TY - JOUR
T1 - High-dose prednisolone and bolus cyclophosphamide in interstitial lung disease associated with systemic sclerosis
T2 - A prospective open study
AU - Wanchu, Ajay
AU - Suryanaryana, Bettadpura Shamanna
AU - Sharma, Shefali
AU - Sharma, Aman
AU - Bambery, Pradeep
PY - 2009
Y1 - 2009
N2 - Aim: Currently, therapy for interstitial lung disease in patients with systemic sclerosis is unsatisfactory. A prospective open label study was conducted in a North Indian tertiary Institute to assess the efficacy of intermittent pulse cyclophosphamide (CYC) and high-dose prednisolone in systemic sclerosis (SSc)-related interstitial lung disease (ILD). Methods: Consecutive patients with SSc and ILD, diagnosed on spirometry, carbon monoxide diffusing capacity (DLCO) and high-resolution computed tomography (HRCT) scan were treated. Pulmonary function tests were carried out at baseline and after 6months. Patients received oral prednisolone 1 mg/kg body weight initially, with tapering to a dose of 7.5 mg/day was reached. Monthly CYC pulses were given for 6months followed by 3-monthly maintenance pulses. CYC was discontinued in patients with declining pulmonary function, adverse effects or static disease after 6 months. Results: Average disease duration of 36 patients was 59.78 ± 63.22 months. Seven patients improved (forced vital capacity [FVC] increase 10% or DLCO increase 15%), five deteriorated (FVC decline 10% or DLCO decline 15%) and 24 had stable disease. Thus, 31 out of 36 patients either improved or had static lung disease. Mean FVC (% of predicted) improved by 4.16% over 6months (P = 0.069). Mean DLCO (% of predicted) improved by 5.66% (P = 0.27). Average % of predicted DLCO at baseline was 39%. Conclusion: High-dose prednisolone with pulse CYC caneither improve or stabilize lung functions in patients with severe systemic sclerosis lung disease irrespective of presence of ground glass appearance on HRCT.
AB - Aim: Currently, therapy for interstitial lung disease in patients with systemic sclerosis is unsatisfactory. A prospective open label study was conducted in a North Indian tertiary Institute to assess the efficacy of intermittent pulse cyclophosphamide (CYC) and high-dose prednisolone in systemic sclerosis (SSc)-related interstitial lung disease (ILD). Methods: Consecutive patients with SSc and ILD, diagnosed on spirometry, carbon monoxide diffusing capacity (DLCO) and high-resolution computed tomography (HRCT) scan were treated. Pulmonary function tests were carried out at baseline and after 6months. Patients received oral prednisolone 1 mg/kg body weight initially, with tapering to a dose of 7.5 mg/day was reached. Monthly CYC pulses were given for 6months followed by 3-monthly maintenance pulses. CYC was discontinued in patients with declining pulmonary function, adverse effects or static disease after 6 months. Results: Average disease duration of 36 patients was 59.78 ± 63.22 months. Seven patients improved (forced vital capacity [FVC] increase 10% or DLCO increase 15%), five deteriorated (FVC decline 10% or DLCO decline 15%) and 24 had stable disease. Thus, 31 out of 36 patients either improved or had static lung disease. Mean FVC (% of predicted) improved by 4.16% over 6months (P = 0.069). Mean DLCO (% of predicted) improved by 5.66% (P = 0.27). Average % of predicted DLCO at baseline was 39%. Conclusion: High-dose prednisolone with pulse CYC caneither improve or stabilize lung functions in patients with severe systemic sclerosis lung disease irrespective of presence of ground glass appearance on HRCT.
KW - Corticosteroids
KW - Cyclophosphamide
KW - Interstitial lung disease
KW - Systemic sclerosis
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U2 - 10.1111/j.1756-185X.2009.01417.x
DO - 10.1111/j.1756-185X.2009.01417.x
M3 - Article
C2 - 20374353
AN - SCOPUS:69149101725
SN - 1756-1841
VL - 12
SP - 239
EP - 242
JO - International Journal of Rheumatic Diseases
JF - International Journal of Rheumatic Diseases
IS - 3
ER -