Sarcoid myopathy presenting with diaphragm weakness.

Sarcoidosis involving muscle occurs frequently, but it is infrequently symptomatic. The clinical, electromyographic, and histologic features of sarcoidosis involving muscle in a 63-year-old woman presenting with diaphragm weakness are described. An electromyogram revealed wide-spread myotonia and an inflammatory myopathic process, suggestive of adult-onset acid maltase deficiency disease. Muscle biopsy showed noncaseating granulomas consistent with sarcoidosis. Clinical improvement followed the initiation of oral prednisone therapy. This case illustrates that muscular sarcoidosis may mimic adult-onset acid maltase deficiency in both its clinical and electromyographic features.

Serum angiotensin-converting enzyme is elevated in association with underground coal mining.

Serum angiotensin-converting enzyme activity (SACE) and lysozyme activity were measured in a group of 40 underground coal miners and two control groups, 20 subjects with sarcoidosis and 15 normal non-dust-exposed volunteers. The miners were grouped first according to whether they had recent exposure (still actively mining or retired three years or less prior to measurement) or temporally more distant exposure (retired more than three years prior to measurement). Secondly, they were grouped as to whether or not they had coal workers' pneumoconiosis (CWP). The subjects with sarcoidosis were grouped according to disease activity. As expected, the subjects with active sarcoidosis had elevated SACE activity compared with normal subjects. The coal miners as a group did not have elevation of their SACE activity. However, the coal miners with recent exposure had elevated SACE activity (57.1 +/- 3.9 U/ml) compared with normal controls (43.8 +/- 1.5 U/ml, p = 0.007). The SACE activity in miners without recent exposure was not elevated (39.8 +/- 1.3 U/ml) compared with the normal controls. No increase in SACE activity was found when the miners were grouped according to the presence or absence of CWP. In contrast, the miners' serum lysozyme activity was not elevated. Since alveolar macrophages are a potential source of SACE, elevation of SACE activity in underground coal miners may reflect alveolar macrophage activation caused by increased pulmonary mixed coal mine dust burden. Furthermore, since both SACE and serum lysozyme are elevated in association with silicosis, these findings may confirm that the macrophage responses to inhaled silica and coal dust differ.

Transbronchial biopsy of pulmonary alveolar microlithiasis.

A middle-aged woman was initially seen with mild breathlessness and a fine reticulonodular pattern noted on chest roentgenogram. Pulmonary alveolar microlithiasis was demonstrated by transbronchial biopsy. Although microlithiasis calcispherites have been described as appearing in bronchial walls, at necropsy, and in lavage fluid, we have found only one previous report of a case diagnosed by transbronchial biopsy.

Nodular sclerosing Hodgkin's disease presenting as nephrotic syndrome.

A case of nephrotic syndrome due to minimal change renal disease was associated with minimally symptomatic nodular sclerosing Hodgkin's disease in a 15-year-old girl. Although minimal renal disease is the most frequently described biopsy finding in this paraneoplastic syndrome, the combination of nodular sclerosing Hodgkin's disease and minimal renal disease is uncommon. Four cases of this association have been reported in the literature. This case makes five.