Am. J. Respir. Crit. Care Med., Vol 151, No. 6, 06 1995, 2037-2040.
Pulmonary capillaritis and diffuse alveolar hemorrhage. A primary manifestation of polymyositis
MI Schwarz, JM Sutarik, JA Nick, JA Leff, JW Emlen and RM Tuder
Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Health Sciences Center, Denver 80262, USA.
Polymyositis is often complicated by either usual interstitial pneumonitis,
diffuse alveolar damage, cellular interstitial pneumonitis, or
bronchiolitis obliterans-organizing pneumonia. Pulmonary capillaritis, a
distinct interstitial reaction associated with diffuse alveolar hemorrhage,
occurs with the systemic vasculitides and some collagen vascular diseases.
It has not been described in patients with polymyositis. We describe two
patients who developed a relatively acute onset of polymyositis based on
the appearance of a severe proximal myopathy, elevated serum creatine
phosphokinase levels, compatible electromyographic abnormalities,
inflammatory muscle biopsies, and in one case, the presence of serum
anti-Jo-1 antibodies. Concomitant with their muscle disease, they developed
respiratory failure that proved to be pulmonary capillaritis with varying
degrees of diffuse alveolar hemorrhage as well as bronchiolitis obliterans-
organizing pneumonia. Although those reactions are reported to occur with
other collagen vascular diseases, these two cases are the first reports of
pulmonary capillaritis and diffuse alveolar hemorrhage complicating
polymyositis.
