Abstract
Interstitial lung diseases (ILDs) comprise a wide group of pulmonary parenchymal disorders. These patients may experience acute respiratory deteriorations of their respiratory condition, termed “acute exacerbation” (AE). The incidence of AE-ILD seems to be lower than idiopathic pulmonary fibrosis (IPF), but prognosis and prognostic factors are largely unrecognized. We retrospectively analyzed a cohort of 158 consecutive adult patients hospitalized for AE-ILD in two Italian university hospitals from 2009 to 2016. Patients included in the analysis were divided into two groups: non-IPF (62%) and IPF (38%). Among ILDs included in the non-IPF group, the most frequent diagnoses were non-specific interstitial pneumonia (NSIP) (42%) and connective tissue disease (CTD)-ILD (20%). Mortality during hospitalization was significantly different between the two groups: 19% in the non-IPF group and 43% in the IPF group. AEs of ILDs are difficult-to-predict events and are burdened by relevant mortality. Increased inflammatory markers, such as neutrophilia on the differential blood cell count (HR 1.02 (CI 1.01–1.04)), the presence of pulmonary hypertension (HR 1.85 (CI 1.17–2.92)), and the diagnosis of IPF (HR 2.31 (CI 1.55–3.46)), resulted in negative prognostic factors in our analysis. Otherwise, lymphocytosis on the differential count seemed to act as a protective prognostic factor (OR 0.938 (CI 0.884–0.995)). Further prospective, large-scale, real-world data are needed to support and confirm the impact of our findings.
Highlights
We retrospectively identified 4894 patients, accounting for 423 unique hospitalizations for acute respiratory failure
We collected 158 patients, 121 (69%) of which were admitted at San Gerardo Hospital, Monza, and 53 (31%) of which were admitted at the University Hospital of Modena meeting the inclusion and exclusion criteria for acute exacerbation” (AE)-Interstitial lung diseases (ILDs) (IPF and non-idiopathic pulmonary fibrosis (IPF))
Among ILDs included in the non-IPF group, the most frequent diagnoses were non-specific interstitial pneumonia (NSIP) (42%) and connective tissue disease (CTD)-ILD (20%)
Summary
Interstitial lung diseases (ILDs) comprise a wide group of pulmonary parenchymal disorders that are classified together because of similarities in their clinical presentation, chest radiographic appearance, and physiologic features. ILDs include idiopathic pulmonary fibrosis (IPF), hypersensitivity pneumonitis (HP), connective tissue disease-related. The prototype of progressive ILD is considered IPF [2], an important subset of patients with other fibrotic ILDs experience a decline in lung function with progressive symptoms, poor response to treatment, and reduced quality of life [3]. Prognostic factors include a detection of an UIP pattern, the severity of hypoxemia, and the alteration of pre-exacerbation lung functional parameters [6]. The incidence of AE-ILD seems to be lower than IPF, but prognosis and prognostic factors are largely unrecognized [8,9]
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