Long-term effects of immunosuppressive therapy on lung function in scleroderma patients

Clin Rheumatol. 2018 Nov;37(11):3043-3050. doi: 10.1007/s10067-018-4266-0. Epub 2018 Aug 24.


The study aims to analyze the effects of induction treatment with cyclophosphamide (CYC) pulse therapy followed by maintenance treatment with other mild immunosuppressive agents on lung function in scleroderma (SSc) patients. Thirty patients with SSc (mean age 52 years, mean disease duration < 2 years) with forced vital capacity (FVC) ≤ 80% and/or diffusing capacity of carbon monoxide (DLco) ≤ 70% were included. Monthly CYC pulses were given for 6 months (induction treatment), followed by 3-monthly maintenance pulses for the next 18 months, and during the next 5 years patients received other mild immunosupressive therapy brought by the competent rheumatologist. The efficacy was evaluated by comparing FVC% and DLco% after 6, 24, and 84 months from the baseline. All patients completed induction and maintenance treatment with CYC. Three patients were lost to follow-up. The rest of 27 patients, during the next 5 years, received other immunosupressive agents (14 azathioprine, 9 methotrexate, and 4 mycophenolate mofetil). Three patients died in the 4 years of follow-up. By 6, 24, and 84 months, the mean FVC and DLco changes were + 0.47 and + 2.10, + 3.30 and - 2.49, and + 1.53 and - 3.76%, respectively. These changes were not significantly different from the baseline values. CYC does not appear to result in clinically significant improvement of pulmonary function but fulfilled criteria of stable disease. Maintenance treatment with other mild immunosupressive agents preserves the benefits achieved during CYC treatment.

Keywords: Cyclophosphamide pulse therapy; Follow-up; Immunosuppressants; Interstitisal lung disease; Lung function; Systemic sclerosis.

MeSH terms

  • Adult
  • Carbon Monoxide / blood
  • Cyclophosphamide / administration & dosage*
  • Female
  • Humans
  • Immunosuppressive Agents / administration & dosage*
  • Lung / physiopathology*
  • Male
  • Middle Aged
  • Pulse Therapy, Drug / methods
  • Scleroderma, Systemic / drug therapy*
  • Scleroderma, Systemic / physiopathology*
  • Time Factors
  • Treatment Outcome
  • Vital Capacity


  • Immunosuppressive Agents
  • Carbon Monoxide
  • Cyclophosphamide