ERS 2019 – ALERT: Pathogenesis and prognosis of interstitial lung disease of known origin

  • Eliana Mesa
  • Conference Reports
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Hypersensitivity pneumonitis (HP) may be related to fungal exposure. A study was performed to determine whether a diagnostic strategy to detect potentially causative fungal agents in the patient’s home environment, would be useful. Standardised environmental sampling took place in the homes of eight patients whose serum samples were positive for antibodies against the standard HP screening antigen panel. Each individual had a positive response to more than one antigen from the environmental sample. This suggests that an indoor environmental study may be crucial to avoid exposure to the causative fungal antigen, allowing an appropriate diagnosis and management of HP. A clinical aspect of HP is the presence of squeaks (SQ), although their prevalence and correlation with clinical features are unknown. In a retrospective review of cases, the presence or absence of SQ was assessed. SQ were found in approximately 40% of patients with HP, more commonly females, and were associated with lower FEV1 and FVC, and higher RV/TLC. The presence of SQ may suggest the diagnosis of HP in the appropriate context.

Pulmonary fibrosis (PF) is a severe lung disease with poor prognosis and few treatment options. A number of studies have aimed to understand its pathogenic mechanisms. The “Precision-cut lung slices” (PCLS) is a novel experimental approach to identify and modulate PF biomarkers ex vivo . In the study presented, PCLS were prepared from lungs of rats treated with bleomycin (BLM) or NaCl and lung tissue from patients with PF (huPF-PCLS). PCLS from both BLM treated rats and huPF-PCLS showed the expression of relevant pro-fibrotic biomarkers. These mediators are efficiently inhibited by nintedanib. Other in vivo and in vitro studies have explored the effects of IL-6 on macrophage phenotypes and their impact on fibrosis. The hypothesis is based on a trial in systemic sclerosis with the anti-IL-6Rα (tocilizumab) that was shown to benefit lung function and decrease a subset of macrophage transcripts in the tissue of patients, which are implicated in the pathogenesis of lung fibrosis. Data recovered show that IL-6 induces a population of fibrogenic macrophages that can be targeted therapeutically, leading to reduced fibrotic disease. 

Dr. Camus presented data on Pneumotox metrics and the use of the web platform. He concluded that Pneumotox is used daily, almost worldwide, with discrepancies among countries and with “silent” zones of the world. Interestingly well-known drugs are accessed frequently while significant pneumotoxicants are not accessed.