Prevalence of distress and changes over time among patients with idiopathic pulmonary fibrosis and progressive pulmonary fibrosis.

OBJECTIVES

Patients with idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) frequently experience respiratory symptoms and declines in activities of daily living (ADL) and quality of life (QOL). However, effective treatment and palliative care strategies remain limited. This study aimed to clarify physical and psychological distress and to evaluate 1-year symptom changes in patients with IPF and PPF.

METHODS

Consecutive patients with IPF or PPF were recruited and evaluated at registration (T0), 26 weeks (T1), and 52 weeks (T2). Symptoms, ADL, QOL, depressive condition, laboratory findings, and other factors were assessed throughout the 52-week follow-up.

RESULTS

Of the 70 patients, 32 had IPF and 38 had PPF. Repeated-measures analysis of variance showed that dyspnea in patients with IPF worsened from T1 to T2 (p = 0.02). In contrast, cough, which is one of the major symptoms in PPF worsened from T0 to T1 (p = 0.01) and T0 to T2 (p = 0.02). QOL in patients with PPF also declined from T0 to T1 (p = 0.03).

SIGNIFICANCE OF

RESULTS

Patients with IPF and PPF demonstrated several clinical signs and symptoms. In this 1-year follow-up study, dyspnea tended to be worse in patients with IPF and cough in PPF patients, and QOL was temporarily worse in PPF patients. Respiratory symptoms are directly related to the patient's daily life and lead to reduced QOL. Therefore, further long-term studies in this population are required.