A patient with amyopathic dermatomyositis associated with fatal rapidly progressive interstitial pneumonitis resistant to therapy is described. Pathologic examination of a transbronchial lung biopsy specimen showed diffuse alveolar damage and nonspecific interstitial pneumonia-organizing pneumonia-like findings. Bronchoalveolar lavage fluid contained many CD8+ lymphocytes, considered to be cytotoxic T cells. Analysis of bronchoalveolar lavage fluid in this case may provide prognostically and pathogenetically important information.
To clarify the mechanism of brain and spinal cord impairment in amyotrophic lateral sclerosis (ALS), we measured the cerebrospinal fluid (CSF) levels of substance P (SP) in 11 patients with sporadic ALS. Findings were compared with those obtained in controls and diseased controls. The CSF SP levels of patients with ALS, and particularly in patients with a disease duration of less than 2.5 years, were significantly higher than those in controls. These findings strongly suggested that SP may play an important role in the pathophysiology of ALS.
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