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Necrotizing myopathy with pipestem capillaries, microvascular deposition of the complement membrane attack complex (MAC), and minimal cellular infiltration

Muscle Research Laboratory and Department of Neurology, Mayo Clinic, Rochester, MN.

Three adult patients, two with undifferentiated connective tissue disease and one with carcinoma, had a distinctive pathologic reaction pattern consisting of necrotizing myopathy, minimal cellular infiltration, and a microangiopathy with thick "pipestem" vessels and microvascular deposits of complement membrane attack complex. Quantitative analysis revealed focal capillary depletion. This pattern represents an immune-mediated microangiopathy and is distinct from that observed in other inflammatory myopathies.

Address correspondence and reprint requests to Dr. Andrew G. Engel, Mayo Clinic, Rochester, MN 55905.

Supported by NIH grant NS-6277 and a research grant from the Muscular Dystrophy Association.

Received September 12, 1990. Accepted for publication in final form November 8, 1990.

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