Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible lung disease characterized by chronic inflammation and fibroproliferation of lung parenchymal cells, leading to chronic respiratory failure and death. It is more common in men and affects over 100,000 people in the U.S. Most cases are sporadic, though 15-20% have a family history linked to autosomal dominance. IPF has limited treatment options and a poor prognosis, with a median survival of 2-3 years after diagnosis. Research is ongoing to improve treatment and quality of life. The pathophysiology of IPF is complex, involving autoimmune mechanisms, epithelial injury, and abnormal healing of the alveolar-capillary barrier. Evidence suggests IPF is an autoimmune disease, with B-cell aggregates, activated T lymphocytes, and immune activation in patients. CD4 T cells play a role in producing cytokines and fibrogenic mediators. IPF progresses through a subclinical period with radiographic findings, followed by a symptomatic phase. The disease course can be rapid, slow, or mixed, with periods of stability and acute decline. Diagnosis involves blood tests, CT scans, bronchoalveolar lavage, and lung biopsy, which may show "honeycombing" and usual interstitial pneumonia (UIP) pattern. Symptoms include worsening dyspnea, dry cough, chest pain, fatigue, and weight loss. Objective findings include crackles, clubbing, and right ventricular lift. Diagnosis is often a process of elimination, with biomarkers like MMP-1, KL-6, and surfactant protein A aiding in staging. Nursing care focuses on supporting patients, managing symptoms, and improving quality of life. Interventions include oxygen therapy, pulmonary rehabilitation, and palliative care. IPF can lead to pulmonary hypertension, right ventricular hypertrophy, and gastroesophageal reflux disease. Treatment options include steroids, immunosuppressants, and N-acetylcysteine. Despite advances in understanding IPF, effective therapy remains elusive. Palliative care is crucial for managing symptoms and improving patient outcomes. Research continues to explore new treatments and better management strategies for this debilitating disease.Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible lung disease characterized by chronic inflammation and fibroproliferation of lung parenchymal cells, leading to chronic respiratory failure and death. It is more common in men and affects over 100,000 people in the U.S. Most cases are sporadic, though 15-20% have a family history linked to autosomal dominance. IPF has limited treatment options and a poor prognosis, with a median survival of 2-3 years after diagnosis. Research is ongoing to improve treatment and quality of life. The pathophysiology of IPF is complex, involving autoimmune mechanisms, epithelial injury, and abnormal healing of the alveolar-capillary barrier. Evidence suggests IPF is an autoimmune disease, with B-cell aggregates, activated T lymphocytes, and immune activation in patients. CD4 T cells play a role in producing cytokines and fibrogenic mediators. IPF progresses through a subclinical period with radiographic findings, followed by a symptomatic phase. The disease course can be rapid, slow, or mixed, with periods of stability and acute decline. Diagnosis involves blood tests, CT scans, bronchoalveolar lavage, and lung biopsy, which may show "honeycombing" and usual interstitial pneumonia (UIP) pattern. Symptoms include worsening dyspnea, dry cough, chest pain, fatigue, and weight loss. Objective findings include crackles, clubbing, and right ventricular lift. Diagnosis is often a process of elimination, with biomarkers like MMP-1, KL-6, and surfactant protein A aiding in staging. Nursing care focuses on supporting patients, managing symptoms, and improving quality of life. Interventions include oxygen therapy, pulmonary rehabilitation, and palliative care. IPF can lead to pulmonary hypertension, right ventricular hypertrophy, and gastroesophageal reflux disease. Treatment options include steroids, immunosuppressants, and N-acetylcysteine. Despite advances in understanding IPF, effective therapy remains elusive. Palliative care is crucial for managing symptoms and improving patient outcomes. Research continues to explore new treatments and better management strategies for this debilitating disease.