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.
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Abbreviations
- Cs A:
-
cyclosporin A
- FEV1 :
-
forced expiratory volume in 1 s
- FVC:
-
forced vital capacity
- IPF:
-
idiopathic pulmonary fibrosis
References
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 70:542–568
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 164:1988–2005
Hewitt CJ, Hull D, Keeling JW (1977) Fibrosing alveolitis in infancy and childhood. Arch Dis Child 52:22–37
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 127:18–22
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 67:1–9
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 136:288–292
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; 22:880–884
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 26:169–173
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 65:661–672
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Fukazawa, M., Kawano, M., Hisano, S. et al. Efficacy of cyclosporin A for idiopathic pulmonary fibrosis. Eur J Pediatr 149, 441–442 (1990). https://doi.org/10.1007/BF02009669
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DOI: https://doi.org/10.1007/BF02009669