Pulmonary manifestations of vinyl and polyvinyl chloride (interstitial lung disease). Newer aspects.

Newer varieties of occupational lung diseases primarily due to the vast increase in industrial technology have been reported recently. Preeminent among such newer agents are vinyl chloride (VC) and polyvinyl chloride. Very few cases have been reported, in Europe only, with descriptive histopathologic changes. To our knowledge, no pathologic studies of VC exposure have been described in the American literature. The biopsy abnormalities in our patients disclosed desquamation of alveolar macrophages into the alveolar lumina and minor interstitial and alveolar inflammatory changes. Pulmonary function abnormalities included restrictive insufficiency. Preventive therapy consists of the avoidance of further exposures, frequent industrial hygiene monitoring, and total avoidance of tobacco smoke, as well as associated atmospheric pollutants. Thus far, none of these patients has exhibited evidence of pulmonary neoplasms. All three patients survived their occupational injuries, and two are still disabled to varying degrees. Urine and blood levels of phthalic acid derivatives were elevated in two patients, the exact significance of which is not fully known. It probably represents a toxicologic response, but must be further pursued before conclusions can be reached.

Pulmonary amyloidoma and hilar adenopathy. Rare manifestations of primary amyloidosis.

Three cases of primary intrathoracic amyloidosis are reported. Two of these three patients initially had localized parenchymal pulmonary amyloid tumors (or pulmonary amyloidomas). The third patient developed bilateral enlargement of the hilar lymph nodes as a manifestation of primary amyloidosis. These rare manifestations of primary amyloidosis are illustrated, and their clinical signifiance is discussed. Amyloidoma should be considered in patients with unexplained pulmonary nodules or enlargement of hilar lymph nodes.

Desquamative interstitial pneumonitis. Cellular phase of fibrosing alveolitis.

Since the original description of desquamative interstitial pneumonitis in 1967, several investigators have questioned the specificity of the disease. The concept of desquamative interstitial pneumonitis and usual fibrosing interstitial pneumonitis as two manifestations of a nonspecific disease spectrum has been proposed. Twenty-six patients with desquamative interstitial pneumonitis were evaluated with respect to clinicopathologic correlates. Biopsies were reviewed without knowledge of the patients' clinical course. The absolute histopathologic criteria for the diagnosis were (1) intra-alveolar accumulations of free alveolar cells with PAS-positive diastaseresistant cytoplasmic granules; (2) mononuclear cell interstitial inflammation; and (3) absence of necrosis, hyaline membranes, intra-alveolar fibrosis, asbestos bodies, and birefringent crystalline dust material. Transmission electron microscopic studies revealed the dominant alveolar lining cell to be the granular pneumocyte, with a prominent population of free alveolar macrophages. Patients were segregated into group 1 (cellular phase) and 2 (cicatrized phase). Although favorable short-term responses to corticosteroid therapy were observed in both groups, long-term responses were variable. Apparent complete remissions were noted in both groups.

Recurrent thrombophlebitis and pulmonary emboli in congenital factor 5 deficiency.

A case of congenital coagulation factor 5 deficiency is reported. Despite this defect in blood coagulation, this patient had frequent episodes of thrombophlebitis which were associated with pulmonary emboli on two occasions. Heparinization caused no hemorrhagic complications.