John D. Chovan, James R. Cacchillo
Lung Diseases, Treatments, Misdiagnosis
Medical Pathology | Medicine and Health Sciences | Nursing | Respiratory Tract Diseases
Pulmonary fibrosis can be described as an excessive amounts of fibrous connective lung tissue in the lung. Idiopathic pulmonary fibrosis(IPF) is the most common form of interstitial lung disease(ILD). When an obvious cause cannot be determined, a diagnosis of Idiopathic pulmonary fibrosis is made. The mean survival for patients is approximately 3-5 years from time of diagnosis. IPF is found primarily in men over 50 years of age, with a history of cigarette smoking with concurrent environmental and occupational inhalation exposures(Leslie, 2011, p. 592). Pulmonary fibrosis has been identified by early pathologists at the beginning of the last century. During autopsy, the signature “honeycombing” of a shrunken, cystic and fibrotic lungs were first identified. IPF was first thought to an inflammatory disorder which was treated with corticosteroids and cytotoxic medications. New understanding of IPF and the pathophysiology have shaped new treatments. “Current concepts suggest that the disease results from aberrant reparative response to alveolar epithelial cell injury characterized by migratory proliferation, activation of fibroblasts and secretion of excessive amounts of extracellular matrix components, leading to scarring of the lungs, architectural distortion, and irreversible loss of function” (Tzouvelekis, Bonella & Spagnolo, 2015, p.359). Working on a vascular thoracic floor, many patients are admitted with a diagnosis of IPF.
Metz, William, "Idiopathic Pulmonary Fibrosis: Understanding Has Led to Exciting Treatments" (2015). Master of Science in Nursing (MSN) Student Scholarship. Paper 124.