Exploring the Autoimmune Spectrum of Combined Pulmonary Fibrosis and Emphysema: Inflammatory Myopathy Cases and a...

Combined Pulmonary Fibrosis and Emphysema (CPFE) is a syndrome characterized by the coexistence of emphysema and pulmonary fibrosis on High-Resolution Computed Tomography (HRCT), typically presenting with preserved lung volumes and markedly reduced diffusing capacity, and may occur in association with connective tissue diseases. A systematic review of the literature was conducted to improve understanding of its clinical profile and present two unusual cases linked to idiopathic inflammatory myopathies.

A systematic search of Medline, Embase, and Scopus was performed following PRISMA guidelines. Sixty articles were included, addressing diagnosis, pathophysiology, risk factors, prognosis, and treatment. A summary of reported cases was also compiled.

An international consensus statement established a definition for clinical and research purposes, requiring emphysema to involve≥15% of the lung parenchyma. Reported risk factors include older age, male sex, smoking, mediastinal lymphadenopathy, fibrosis extent, high-titer rheumatoid factor, or anti-Ro/SSA antibodies. Prognostic determinants of mortality or progression include the extent of fibrosis, decline in DLCO, and pulmonary hypertension. Paraseptal emphysema and usual interstitial pneumonia are the most common radiologic findings. Twenty-seven studies reported patients, mainly associated with systemic sclerosis (19.7% in smokers, 7.5% in never-smokers) and rheumatoid arthritis, while only two cases were linked to myopathies. Management relies on immunosuppressive therapy directed at the underlying disease, with early initiation potentially modifying disease course. Additional measures include inhalation therapy, antifibrotic agents, pulmonary hypertension treatment, and rehabilitation.

CPFE is an uncommon but clinically relevant complication with distinct risk and prognostic factors. Pulmonary hypertension is frequent. Cases associated with myopathies are rare. Immunosuppressive therapy remains central and may slow disease progression.

CPFE is a distinct and clinically significant condition associated with increased morbidity and mortality, requiring accurate recognition and multidisciplinary management.