Idiopathic Pulmonary Fibrosis—an Epidemiological and Pathological Review

Abstract

Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease (ILD) affecting the pulmonary interstitium. Other forms of interstitial lung disease exist, and in some cases, an environmental etiology can be delineated. The diagnosis of IPF is typically established by high-resolution CT scan. IPF tends to have a worse prognosis than other forms of ILD. Familial cases of IPF also exist, suggesting a genetic predisposition; telomerase mutations have been observed to occur in familial IPF, which may also explain the increase in IPF with advancing age. Alveolar epithelial cells are believed to be the primary target of environmental agents that have been putatively associated with IPF. These agents may include toxins, viruses, or the autoantibodies found in collagen vascular diseases. The mechanism of disease is still unclear in IPF, but aberrations in fibroblast differentiation, activation, and proliferation may play a role. Epithelial-mesenchymal transition may also be an important factor in the pathogenesis, as it may lead to accumulation of fibroblasts in the lung and a disruption of normal tissue structure. Abnormalities in other components of the immune system, including T cells, B cells, and dendritic cells, as well as the development of ectopic lymphoid tissue, have also been observed to occur in IPF and may play a role in the stimulation of fibrosis that is a hallmark of the disease. It is becoming increasingly clear that the pathogenesis of IPF is indeed a complex and convoluted process that involves numerous cell types and humoral factors.

C-X-C Motif Chemokine 13 (CXCL13) Is a Prognostic Biomarker of Idiopathic Pulmonary Fibrosis

“…Glucocorticoids do not favorably alter the natural history of IPF (1). However, these nonspecific agents are similarly ineffectual as primary (or sole) therapy of numerous (if not all) other antibody-mediated lung diseases, including granulomatosis with polyangitis (Wegener's) (or antineutrophil cytoplasmic antibody-associated pulmonary vasculitides); Goodpasture syndrome; interstitial lung diseases (especially acute exacerbations) associated with polymyositis, scleroderma, and other conventional connective tissue diseases; or donor-specific antibodies in lung transplant recipients with chronic allograft rejection.…”

Simvastatin Attenuates TGF-�1-Induced Epithelial-Mesenchymal Transition in Human Alveolar Epithelial Cells

“…Thus, we succeeded in establishing an EMT model in AECII to study the therapeutic effect of Sim on this pathological process. The incidence of IPF increases with advancing age, peaking in patients ≥ 75 years old, which indicates that IPF is a geriatric disease [40]. It is generally known that aging causes changes in drug pharmacokinetics, which are dependent on body composition, albumin concentration, liver metabolism and drug elimination, all of which may change with age.…”

A Novel Genomic Signature with Translational Significance for Human Idiopathic Pulmonary Fibrosis

“…UIP features alternations of normal lung structures and regions of dense fibrosis, whereas signs of inflammation are usually mild. The presence of infiltrating lymphocytes and macrophages has been reported (4). The disease etiology remains unknown, but it is thought to involve chronic epithelial lung injury, myofibroblast formation, and excessive deposition of extracellular matrix in the lung parenchyma (5)(6)(7)(8)(9)(10).…”