Environmental and Occupational Lung Disease: Interstitial Lung Disease

Interstitial Lung Disease

These diverse disorders - more than 100 variations exist - are characterized by their acute transformation of the pulmonary interstitium (the framework of the lung substance). In patients with such disorders, polymorphonuclear leukocytes, plasma cells, lymphocytes and eosinophils abnormally infiltrate and accumulate in the interstitium, which houses the cells that control gas exchange. These diverse diseases have in common the feature of transforming the delicate elastic framework of the lung to a stiff, rigid, scarred structure through which gas exchange becomes progressively impaired. Patients with more advanced disease often have scarring and thickening of deep lung tissues attributed to extensive fibrosis.

A multidisciplinary team involving thoracic surgeons, radiation specialists, pathologists and pulmonologists of the Allegheny Center for Lung and Thoracic Disease develops treatment plans tailored to each patient's disease pattern. Diagnosis may involve all of the following: intensive patient history and evaluation, X-rays, pulmonary function tests and surgical lung biopsy. In most cases, minimally invasive thoracoscopic techniques may be applied to obtain the surgical biopsies needed to characterize the disease. This can reduce the risk of biopsy in these patients with significant lung dysfunction. The center's surgeons are among the most experienced in North America with these minimally invasive approaches.

Treatment for idiopathic disease usually involves a regimen of corticosteroid and/or anti-inflammatory agents to reduce inflammation and suppress the overactive immune system of the lungs leading to the inflammation. The center's researchers aim to enhance these existing approaches by closely examining the cellular mechanisms involved in interstitial lung disease's inflammatory response.

At the Allegheny Center for Lung and Thoracic Disease, the physician team wants to provide a streamlined, but rational approach to the evaluation of patients with unexplained respiratory complaints and radiographic abnormalities suggestive of interstitial lung disease.

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