Recent updates in advanced pulmonary sarcoidosis

This review describes recent developments in the phenotyping, monitoring, and treatment of advanced pulmonary sarcoidosis (APS), highlighting the positive strides made to recognise and treat this severe form of disease.

Key to this is the use of recently described high-resolution chest computed tomography (HRCT)-based phenotyping, the prognostic use of fibrosis extent coupled with pulmonary function tests, early echocardiography, and the increasing application of FDG-PET CT as a functional marker of inflammatory burden. Treatment paradigms have changed, with a shift from excessive corticosteroid use to steroid-sparing agents with less cumulative toxicity. Sarcoidosis-associated pulmonary hypertension (SAPH) is a well described lethal complication of APS with multiple potential underlying pathogenetic mechanisms. Directed therapies are dependent on the underlying mechanism but often vasodilators would be trialled given the multifactorial cause of the disease once SAPH has been confirmed.

APS is used to describe end-stage sequelae of pulmonary sarcoidosis, including established fibrotic lung disease with or without active inflammation and sarcoidosis-associated pulmonary hypertension. These manifestations are associated with significant morbidity and mortality, underscoring the need for early assessment, multidisciplinary involvement and ongoing vigilance.