SUMMARYWe describe a 33-yr-old man suffering from severe vertebral osteoporosis and urticaria pigmentosa due to systemic mast cell disease (SMCD). Because i.v. clodronate therapy could not prevent further vertebral fractures, an additional treatment with interferon alpha-2b was initiated. During 24 months of treatment, our patient had no further pain episodes, no new vertebral fractures were discovered, trabecular bone mineral density (BMD) increased significantly and urticarial symptoms improved. Nevertheless, the extent of skin lesions remained unchanged. On histological examination, a remarkable decrease of mast cells was observed in the bone marrow, but not in the skin. Five months after discontinuation of interferon alpha-2b, trabecular BMD decreased and urticarial symptoms deteriorated. These findings illustrate a beneficial effect of interferon alpha-2b on SMCD-induced osteoporosis as well as urticarial symptoms, and raise the question whether this treatment may have a diverse impact on mast cell populations in different tissues.

Classically, the middle lobe syndrome has been described as being caused by a central obstruction. Clinical records and radiologic findings were reviewed in 129 patients examined between 1955-1981 who had chronic disease in the right middle lobe and/or lingula. Fifty-eight patients (45%) had no evidence of a central obstructive lesion. The majority were middle-aged women with histories of chronic cough and chest pain. Bronchoscopic and radiologic evaluation served to exclude central lesions. Surgical confirmation was available in 38 patients. Pathologic study showed varying degrees of chronic inflammation, pneumonia, and bronchiectasis. Surgical results were excellent in isolated disease. Chronic atelectasis and pneumonitis of the right middle lobe and/or lingula do not always imply central obstruction. A lack of collateral ventilation is a plausible theory to explain the pathophysiology in such patients.

No abstract

The most common causes of pulmonary edema are cardiac failure, renal failure and injury edema (diffuse alveolar damage). The injury edema typically shows airspace consolidation due to exsudation of fluid in the periphery of the lung with air bronchograms, no interstitial fluid accumulation can be found and only rarely pleural effusions are present. Cardiac and renal edemas often show a mixed interstitial and alveolar transudation without air bronchograms. Pleural effusions are often present. Both usually have an increased heart-size and an increased vascular pedicle width. To distinguish them better one has to look at the distribution of the pulmonary edema: The cardiac edema typically shows a gravitational and the renal edema a central distribution.

No abstract