Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF), the most common form of idiopathic interstitial pneumonia, causing progressive pulmonary fibrosis. The symptoms develops insidiously over months and years and includes exertional dyspnea, cough and crepitation (Sklerosiphonie). The diagnosis is, if necessary, provided by renal biopsy based on history, physical examination, HRCT and / or. Treatment may include anti-fibrotic medication and oxygen therapy. The condition of most patients is deteriorating; the median survival rate from diagnosis for about 3 years.

IPF, histologically usually identified as interstitial pneumonia, accounts for most cases of idiopathic interstitial pneumonia. IPF affects men and women> 50 at a ratio of 2: 1, with a significantly increased incidence with each passing decade. Current or former cigarette smoking is most strongly associated with the disease. There is a slight genetic predisposition; in up to 20% of cases familial clustering occur.

Idiopathic pulmonary fibrosis (IPF), the most common form of idiopathic interstitial pneumonia, causing progressive pulmonary fibrosis. The symptoms develops insidiously over months and years and includes exertional dyspnea, cough and crepitation (Sklerosiphonie). The diagnosis is, if necessary, provided by renal biopsy based on history, physical examination, HRCT and / or. Treatment may include anti-fibrotic medication and oxygen therapy. The condition of most patients is deteriorating; the median survival rate from diagnosis for about 3 years. IPF, histologically usually identified as interstitial pneumonia, accounts for most cases of idiopathic interstitial pneumonia. IPF affects men and women> 50 at a ratio of 2: 1, with a significantly increased incidence with each passing decade. Current or former cigarette smoking is most strongly associated with the disease. There is a slight genetic predisposition; in up to 20% of cases familial clustering occur. Etiology A combination of environmental, genetic and other unknown factors is believed to contribute to a dysfunction or reprogramming of alveolar epithelial cells, resulting in an abnormal fibroproliferation in the lungs. The current research focuses on genetic influences, influences of environmental stimuli, inflammatory cells of the alveolar epithelium, mesenchyme and the matrix. Pathology The main histological changes are a pleuranahe fibrosis with nests of proliferating fibroblasts (Fibroblastenfoci) and dense scar tissue, which alternates with areas normal lung tissue (heterogeneity). It occurs diffusely distributed interstitial inflammatory reaction with infiltration of lymphocytes, plasma cells and histiocytes. Cystic anomaly (honeycomb lung) is observed in all the patients and increases with progression of the disease. A similar histological pattern rarely occurs in interstitial lung disease of known etiology (see Table: Main characteristics of idiopathic interstitial pneumonia). Symptoms and signs The symptoms typically develops over a period of 6 months to several years ago and consists of exertional dyspnea and nonproductive cough. General symptoms such as mild fever and myalgias are rare. The classic auscultation a IPF is fine, dry, inspiratory crepitation (Sklerosiphonie) on both lung sections. Clubbing of the cases occur in approximately 50%. The remainder of the physical examination is unremarkable until the disease progresses. At this time, symptoms of pulmonary hypertension and right ventricular systolic dysfunction can be added. Diagnosis High-resolution CT (HRCT) Sometimes surgical lung biopsy, the diagnosis is made nonproductive cough and Sklerosiphonie in breast examination in patients with subacute dyspnea. A IPF but initially often overlooked because of the clinical similarity with other frequently occurring diseases such as bronchitis, asthma and heart failure. Diagnosis requires HRCT and in some cases surgical lung biopsy. The chest x-ray image typically shows a diffuse reticular pattern on the lower and peripheral lung zones. Other findings include small cystic lesions (honeycomb lung) and advanced airways by Traktionsbronchiektasen. In HRCT is a diffuse, patchy, pleuranahe reticular pattern with irregularly thickened Interlobularsepten and lines and pleuranahe honeycombed changes and Traktionsbronchiektasen show. Frosted glass opacities of> 30% of lung suggest another diagnosis. Idiopathic pulmonary fibrosis Image courtesy of Harold R. Collard, M.D. var model = {thumbnailUrl: ‘/-/media/manual/professional/images/idiopathic_pulmonary_fibrosis_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/-/media/manual/professional/images/idiopathic_pulmonary_fibrosis_high_de.jpg?la = en & thn = 0 ‘, title:’ Idiopathic pulmonary fibrosis ‘, description:’ u003Ca id = “v37893178 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “”para “” u003e u003cp u003eSubpleurale honeycombing and Traktionsbronchiektasen can be seen on this high-resolution CT scan of a patient with idiopathic pulmonary fibrosis (IPF). There are no findings such as nodules

Health Life Media Team

Leave a Reply