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Pulmonary involvement in mixed connective tissue disease (MCTD) is common, frequently severe, and is often clinically inapparent and variably responsive to corticosteroid/cyclophosphamide treatment. Serial pulmonary evaluation of patients with MCTD is important, since deterioration, as in the diffusing capacity over time, may alert the physician to the need for more invasive evaluation. Patients with a greater degree of overlap in rheumatological symptoms with an element of systemic sclerosis (PSS) may later develop severe disease. Nailfold capillary microscopy also may help in determining which patients will develop severe pulmonary involvement. Significant pulmonary hypertension occurs and cannot be accurately predicted on the basis of history, physical examination, pulmonary function tests, gallium scanning, or exercise testing. The characteristic pathological finding was intimal proliferation with medial muscular hypertrophy in the pulmonary arterioles. In contrast, pulmonary interstitial abnormalities were minimal, suggesting the proliferative vascular lesions are more closely associated with pulmonary hypertension in MCTD. Some patients develop rapidly progressive disease with varying response to corticosteroid and cytotoxic agents. More commonly, however, MCTD patients with long-term disabling disease, including pulmonary dysfunction, have had significant improvement with steroid and/or cyclophosphamide treatment, and clinical remission has occurred in 38% of the patients in this series.

The authors report a case of leiomyosarcoma in the mediastinum presenting with superior vena cava obstruction, and also demonstrate the aggressive nature of this tumor in spite of radiotherapy. Where technically feasible, surgery appears to be the treatment of choice. The pathologic and electron microscopic features of this tumor are also described.

We report a case of pulmonary venoocclusive disease in which chest radiography, when supplemented with computed tomography after contrast medium administration, was specific enough to make the diagnosis without resorting to more invasive, potentially dangerous procedures. A brief review of the literature, with emphasis on etiology, pathogenesis, and possible therapeutic approaches, emphasizes the importance of early diagnosis of this rare and usually fatal disease.

A 33-year-old woman was seen with fever, confusion, and respiratory distress. A diagnosis of thrombotic thrombocytopenic purpura (TTP) was made when renal failure, microangiopathic hemolytic anemia, and thrombocytopenia subsequently developed. An open kidney biopsy confirmed the clinical diagnosis. The patient initially was seen with the roentgenographic and clinical appearance of pulmonary hemorrhage, which was confirmed histologically and, to our knowledge, previously has not been associated with TTP.