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

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Abstract

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.

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Authors and Affiliations

  1. Faculty of Medicine, University of Belgrade, Institute of Rheumatology Belgrade, Serbia, Resavska 69, Belgrade, 11000, Serbia

    Slavica Pavlov-Dolijanovic, Nada Vujasinovic Stupar & Predrag Ostojic

  2. Faculty of Medicine University of Belgrade, Clinic for Pulmology, Clinical Center of Serbia, Belgrade, Serbia

    Vladimir Zugic

  3. Institute of Rheumatology, Resavska 69, Belgrade, 11000, Serbia

    Ana Zekovic, Tatjana Zivanovic Radnic & Ivan Jeremic

  4. Faculty of Pharmacy Department of Social Pharmacy and Pharmacy Legislation, University of Belgrade Faculty of Pharmacy, Vojvode Stepe 450, Belgrade, 11000, Serbia

    Ivana Tadic

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  1. Slavica Pavlov-Dolijanovic
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Correspondence to Slavica Pavlov-Dolijanovic.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Pavlov-Dolijanovic, S., Vujasinovic Stupar, N., Zugic, V. et al. Long-term effects of immunosuppressive therapy on lung function in scleroderma patients. Clin Rheumatol 37, 3043–3050 (2018). https://doi.org/10.1007/s10067-018-4266-0

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