Abstract
BackgroundCombined pulmonary fibrosis and emphysema (CPFE) is an umbrella term encompassing upper lobe emphysema and lower lobe pulmonary fibrosis with pathogenesis elusive. The aim of our study was to investigate the incidence of autoimmune markers in patients with CPFE.MethodsIn this multicenter study we retrospectively evaluated records from patients with CPFE (n=40) and IPF (n=60) without emphysema. Baseline demographic characteristics, high-resolution computed tomography (HRCT), spirometry, histopathological, treatment, serum immunologic and survival data were investigated. B cell presence was estimated with CD20 immunostaining in representative lung biopsy samples from CPFE patients and control subjects.ResultsA statistically significant increased number of CPFE patients with elevated serum ANA with or without positive p-ANCA titers compared to patients with IPF without emphysema was observed. Patients with CPFE and positive autoimmune markers exhibited improved survival compared to patients with a negative autoimmune profile. A massive infiltration of clusters of CD20+ B cells forming lymphoid follicles within the fibrotic lung in CPFE patients with positive serum immunologic profile compared to patients with negative profile, was noted and positively correlated with improved survival.ConclusionsA significant proportion of patients with CPFE may present with underlying auto-immune disorders that may reside insidiously and be associated with favorable prognosis. Early identification of these patients using a panel of auto-antibodies may lead to more targeted and effective therapeutic applications.
Highlights
Combined pulmonary fibrosis and emphysema (CPFE) is an umbrella term encompassing upper lobe emphysema and lower lobe pulmonary fibrosis with pathogenesis elusive
The combination of pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome, encompassing a distinct radiologic, revealing both upper lobe emphysema and lower lobe fibrosis in high resolution computed tomography (HRCT) of the chest, as well as lung function profile, with apparently preserved lung volumes contrasting with disproportionally impaired gas exchange, as assessed by reduced diffusing lung capacity for carbon monoxide (DLco) [1,2,3]
2) An increased number of CPFE patients with elevated serum pANCA titers (7/40, 17.5%) compared to none with idiopathic pulmonary fibrosis (IPF) without emphysema (p < 0.05). The latter finding was accompanied by an increased prevalence of microscopic polyangiitis (MPA) (3 out of 7 patients with positive p-antineutrophil cytoplasm antibodies (ANCA) profile) based on renal biopsy showing pauci-immune necrotizing glomerulonephritis coupled with diffuse alveolar hemorrhage while in the remaining 4 patients immunological clinical and serological findings were suggestive of an occult autoimmune disorder compatible with systemic vasculitis namely MPA. 3) A statistically significant improved survival of patients with CPFE and positive autoimmune profile compared to those with negative one suggesting that presence of autoimmunity may be associated with favorable prognosis
Summary
Combined pulmonary fibrosis and emphysema (CPFE) is an umbrella term encompassing upper lobe emphysema and lower lobe pulmonary fibrosis with pathogenesis elusive. The combination of pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome, encompassing a distinct radiologic, revealing both upper lobe emphysema and lower lobe fibrosis in high resolution computed tomography (HRCT) of the chest, as well as lung function profile, with apparently preserved lung volumes contrasting with disproportionally impaired gas exchange, as assessed by reduced diffusing lung capacity for carbon monoxide (DLco) [1,2,3] It is associated with severe exercise hypoxemia and increased prevalence of. A close linkage between pulmonary fibrosis and microscopic polyangiitis (MPA), a type of systemic necrotizing small vasculitis characterized by both pulmonary and renal involvement and associated with circulating antineutrophil cytoplasm antibodies (ANCAs) against myeloperoxidase (MPO), has been recently identified in both clinical [15] and experimental setting [16] The latter implies that an ongoing autoimmune process through recognition of self-antigens may take place in a subgroup of patients initially presented with a diagnosis of IPF. In line with this notion, a considerable number of patients seminally set under the diagnostic umbrella of idiopathic interstitial pneumonia (IIP), either non-specific (NSIP) or IPF meet the case definition of undifferentiated connective tissue disorder and may evolve through disease course into a specific connective tissue disorder with compatible clinical and serum immunologic profile [17,18]
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