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Systemic Sclerosis and Interstitial Lung Disease: A Pilot Study Using Pulse Intravenous Methylprednisolone and Cyclophosphamide to Assess the Effect on High Resolution Computed Tomography Scan and Lung Function BRIDGET GRIFFITHS, SARAH MILES, HILARY MOSS, ROD ROBERTSON, DOUGLAS VEALE, and PAUL EMERY
ABSTRACT.
Methods. Fourteen consecutive patients with SSc and lung involvement were treated with 6 pulses of IV MP (10 mg/kg) and IV CYC (15 mg/kg) given at 3-4 weekly intervals. HRCT scans and lung function tests were performed at baseline and after the 6th pulse. Further lung function tests were repeated at 12 months and annually thereafter. Results. Modified Rodnan skin scores improved significantly by 35% from a median baseline score of 17 (IQR 14-26.5) to a posttreatment score of 13 (IQR 10.5-18.5; p = 0.0058). HRCT scan scores improved significantly (p = 0.04). Twelve of 13 patients experienced either improvement or stabilization of the HRCT score. Median DLCO and lung volumes remained stable during the first 12 months. After a median followup of 26 months (IQR 19-43), 67% of patients experienced deterioration in DLCO. Median deterioration was 23% (IQR 44-0.6), with the median rate of deterioration of the predicted value of the DLCO/month being 0.87% (IQR 1.24-0.02). The treatment was safe and well tolerated. Conclusion. This IV regimen stabilized lung disease in patients with SSc. When treatment was stopped, or reduced in intensity, a deterioration in lung function occurred in the majority of patients. Rate of deterioration of DLCO may be a useful marker for determining the intensity of treatment. These findings have implications for treating lung disease and designing clinical trials in patients with SSc. (J Rheumatol 2002;29:2371-8) Key Indexing Terms:
SYSTEMIC SCLEROSIS From the Department of Rheumatology, Freeman Hospital, Newcastle Upon Tyne; the Rheumatology and Rehabilitation Research Unit, University of Leeds, Leeds; Leeds General Infirmary, Leeds, England; and St. Vincent's Hospital, Dublin, Ireland. B. Griffiths, MD, MRCP, Consultant Rheumatologist, Department of Rheumatology, Freeman Hospital; S. Miles, MRCP, Consultant Rheumatologist; P. Emery, MD, FRCP, ARC Professor of Rheumatology, Rheumatology and Rehabilitation Research Unit, University of Leeds; H. Moss, MRCP, FRCR, Consultant Radiologist; R. Robertson, MRCP, FRCR, Consultant Radiologist, Leeds General Infirmary; D. Veale, MD, FRCPI, Consultant Rheumatologist, St. Vincent's Hospital, Dublin. Address reprint requests to Dr. B. Griffiths, Department of Rheumatology, Musculoskeletal Unit, Level 2, Freeman Hospital, Newcastle Upon Tyne NE7 7DN, UK. E-mail: bridget.griffiths@ncl.ac.uk Submitted January 8, 2002; revision accepted May 6, 2002.
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