IPF Prognosis Generally Better for Women and Younger People, Registry-based Study Says

Female sex, younger age, and a better lung function at time of diagnosis tend to support a a better overall prognosis among people with idiopathic pulmonary fibrosis (IPF), a large registry-based study reports.

In contrast, the presence of pulmonary hypertension (PH), lung cancer, and long-term use of oxygen therapy were associated with a higher risk of death.

The study, “The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis,” was published in the journal Respiratory Research.

Although the prognosis for IPF is usually poor, its clinical course is highly variable, ranging from a rapid decline in lung function in some patients to a slower progression in others.

Patient registries are important not only to characterize the course of IPF in untreated patients (natural history studies), but also to evaluate the effectiveness and safety of therapies used to manage the disease. Those include Esbriet (pirfenidone), marketed by the Roche subsidiary Genentech, and Ofev (nintedanib), marketed by Boehringer Ingelheim.

However, most IPF registries are restricted to a single country, and only contain data from a small number patients who are often followed for a short period of time.

Researchers involved in the European MultiPartner IPF Registry (EMPIRE) studied data from 1,620 IPF patients enrolled in the registry, with the aim of estimating mortality rates and validating prognostic factors that might be used to estimate disease progression and survival.

EMPIRE has been enrolling IPF patients from 10 central and eastern European countries since 2014. Its goal is to assess IPF prevalence and mortality, as well as the basic characteristics of patients in the region, who aren’t well represented in most other IPF registries.

The study used long-term data from the 1,620 patients, who had a mean age at diagnosis of 67.6, from the registry’s start until October 2018. Some 71% were male, and 63% had a smoking history.

Most of the patients (74.9%) were diagnosed within six months before enrollment in EMPIRE. About 40% of all patients were receiving or had previously received treatment for IPF, including immunosuppressants and anti-fibrotic medications, at or prior to enrollment.

According to the GAP staging system — a risk-prediction index for IPF — 47.7% of the patients were at stage 1, 43.8% at stage 2, and 8.5% at stage 3.

The GAP model takes into account patients’ gender, age, and two lung function measures — forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DLCO or TLCO) — to estimate mortality risk. Stage 1 indicates a better prognosis.

Most patients (89.2%) were still alive one year after being diagnosed with IPF. Median survival dropped to 65.5% in the three years following diagnosis, and to 46.4% in the five years following diagnosis. The median overall survival of patients in the registry was 4.5 years.

Some 357 deaths were reported during follow-up. The mortality rate was higher among those with GAP stages 2 and 3.

Those who were younger, female, and had higher values of FVC and TLCO (indicating better lung function) at the time of diagnosis had a better overall prognosis.

In contrast, those who had PH, lung cancer, or had been on long-term oxygen therapy at the time of diagnosis had a higher risk of death (2.04 times higher for those with PH; 2.55 times higher for those with lung cancer; and 1.49 times higher for those on long-term oxygen therapy). The association with higher mortality was “above and beyond the effect of GAP,” the researchers said.

“In all, with its large size of over 1,600 patients with IPF with long follow-up, the EMPIRE registry provided some novelty by its more accurate confirmation of the prognostic factors and co-morbidities on longer term five-year mortality,” they said.

The results suggest “that some fine-tuning of the indices for mortality may provide a more accurate long-term prognostic profile for these patients,” the researchers said. Adjustments to the GAP index to better account for age and TLCO values could make prognosis more precise, they said.