Page 68 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

44 David S. Younger

man with a five year history of progressive generalized painful peripheral neuropathy that
was so severe before death that he was partially paralyzed, and unable to speak or swallow.
Postmortem examination showed PAN limited to the nerve trunks of the arms and legs.

The brain, cranial nerves, and spinal cord were normal except for early acute changes
without evidence of vasculitis.

Examination of all other organs failed to reveal a single vascular lesion, except one small
artery in the capsule of the prostate gland. Torvik and Berntzen [211] described a 76-year-old
woman with diffuse fever, pain, and central scotoma of the eye that improved with
corticosteroids.

A biopsy of the temporal artery and pectoralis muscle disclosed necrotizing arteries of
small arteries and arterioles of the pectoral muscle, and in small adventitial vessels of the
temporal artery without frank temporal arteritis. Postmortem examination showed no signs of
vasculitis from any visceral organ as well as the CNS, only evidence of healed or slightly
active vasculitic in numerous small arteries and arterioles of muscle and nerve tissue
measuring 50 to 200 µ in diameter. Younger and colleagues [212] reported a 59-year-old
diabetic man with lumbosacral radiculoplexus neuropathy (LSRPN) in which sural nerve
biopsy demonstrated non-necrotizing vasculitis with vessel wall inflammation and reactive
luminal connective tissue consistent with recanalization of a thrombus, and an adjacent nerve
fascicle that displayed marked focal loss of myelinated nerve fibers. He was treated with
intravenous corticosteroids and cyclophosphamide but died several weeks later. Postmortem
examination showed no evidence of vasculitis in any visceral organ or in tissue blocks from
the femoral, sciatic or extradural lumbar plexus. However there was perivasculitis of
epineurial and adjacent endoneurial vascular inflammation.

There have far more studies of living cohorts with NSPNV. Kissel and colleagues [213]
reported that 4.5% of 350 consecutive nerve biopsies performed at a single institution
evidenced peripheral neuropathy secondary to necrotizing angiopathy. Six patients manifested
a distal symmetrical sensorimotor polyneuropathy, while ten had a mononeuritis multiplex
presentation, eight of whom had overlapping involvement of peripheral nerves that obscured
the picture of mononeuritis. In three-quarters (12 patients), a specific underlying collagen
vascular disease was not diagnosed despite extensive clinical, radiologic, and serological
evaluation. Said and colleagues [214] studied 100 patients with necrotizing arteritis in muscle
or nerve biopsy tissue that occurred in the context of a connective tissue disorder in fifty-five
patients and in association with a disorder unrelated to connective tissue pathology in thirteen
others. The commonest complaints at presentation in this cohort were specific cutaneous
manifestation of vasculitis, including livedo, cutaneous necrosis, and nodules in one-third.
Thirty-two patients had neuropathy only and necrotizing arteritis, the most common
complaints at of which were spontaneous pain of neurogenic or muscle origin (48%). More
recently, Collins and colleagues [215] described forty-eight patients with NSPNV, 85% of
whom had extensive, overlapping involvement of multiple nerves. Peroneal nerve and
peroneal muscle tissue biopsy was 58% diagnostically sensitive compared to 47% for sural
nerve biopsy for the diagnosis of vasculitis. Combination therapy with corticosteroids and
cytotoxic agents was more effective than corticosteroids monotherapy for inducing remission
and improving disability, with trends toward reduced relapses and chronic pain.

Overall, ten patients died (21%) over the period of 63 months follow-up, five (10%) of
whom were related to the disease or treatment, including two patients who succumbed to
pulmonary emboli as a result of limited mobility of the legs or myocardial infarction in

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