Antifibrotics in interstitial lung disease related to connective tissue diseases - a paradigm shift in treatment and outcome

Acta Reumatol Port. 2019 Apr-Jun;44(2):161-162.

Abstract

Interstitial lung disease (ILD) is one of the major causes of morbidity and mortality in patients with connective tissue disease (CTD) and the treatments available until nowadays are in most cases unable to halt disease progression. CTD-ILD pathogenesis includes an initial inflammatory phase, followed by a fibrotic phase, in which extracellular matrix proteins are produced and fibrotic scaring tissue within the lung develops. Steroids and immunosuppressants are the weapons we currently have to treat CTD-ILD. However, mortality rates remain high and identification of new therapeutic targets is crucial. Antifibrotic drugs, which include nintedanib and pirfenidone, have been approved for the treatment of idiopathic pulmonary fibrosis (IPF) and due to similar pathogenesis between IPF and CTD-ILD, their use seems attractive in patients with CTD-IL. We report 3 cases of patients with different CTDs, with predominantly fibrotic changes in high resolution computed tomography that progressed despite immunosuppression, and who have attained disease stability after introduction of antifibrotic drugs.

Publication types

  • Case Reports
  • Letter

MeSH terms

  • Aged
  • Azathioprine / therapeutic use
  • Cyclophosphamide / therapeutic use
  • Drug Substitution
  • Female
  • Humans
  • Hydroxychloroquine / therapeutic use
  • Indoles / therapeutic use*
  • Infliximab / therapeutic use
  • Lung Diseases, Interstitial / drug therapy*
  • Lung Diseases, Interstitial / etiology
  • Middle Aged
  • Mycophenolic Acid / therapeutic use
  • Prednisolone / therapeutic use
  • Pyridones / therapeutic use*
  • Scleroderma, Systemic / complications*
  • Sjogren's Syndrome / complications*
  • Treatment Outcome

Substances

  • Indoles
  • Pyridones
  • Hydroxychloroquine
  • Cyclophosphamide
  • Prednisolone
  • Infliximab
  • pirfenidone
  • nintedanib
  • Mycophenolic Acid
  • Azathioprine