Diagnosis of Pulmonary fibrosis
Pulmonary fibrosis Diagnosis: Book Excerpts
Diagnostic Tests for Pulmonary fibrosis: Online Medical Books
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Idiopathic pulmonary fibrosis:
Diagnosis
(Professional Guide to Diseases (Eighth Edition))
Diagnosis begins with a thorough patient history to exclude a more common cause of interstitial lung disease.
CONFIRMING DIAGNOSIS Lung biopsy is helpful in the diagnosis of IPF. In the past, an open lung biopsy was the only acceptable procedure, but now biopsies may be done through a thoracoscope or bronchoscope.
Histologic features of the biopsy tissue vary, depending on the stage of the disease and other factors that aren’t yet completely understood. The alveolar walls are swollen with chronic inflammatory cellular infiltrate composed of mononuclear cells and polymorphonuclear leukocytes. Intra-alveolar inflammatory cells may be found in early stages. As the disease progresses, excessive collagen and fibroblasts fill the interstitium. In advanced stages, alveolar walls are destroyed and are replaced by honeycombing cysts.
Chest X-rays may show one of four distinct patterns: interstitial, reticulonodular, ground-glass, or honeycomb. Although chest X-rays are helpful in identifying the presence of an abnormality, they don’t correlate well with histologic findings or pulmonary function tests (PFTs) in determining the severity of the disease. They also don’t help distinguish inflammation from fibrosis. However, serial X-rays may help track the progression of the disease.
High-resolution computed tomography scans provide superior views of the four patterns seen on routine X-ray film and are used routinely to help establish the diagnosis of IPF. Research is currently under way to determine whether the four patterns of abnormality seen on these scans correlate with responsiveness to treatment.
PFTs show reductions in vital capacity and total lung capacity and impaired diffusing capacity for carbon monoxide. Arterial blood gas (ABG) analysis and pulse oximetry reveal hypoxemia, which may be mild when the patient is at rest early in the disease but may become severe later in the disease. Oxygenation will always deteriorate, usually to a severe level, with exertion. Serial PFTs (especially carbon monoxide diffusing capacity) and ABG values may help track the course of the disease and the patient’s response to treatment.
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Source: Professional Guide to Diseases (Eighth Edition), 2005
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