Peripheral Immune Cell Profiling Reveals Distinct Immune Hallmarks in Progressive Pulmonary Fibrosis

Progessive Pulmonary Fibrosis
«Peripheral Immune Cell Profiling Reveals Distinct Immune Hallmarks in Progressive Pulmonary Fibrosis» investigates Within the spectrum of fibrosing interstitial lung diseases (f-ILDs) there is a subset of patients who exhibit a similar clinical course and prognosis to those with idiopathic pulmonary fibrosis (IPF), named progressive pulmonary fibrosis (PPF). The mechanisms underlying PPF are unclear.

Observational study scope

This observational study included 33 patients with f-ILDs, other than IPF. Diagnosis of ILDs was established through a multidisciplinary discussion based on clinical characteristics along with HRCT scan, bronchoalveolar lavage, and lung biopsy patterns if appropriate.

Disease progression was defined as per international guidelines (ATS/ERS/JRS/ALAT), by ≥2 of the following criteria: worsening respiratory symptoms, lung function progression (absolute change in FVC ≥5% and/or DLCO ≥10%) and/or radiological progression.

33.3% patients exhibited PPF during follow-up

Eleven patients (33.3%) exhibited PPF during follow-up, whereas 22 (66.7%) remained stable. Table 1 presents the main demographic and clinical characteristics of these two groups at diagnosis, with further stratification by non-idiopathic pulmonary fibrosis subtypes available in Table S1.

Age, gender, smoking status and proportion of f-ILD subtypes were similar across main groups. Most patients were naïve to immunosuppressive and antifibrotic treatment.

In the entire population studied (n=33), we found significant correlations between several blood immune markers and lung function.


Novel Observations

The main and novel observations of this study are that:

  1. In the entire population of f-ILDs, several CD8 and CD4 subpopulations correlate with lung function severity at the time of diagnosis and
  2. NKT-like cells levels at diagnosis are associated with PPF.


In summary

This study shows that in patients with f-ILDs several immune populations in circulating blood, skewed toward an aged and exhausted immune profile, relate to lung function impairment at diagnosis, and that PPF is associated with an increased cytotoxic immune response. Collectively, these findings highlight biomarkers of potential clinical utility, although we acknowledge that these observations will have to be confirmed in larger cohorts.


Fernanda Hernandez-Gonzalez, Nuria Mendoza, Sandra Casas-Recasens, Tamara Cruz, Nuria Albacar, Gemma López-Saiz, Xavier Alsina-Restoy, Mauricio Rojas, Alvar Agusti, Jacobo Sellarés, Rosa Faner.

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