ERS 2019 – State of the art session: Interstitial lung diseases (1st part)

  • Eliana Mesa
  • Conference Reports
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Firstly, the treatment of pulmonary sarcoidosis to reduce the risk of respiratory failure and/or improve the quality of life (QoL) was discussed. Today, there exists only two FDA-approved drugs for pulmonary sarcoidosis; prednisone and corticotropin, while only off-label medications are available for extrapulmonary sarcoidosis. Factors taken into account for who to treat for sarcoidosis depend on the health care provider’s assessment of; lung function, radiology, adverse effects, and patient status; QoL, functionality, survival, adverse effects. Factors of risk include anti-inflammatory (steroids, cytotoxics, anti-TNF), pulmonary hypertension (vasodilators), fibrosis, infections and lastly, transplant. The proposed clinical phenotype based on Delphi results are: Asymptomatic:  no therapy, Acute:  corticosteroids therapy, Chronic:  cytotoxic and other second-line therapies, Advanced : third-line therapy.
A stepwise-approach to therapy was suggested: Step 1: corticosteroids, Step 2:  antimetabolites, Step 3:  anti-TNF or infliximab, and Step 4:  additional agents (eg. rituximab). Steroid sparing agents such as methotrexate should be used to minimise long-term steroid toxicity. Newer agents are being explored (CLEAR, Anakinra, Nicotine) and other commercially-available drugs being investigated in sarcoidosis include; tofatinib, pentoxifylline, aprelimast, roflumilas.

Secondly, topics were discussed on how to recognise, stratify and prevent major complications in pulmonary sarcoidosis. Diagnosis includes histology with no caseating granulomas and exclusion of alternative diagnoses. Histology is needed to make a confident diagnosis. The majority of patients with sarcoidosis have a benign course but a minority have progressive pulmonary fibrosis which may shorten their life expectancy. Treatment is aimed at preventing the progression of the fibrosis or even reverse it. An algorithm for the management of sarcoidosis was proposed (Boucly A et al). In conclusion, a significant minority of patients with pulmonary sarcoidosis have a poor prognosis. Lung function impairment, the extent of fibrosis and pulmonary hypertension are key prognostic factors. The role of fluorodeoxyglucose-positron emission tomography (FDG-PET) in predicting the response to treatment in selected patients, particularly in the context of fibrosis, need to be further explored.