ABSTRACT
Idiopathic pulmonary fibrosis (IPF), also called interstitial pneumonia or fibrosing alveolitis, is a progressive interstitial lung disease of unknown origin. Two distinct forms are known which differ in course, morphologic features and cytological findings in bronchoalveolar lavage: a cellular "desquamative interstitial pneumonia" and a hypocellular and more fibrotic "usual interstitial pneumonia". The two types appear to represent different stages of the same disease. The clinical findings in IPF are dyspnoea, unproductive cough, clubbing and rales. Pulmonary function tests reveal restriction, reduced diffusion capacity and hypoxaemia during exercise. Chest X-rays show localized linear or nodular densities usually accentuated at the lung bases; in 10% of patients with IPF, chest radiography is normal. To assess the inflammatory process bronchoalveolar lavage and transbronchial or open lung biopsy have proven necessary. Before beginning treatment the activity of the inflammatory process and degree of lung function impairment should be established. Therapy of IPF is unspecific and its aim is to suppress alveolitis and the fibrotic process. Corticosteroids are the initial therapy. If there is no improvement after two months, immunosuppressive agents are added. Regular clinical and functional follow-up is required. Course and prognosis vary in different forms of IPF. Without treatment, median survival in the cellular form is over 10 years and in the hypocellular form between 3.2 and 5.6 years. Only 12-30% of patients respond to antiinflammatory treatment.
Subject(s)
Pulmonary Fibrosis/physiopathology , Adrenal Cortex Hormones/therapeutic use , Diagnosis, Differential , Drug Therapy, Combination , Humans , Immunosuppressive Agents/therapeutic use , Lung Diseases/diagnosis , Pulmonary Fibrosis/diagnosis , Pulmonary Fibrosis/drug therapy , Respiratory Function TestsABSTRACT
Serum ANP levels were measured by radioreceptor assay in 40 patients with various forms of secondary hypertension and 6 patients with heart failure. In addition, serum ANP was determined in 4 patients with renal artery stenosis before and after dilatation, as well as in 5 anephric patients before and after haemodialysis. Our results showed elevated serum ANP level in most patients with various forms of secondary hypertension and chronic heart failure. A distinction between these two groups and a control group of healthy individuals was not possible due to the wide range and occasional normal levels in the first two groups. ANP levels in patients with renal stenosis decreased after dilatation but there was no correlation with the success of this procedure. A positive correlation between ANP and plasma renin level was detectable in patients with renal artery stenosis, but was also elevated in anephric patients with absent renin production. In summary, our results show that measurements of serum-ANP are of little significance in the diagnosis of hypertension and chronic cardiac failure.
