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Myopathy and Neuropathy with Pipestem Capillaries and Vascular Activated Complement Deposition

Necrotizing myopathy with pipestem capillaries defines an immune-mediated disorder of skeletal muscle characterized by minimal cellular infiltration, muscle fiber necrosis, and microangiopathy with complement membrane attack complex (MAC; C5b9) deposition. Thickened, hyalinized (“pipestem”) vessels, ultrastructurally embedded in amorphous material, and capillary loss are further features.1 The rare patients reported showed proximal weakness and high creatine kinase,1,–,4 frequently associated with myalgia. They presented with other symptoms suggesting autoimmune disorders (including cerebral vasculitis) or cancer and an overall unfavorable prognosis,1,2 despite the response of muscle symptoms to immunosuppressive treatments. A pathophysiologic concept to link these multiorgan symptoms is not known, possibly because only one sample outside muscle has so far been taken for comparison of histopathologic features. That skin probe showed “mild fibrosis and lymphocytic perivascular cuffs in superficial dermis.”2

A 57-year-old normotensive, nondiabetic man presented with rapid onset edema of the distal legs, dysesthesia, loss of ankle reflex, and bilateral foot drop (Medical Research Council grade 0). Proximal and upper limb strength and reflexes were intact. Neurophysiologic examination demonstrated loss of compound muscle action potentials (CMAPs) of both peroneal nerves and the left tibial nerve, reduced CMAP amplitude of the right tibial nerve, and loss of sensory nerve action potentials of both sural nerves. Neurography of the right median and ulnar nerves showed normal results. Needle myography showed abnormal spontaneous activity and only a few recordable muscle unit potentials in the tibialis anterior …