathogensis, staging and therapy. Am J Med 70:542–568CrossRefPubMedGoogle Scholar
  2. 2.
    Hara T, Jung LKL, Bjorndahl JM, Fu SM (1986) Human T cell activation. III. Rapid induction of a phosphorylated 28 kD/32 kD disulfilde-linked early activation antigen (EA 1) by 12-o-Tetradecanoyl phorbol-13-acetate, mitogens, and antigens. J Exp Med 164:1988–2005CrossRefPubMedGoogle Scholar
  3. 3.
    Hewitt CJ, Hull D, Keeling JW (1977) Fibrosing alveolitis in infancy and childhood. Arch Dis Child 52:22–37PubMedGoogle Scholar
  4. 4.
    Keogh BA, Bernardo J, Hunninghake GW, Line BR, Price DL, Crystal RG (1983) Effect of intermittent high dose parenteral corticosteroids on the alveolitis of idiopathic pulmonary fibrosis. Am Rev Respir Dis 127:18–22PubMedGoogle Scholar
  5. 5.
    Nagai S, Fujimura N, Hirata T, Izumi T (1985) Differentiation between idiopathic pulmonary fibrosis and interstitial pneumonia associated with collagen vascular diseases by comparison of the ratio of OKT4+ cells and OKT8+ cells in BALF T lymphocytes. Eur J Respir Dis 67:1–9PubMedGoogle Scholar
  6. 6.
    O'Donnell K, Keogh B, Cantin A, Crystal RG (1987) Pharmacologic suppression of the neutrophil component of the alveolitis in idiopathic pulmonary fibrosis. Am Rev Respir Dis 136:288–292PubMedGoogle Scholar
  7. 7.
    Okudaira H, Ogita T, Miyamoto T, Shiga I, Suko M, Okudaira K, Terada E, Ghoda A, Terada K, Saito M, Nomura T (1984) Treatment of interstitial pneumonia in autoimmune mice with Cyclosporin A. Japanese J Thorac Dis 1984; 22:880–884Google Scholar
  8. 8.
    Sendelbach LE, Lindenschmidt RC, Witschi HP (1985) The effect of cyclosporin A on pulmonary fibrosis induced by butylated hydroxytoluene, bleomycin and beryllium sulfate. Toxicol Lett 26:169–173CrossRefPubMedGoogle Scholar
  9. 9.
    Winterbauer RH, Hammar SP, Hallman KO, Hays JE, Pardee NE, Morgan EH, Allen JD, Moores KD, Bush W, Walker JH (1978) Diffuse interstitial pneumonitis: clinicopathologic correlations in 20 patients treated with prednisone/azathioprine. Am J Med 65:661–672CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag 1990

Authors and Affiliations

  • M. Fukazawa
    • 1
  • M. Kawano
    • 1
  • S. Hisano
    • 1
  • K. Ueda
    • 1
  • K. Matsuba
    • 2
  1. 1.Department of PaediatricsFukuoka 812Japan
  2. 2.Research Institute for Diseases of the Chest, Faculty of MedicineKyushu UniversityFukuoka 812Japan

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