Idiopathic Pulmonary Fibrosis (IPF) PDF

DONE BY : A.K.N Idiopathic pulmonary fibrosis (IPF) is defined as a progressive fibrosing interstitial pneumonia of unknown cause, occurring in adults and associated with the histological or radiological pattern of UIP (Usual Interstitial Pneumonia)  Age >50 yrs. Gender: More common in males....

DONE BY : A.K.N Idiopathic pulmonary fibrosis (IPF) is defined as a progressive fibrosing interstitial pneumonia of unknown cause, occurring in adults and associated with the histological or radiological pattern of UIP (Usual Interstitial Pneumonia)  Age >50 yrs. Gender: More common in males. more in smokers. Familial cases are rare. ❑ Symptoms : Progressive dyspnea. Dry cough. Constitutional symptoms are unusual. ❑ Signs: Decrease movement. Inspection finger clubbing. Shifted to trachea. Palpation Dull in affected area. Percussion Bronchial breathing. Auscultation Increase vocal resonance. Fine bilatral basal late inspiratory crepitation. Laboratory : ❖ CBC : Lymphopenia in sarcoiodosis , Eosinophilia in pulmonary eosinophilias and drug reactions , neutrophilia in hypersensitvity pneumonitis. ❖ Lactate dehydrogenase (LDH) : maybe elevated in active alveolitis. ❖ ESR & CRP : non-specifically raised ❖ Anti-cyclic citrullinated peptide (Anti-CCP) : may suggest Connective tissue disease.(uncommon) ❖ ABG : Hypoxaemia , but as IPF advances arterial hypoxaemia & hypocapnia are present at rest. Con… Investigation Imaging Studies : CXR show : Bilateral basal reticulonodular markings ,. NB: CXR maybe normal in early stage of the disease. High-resolution CT : is the best imaging modality , may show ground glass change , Honeycomb cysts and traction bronchiectasis depending on stage of disease  PFTs : Restrictive pattern.  Bronchoalveolar lavage (BAL) : Neutrophilia ± Eosinophil's.  Lung biopsy. ❑ The management options for IPF are improving. If the vital capacity is between 50% and 80% predicted , patients may be offered either : ❑ pirfenidone (an anti fibrotic agent) ❑ nintedanib (a tyrosine kinase inhibitor) ❑ Both of these agents have been shown to reduce the rate of decline in lung function. ❑ The optimum treatment for acute exacerbations is unknown. ❑ Treatment is largely supportive. ❑ Oxygen should be considered for palliation of breathlessness. ❑ Broad-spectrum antibiotics maybe combined with corticosteroids (prednisolone 40-60 mg/day for 6-8 weeks) and sometimes additional immunosuppression but there are few data to support this approach. ❑ lung transplantation should be considered in sever case. In advance case the median survival of 3 years is widely quoted ; the rate of disease progression varies considerably, However , from death within a few months to survival with minimal symptoms for many years.