Abstract
Idiopathic pulmonary fibrosis (IPF) is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000) than in women (13.2/100,000). The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock). IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP). The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis), forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational) exposures. IPF is typically progressive and leads to significant disability. The median survival is 2 to 5 years from the time of diagnosis. Medical therapy is ineffective in the treatment of IPF. New molecular therapeutic targets have been identified and several clinical trials are investigating the efficacy of novel medication. Meanwhile, pulmonary transplantation remains a viable option for patients with IPF. It is expected that, during the next decade, considerable progress will be made toward the understanding and treatment of this devastating illness.
Highlights
Synonym: cryptogenic fibrosing alveolitis (CFA) was the preferred term in Europe until terminology was simplified by consensus conference [1]
Idiopathic pulmonary fibrosis (IPF) belongs to a family of lung disorders known as the interstitial lung diseases (ILD) or, more accurately, the diffuse parenchymal lung diseases (DPLD)
Within this broad category of diffuse lung diseases, IPF belongs to the subgroup known as idiopathic interstitial pneumonia (IIP)
Summary
Radiographic findings The chest roentograph is abnormal in most patients with IPF (Figure 1). Exclusion of other known causes for interstitial lung disease (such as drug toxicity, environmental exposure and connective tissue disease) http://www.ojrd.com/content/3/1/8 the utility of this modality has been consistently demonstrated in clinical trials, HRCT has become a more important tool in diagnostic algorithms. Non-specific interstitial pneumonia (NSIP) will always remain in the differential and, in some cases, can only be excluded by biopsy Connective tissue diseases such as systemic sclerosis, polymyositis or rheumatoid arthritis can mimic IPF, both clinically and radiographically. When radiographic fibrosis and histopathologic fibrosis were assigned scores, they were found to be equivalent with respect to predicting death or clinical worsening Another interesting study examined the relationship between the "confident" IPF pattern and survival. A small prospective, but uncontrolled, study following 18 IPF patients receiving NAC demonstrated restoration of glutathione levels and improvement of lung function measures [94]. It is likely that considerable progress will be made toward understanding and treating this devastating illness
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