Two-year experience with mycophenolate mofetil in patients with scleroderma lung disease: a case series

Int J Rheum Dis. 2014 Nov;17(8):923-8. doi: 10.1111/1756-185X.12399. Epub 2014 May 26.

Abstract

To assess the effect of mycophenolate mofetil (MMF) on pulmonary functions in patients with systemic sclerosis-associated lung disease (SSc-ILD) who experienced an inadequate response to first line cyclophosphamide (CYC) therapy. Twelve consecutive SSc-ILD patients who received MMF due to inadequate response to CYC as a first line agent, were retrospectively reviewed. Over the course of 2 years, pulmonary function tests (PFT) and high-resolution computed tomography (HRCT) scans were performed. Following initial baseline tests, PFTs were continued at a frequency of every 6 months and HRCT scans were performed every 12 months. After MMF treatment, values of forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO) improved in three (25%) and two (16.6%) patients, respectively. It is also noted that the evaluation of serial HCRT scans showed no change in 54.5% of patients. Our case series suggested that PFT and imaging scores seemed to be stabilized by MMF in SSc-ILD patients who were inadequate responders to CYC.

Keywords: interstitial lung disease; mycophenolate mofetil; scleroderma.

Publication types

  • Observational Study

MeSH terms

  • Adult
  • Aged
  • Anti-Inflammatory Agents, Non-Steroidal / therapeutic use*
  • Cyclophosphamide / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Lung Diseases, Interstitial / drug therapy*
  • Lung Diseases, Interstitial / etiology
  • Lung Diseases, Interstitial / physiopathology
  • Male
  • Middle Aged
  • Mycophenolic Acid / analogs & derivatives*
  • Mycophenolic Acid / therapeutic use
  • Pulmonary Diffusing Capacity
  • Retrospective Studies
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / drug therapy*
  • Scleroderma, Systemic / physiopathology
  • Vital Capacity

Substances

  • Anti-Inflammatory Agents, Non-Steroidal
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Mycophenolic Acid