Ray E. Stanford

Pulmonary manifestations of systemic lupus erythematosus: review of twelve cases of acute lupus pneumonitis.

Acute lupus pneumonitis was the presenting manifestation of systemic lupus erythematosus in six of 12 cases in this series. The clinical picture was characterized by severe dyspnea, tachypnea, fever and arterial hypoxemia. Radiographic findings included an acinar filling pattern which was invariably found in the lower lobes and was bilateral in 10 of the cases. Studies failed to reveal evidence of infection as a cause of the acute pulmonary infiltrates. All patients were treated with oxygen and corticosteroids; seven received azathioprine. Six patients survived and are clinically well 14 months to four years following their acute illness. Three of these patients have residual interstitial infiltrates with persistent pulmonary function test abnormalities indicating progression to chronic interstitial pneumonitis. Histologic sections of the lungs available from four patients revealed hyaline membranes and interstitial edema (four cases), acute alveolitis (two cases), arteriolar thrombosis (one case) and a prominent lymphocytic interstitial pneumonitis with organizing bronchiolitis (one case).

Circulating immune complexes in the idiopathic interstitial pneumonias.

We determined circulating immune complex levels and their correlation with pulmonary histopathology, immunofluorescence and steroid responsiveness in 24 patients with idiopathic interstitial pneumonias. Levels were elevated in all but three of 16 patients with cellular disease, but in none of eight with diffuse fibrosis (P less than 0.001). Granular deposition of IgG, usually with elevated levels, but in only 11 per cent of those with normal levels (P less than 0.001). The radiographic and physiologic response to corticosteroid therapy was better in patients with initially elevated levels than in those with normal levels (P less than 0.03). Circulating immune complexex are present in patients with cellular idiopathic interstitial pneumonias, have a pathogentic role in this disease and may identify a patient population that is potentially steroid responsive.

INTERSTITIAL LUNG DISEASE IN POLYMYOSITIS AND DERMATOMYOSITIS: ANALYSIS OF SIX CASES AND REVIEW OF THE LITERATURE

Interstitial pneumonitis may be the presenting manifestation of polymyositis-dermatomyositis, or may occur later in the evolution of the disease. The clinical picture is characterized by non-productive cough, dyspnea and hypoxemia. The chest radiograph demonstrates interstitial infiltrates with predilection for the lung bases, often with an alveolar pattern in addition. The histopathologic features are those of organizing and interstitial pneumonitis and pleuritis, with variable fibrosis. In the present series, the patients with mixed alveolar and interstitial infiltrates on chest radiograph and organizing pneumonia and bronchiolitis obliterans in addition to interstitial pneumonitis. In one patient evolution from pulmonary inflammation to interstitial fibrosis was demonstrated. The etiology of primary lung disease in PM-DM is not known, but cell-mediated autoimmunity to an unidentified component of lung tissue is suggested. Including the present series, 50 percent of patients have responded favorably to corticosteroids with decreased dyspnea, clearing of the chest radiograph and improved pulmonary function tests.

Interstitial Pneumonia in Angio-Immunoblastic Lymphadenopathy with Dysproteinemia: A Case Report with Special Histopathologic Studies

A patient presented with characteristic historical, physical, and laboratory findings of angio-immunoblastic lymphadenopathy with dysproteinemia. This newly described entity apparently represents a nonneoplastic proliferation of the B-lymphocyte system with immunoblastic transformation of many lymphocytes and excessive production of immunoglobulins. It is associated with fever, sweats, weight loss, skin rash, lymphadenopathy, splenomegaly, hepatomegaly, and characteristic histologic features of the involved lymph nodes. Noteworthy in the patient reported here are the extent and course of radiographically and clinically evident pulmonary involvement and the biopsy documentation of an interstitial pneumonia marked by histopathologic changes closely resembling those found in the lymph nodes, with immunohistologic demonstration of immunoglobulins in the alveolar walls.