Myositis-Specific Autoantibodies

SELECTED CASE A 27-year-old stonemason, who was previously well, had acute onset of weakness, stiffness, and pain in the proximal arm, leg, and back muscles. A week later low-grade evening fevers, diffuse swelling of his extremities and face, and a bluish rash over his eyelids developed; 2 weeks after that, he noticed shortness of breath, dysphagia, and a thickening of his voice. During the next several days, his condition deteriorated rapidly, with increasing shortness of breath and cyanosis, and he died of a presumed pulmonary arrest. Autopsy showed firm, fluid-filled lungs, splenomegaly, and swollen proximal muscles; the distal muscles, eye muscles, and tongue muscles were spared. Histopathologic examination showed a focal round-cell infiltration permeating the interstitium of the affected muscles and muscle cells in various stages of degeneration. No microorganisms were seen. THE IDIOPATHIC INFLAMMATORY MYOPATHIES With the publication of this case over 100 years ago,¹Professor Hans Unverricht