In addition to facilitating healthcare delivery planning, reliable information about prognosis is essential for treatment decisions in patients with idiopathic pulmonary fibrosis (IPF). This review aimed to evaluate the prognosis of patients with IPF without anti-fibrotic therapy. We included all cohort studies and the placebo arms of randomised controlled trials (RCTs) in IPF and follow-up of ≥12 months. Two reviewers independently evaluated studies for inclusion, assessed risk of bias and extracted data. A total of 154 cohort studies and 16 RCTs were included. The pooled proportions of mortality were 0.12 (95% CI 0.09-0.14) at 1-2 years, 0.38 (95% CI 0.34-0.42) between 2-5 years, and 0.69 (95% CI 0.59-0.78) at ≥5 years. The pooled mean overall survival was 4 years (95% CI 3.7-4.6) for studies with a follow-up duration of 10 years. At <2 years, forced vital capacity and diffusing capacity of the lung for carbon monoxide declined by a mean of 6.76% predicted (95% CI -8.92 -4.61) and 3% predicted (95% CI -5.14 -1.52), respectively. Although heterogeneity was high, subgroup analyses revealed lower pooled proportions of mortality at 1 year in the RCT participants (0.07 (95% CI 0.05-0.09)) versus cohort study participants (0.14 (95% CI 0.12-0.17)). This review provides comprehensive information on the prognosis of IPF, which can inform treatment discussions with patients and comparisons for future studies with new therapies.

https://err.ersjournals.com/content/errev/29/157/190158.full.pdf

Prognosis of idiopathic pulmonary fibrosis without anti-fibrotic therapy: a systematic review

Pneumothorax: Classification and Etiology

PURPOSE

To conduct a meta-analysis to determine specific computed tomography (CT) patterns and clinical features that discriminate between nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP).

MATERIALS AND METHODS

The PubMed/Medline and Embase databases were searched for studies describing the radiological patterns of UIP and NSIP in chest CT images. Only studies involving histologically confirmed diagnoses and a consensus diagnosis by an interstitial lung disease (ILD) board were included in this analysis. The radiological patterns and patient demographics were extracted from suitable articles. We used random-effects meta-analysis by DerSimonian & Laird and calculated pooled odds ratios for binary data and pooled mean differences for continuous data.

RESULTS

Of the 794 search results, 33 articles describing 2,318 patients met the inclusion criteria. Twelve of these studies included both NSIP (338 patients) and UIP (447 patients). NSIP-patients were significantly younger (NSIP: median age 54.8 years, UIP: 59.7 years; mean difference (MD) -4.4; p = 0.001; 95% CI: -6.97 to -1.77), less often male (NSIP: median 52.8%, UIP: 73.6%; pooled odds ratio (OR) 0.32; p<0.001; 95% CI: 0.17 to 0.60), and less often smokers (NSIP: median 55.1%, UIP: 73.9%; OR 0.42; p = 0.005; 95% CI: 0.23 to 0.77) than patients with UIP. The CT findings from patients with NSIP revealed significantly lower levels of the honeycombing pattern (NSIP: median 28.9%, UIP: 73.4%; OR 0.07; p<0.001; 95% CI: 0.02 to 0.30) with less peripheral predominance (NSIP: median 41.8%, UIP: 83.3%; OR 0.21; p<0.001; 95% CI: 0.11 to 0.38) and more subpleural sparing (NSIP: median 40.7%, UIP: 4.3%; OR 16.3; p = 0.005; 95% CI: 2.28 to 117).

CONCLUSION

Honeycombing with a peripheral predominance was significantly associated with a diagnosis of UIP. The NSIP pattern showed more subpleural sparing. The UIP pattern was predominantly observed in elderly males with a history of smoking, whereas NSIP occurred in a younger patient population.

/pdf/meta-analysis-of-the-radiological-and-clinical-features-of-wjb4k3tpyk.pdf

Meta-analysis of the radiological and clinical features of Usual Interstitial Pneumonia (UIP) and Nonspecific Interstitial Pneumonia (NSIP)

Is Routine Chest Radiography After Transbronchial Biopsy Necessary? A Prospective Study Of 121 Patients

https://onlinelibrary.wiley.com/doi/pdf/10.1111/resp.13253

Secondary spontaneous pneumothorax in idiopathic pulmonary fibrosis: Grim news.

Upper Lobe Emphysema Is Associated With Lower Survival In Patients With Idiopathic Pulmonary Fibrosis

Correlation Between Pulmonary Artery Diameter And Mean Pulmonary Artery Pressure In IPF Patients

Some patients with diffuse interstitial lung disease (ILD) undergo bronchoscopy with transbronchial biopsy (TBB) as part of their diagnostic evaluation. It is unclear what the incidence and risk factors for pneumothorax (PTX) following TBB are in this patient population. Ninety-seven subjects with pulmonary fibrosis who underwent a research bronchoscopy with TBB as part of the multicenter correlating outcomes with biochemical markers to estimate time-progression in idiopathic pulmonary fibrosis (COMET) trial were retrospectively reviewed. We compared subjects who developed a PTX during research bronchoscopy with TBB versus those who did not. Seven patients (7.2%) experienced a PTX during research bronchoscopy with TBB. Subjects who experienced PTX during TBB had significantly lower DLCO percent predicted (29 ± 8 vs. 45 ± 15, P = 0.006) and had lower resting room air saturation of peripheral oxygen (SPO2) on 6-min walk testing (91 ± 10 vs. 95 ± 3, P = 0.02). No differences between groups were found with respect to age, gender, race, BMI, HRCT characteristics, or the number of transbronchial biopsies performed. The incidence of PTX following research bronchoscopy with TBB in patients with pulmonary fibrosis was found to be 7.2% in this study. Patients who developed a pneumothorax had greater impairments in gas exchange at baseline evidenced by a lower DLCO  % predicted and a lower resting room air SPO2 compared with subjects without PTX as a complication.

/pdf/pneumothorax-after-transbronchial-biopsy-in-pulmonary-2givi498j6.pdf

Pneumothorax After Transbronchial Biopsy in Pulmonary Fibrosis: Lessons from the Multicenter COMET Trial.

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Papers

Upper Lobe Emphysema Is Associated With Lower Survival In Patients With Idiopathic Pulmonary Fibrosis

Correlation Between Pulmonary Artery Diameter And Mean Pulmonary Artery Pressure In IPF Patients

Pneumothorax After Transbronchial Biopsy in Pulmonary Fibrosis: Lessons from the Multicenter COMET Trial.