IPF is also called Cryptogenic fibrosing alveolitis. It is a pulmonary disorder of unknown aetiology characterized by diffuse interstitial pulmonary fibrosis.
Males are affected more than are females and 2/3rd of the patients are older than 60 years of age at the time of presentation
Diagnosis and Differential Diagnosis
The histological pattern of fibrosis is referred to usual interstitial pneumonia (UIP) which is required for the diagnosis of IPF.
Two other conditions also cause UIP, these are asbestosis and collagen vascular disease. Therefore these conditions must be ruled out before using the word "idiopathic".
Pathogenesis:
The exact cause of the fibrosis is unknown that's why it is called idiopathic. Morphologically, the pleural surfaces of the lung have the appearance of cobblestones because of retraction of scars along the interlobular septa. The cut surface shows fibrosis. The lower lobe is predominantly involved. The pattern of fibrosis is called usual interstitial pneumonia. Histologically it is identified by the presence of patchy interstitial fibrosis which varies in intensity with time. The dense fibrosis causes a collapse of the alveolar walls and formation of cystic spaces lined by type II pneumocytes or bronchial epithelium, this is referred to as honeycomb fibrosis.
The inflammatory cells include mostly lymphocytes and occasional plasma cells, mast cells and eosinophils. Intimal fibrosis and medial thickening of the pulmonary arteries are often present.
Clinical Signs and Symptoms:
1. Gradual onset of non-productive cough.
2. Progressive dyspnea (shortness of breath)
3. Dry "velcro" like crackles upon auscultation
4. Cyanosis
5. Hypoxia
6. Cor pulmonale
7. Peripheral oedema.
Treatment:
Lung transplantation is the only definitive therapy
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