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

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.

PORTUGUESE RECOMMENDATIONS FOR THE USE OF BIOLOGICAL THERAPIES IN PATIENTS WITH PSORIATIC ARTHRITIS--2015 UPDATE.

OBJECTIVE: To update recommendations for the treatment of psoriatic arthritis with biological therapies, endorsed by the Portuguese Society of Rheumatology (SPR). METHODS: These treatment recommendations were formulated by Portuguese rheumatologists based on literature evidence and consensus opinion. At a national meeting the 16 recommendations included in this document were discussed and updated. The level of agreement among Portuguese Rheumatologists was assessed using an online survey. A draft of the full text of the recommendations was then circulated and suggestions were incorporated. A final version was again circulated before publication. RESULTS: A consensus was achieved regarding the initiation, assessment of response and switching biological therapies in patients with psoriatic arthritis (PsA). Specific recommendations were developed for several disease domains: peripheral arthritis, axial disease, enthesitis and dactylitis. CONCLUSION: These recommendations may be used for guidance in deciding which patients with PsA should be treated with biological therapies. They cover a rapidly evolving area of therapeutic intervention. As more evidence becomes available and more biological therapies are licensed, these recommendations will have to be updated.