Concomitant Systemic Sclerosis and Sarcoidosis with Combined Pulmonary Fibrosis and Emphysema

Combined pulmonary fibrosis and emphysema syndrome in a patient age 28 years with severe systemic sclerosis.

Combined pulmonary fibrosis and emphysema.

Combined pulmonary fibrosis and emphysema (CPFE) is a distinct clinical entity characterized by the simultaneous coexistence of both upper lobe emphysema and lower lobe pulmonary fibrosis. Although it was first described in 1990, it has not received enough recognition. Due to its unique lung function and clinical profile, pulmonologists should be aware of its existence while evaluating patients...

Combined Pulmonary Fibrosis and Emphysema (CPFE)

Emphysema is characterized by the permanent abnormal enlargement of airspaces distal to the terminal bronchioles, accompanied by destruction of their walls. The characteristics of emphysema do not, by definition, include thickening of the alveolar septa and fibrosis. However, coincidental idiopathic pulmonary fibrosis (IPF) and emphysema was firstly reported in 1990 by Wiggins et al (Wiggins J,...

Carbonylated proteins in bronchoalveolar lavage of patients with sarcoidosis, pulmonary fibrosis associated with systemic sclerosis and idiopathic pulmonary fibrosis.

Oxygen-derived free radicals produced by phagocytes have been postulated to contribute to lung tissue damage occurring during diffuse lung diseases (DLD). The two-dimensional electrophoretic (2-DE) analysis of bronchoalveolar lavage (BAL) protein composition revealed different protein profiles in sarcoidosis (S), idiopathic pulmonary fibrosis (IPF) and systemic sclerosis (SSc) with a significan...

Combined pulmonary fibrosis and emphysema syndrome associated with ABCA3 mutations.

We report a case of extensive upper-lobe emphysema and lower-lobe pulmonary fibrosis consistent with combined pulmonary fibrosis and emphysema (CPFE) syndrome in a nonsmoker adult patient. Sequence analysis of surfactant protein C gene (SFTPC, MIM 178620) and ATP-binding cassette subfamily A member 3 gene (ABCA3, MIM 601615) identified no mutation in SFTPC gene but compound heterozygosity in AB...