High-dose prednisolone and bolus cyclophosphamide in interstitial lung disease associated with systemic sclerosis: A prospective open study

Ajay Wanchu, Bettadpura Shamanna Suryanaryana, Shefali Sharma, Aman Sharma, Pradeep Bambery

Research output: Contribution to journalArticle

22 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)239-242
Number of pages4
JournalInternational Journal of Rheumatic Diseases
Volume12
Issue number3
DOIs
StatePublished - 2009
Externally publishedYes

Fingerprint

Systemic Scleroderma
Interstitial Lung Diseases
Prednisolone
Cyclophosphamide
Prospective Studies
Vital Capacity
Lung Diseases
Tomography
Lung
Spirometry
Respiratory Function Tests
Carbon Monoxide
Glass
Body Weight
Maintenance

Keywords

  • Corticosteroids
  • Cyclophosphamide
  • Interstitial lung disease
  • Systemic sclerosis

ASJC Scopus subject areas

  • Rheumatology

Cite this

High-dose prednisolone and bolus cyclophosphamide in interstitial lung disease associated with systemic sclerosis : A prospective open study. / Wanchu, Ajay; Suryanaryana, Bettadpura Shamanna; Sharma, Shefali; Sharma, Aman; Bambery, Pradeep.

In: International Journal of Rheumatic Diseases, Vol. 12, No. 3, 2009, p. 239-242.

Research output: Contribution to journalArticle

Wanchu, Ajay ; Suryanaryana, Bettadpura Shamanna ; Sharma, Shefali ; Sharma, Aman ; Bambery, Pradeep. / High-dose prednisolone and bolus cyclophosphamide in interstitial lung disease associated with systemic sclerosis : A prospective open study. In: International Journal of Rheumatic Diseases. 2009 ; Vol. 12, No. 3. pp. 239-242.
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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.

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