Nonsystemic Vasculitic Neuropathy Research Articles

Matrix metalloproteinase upregulation in chronic inflammatory demyelinating polyneuropathy and nonsystemic vasculitic neuropathy.

To determine the expression pattern and cellular source of matrix metalloproteinases (MMPs) in chronic inflammatory demyelinating polyneuropathy (CIDP) and nonsystemic vasculitic neuropathy (NSVN). MMPs are endopeptidases involved in tissue destruction and infiltration by immune cells in multiple sclerosis and Guillain-Barré syndrome. Enzyme inhibitors of MMPs attenuate clinical symptoms in corresponding animal models of these diseases. MMP inhibition may therefore be a novel approach for the treatment of CIDP and NSVN. However, the spectrum of MMPs expressed in chronic inflammatory neuropathies has not been established. The expression of MMP-2, MMP-3, MMP-7, and MMP-9 in T cells, macrophages, and stromal cells in CIDP, NSVN, and noninflammatory neuropathies (NIN) was quantitated by immunohistochemistry. Results were correlated with clinical and electrophysiologic findings. The production of MMP-2 and MMP-9 is increased in nerve tissue in CIDP and NSVN compared with NIN. T cells are the predominant source of MMP-2 and MMP-9 in CIDP and NSVN, whereas macrophages contribute only to a minor extent. Stromal cells of the perineurium/epineurium are an additional source of MMP-2 in NSVN, but not in CIDP. Expression of MMP-3 and MMP-7 was not detectable in CIDP or NSVN. Expression of MMP-2 and MMP-9 did not correlate with clinical disease activity and electrophysiologic measurements. The upregulation of MMP-2 and MMP-9 is a specific feature of CIDP and NSVN, and selective inhibitors of these enzymes could be used to prevent inflammatory tissue damage. The similar increase of MMP-2 and MMP-9 in both demyelinating (CIDP) and nondemyelinating (NSVN) neuropathies raises doubts about whether MMPs play a primary role in demyelination.

Nonsystemic vasculitic neuropathy presenting with truncal segmental sensory disturbance and hyperhidrosis

Nonsystemic vasculitic neuropathy presenting with truncal segmental sensory disturbance and hyperhidrosis

PARANEOPLASTIC VASCULITIS OF THE PERIPHERAL NERVOUS SYSTEM

Paraneoplastic vasculitic neuropathy (PVN) is a rare paraneoplastic syndrome characterized by non-systemic subacute vasculitic neuropathy. The two cancers most commonly associated with PVN are small cell lung cancer (SCLC) and lymphomas. Neuropathy varies from mononeurotherapy multiplex to symmetrical polyneuropathy. Two helpful laboratory abnormalities are a high erythrocyte sedimentation rate (ESR) and high cerebro spinal fluid (CSF) protein content. Axonal neuropathy is the characteristic finding in electrophysiological studies. Nerve biopsy is crucial to document microvasculitis. Recognition of this entity is important because of its potential treatability. Unlike other paraneoplastic syndromes, anti-cancer chemotherapy and immunotherapy for vasculitis are both effective in this disorder.

Nonsystemic vasculitic neuropathy.

Among 65 patients with necrotizing vasculitis, 45 had systemic and 20 had nonsystemic vasculitic neuropathy. In nonsystemic vasculitic neuropathy, clinically only nerves are affected; there are no, or few, constitutional symptoms or serological abnormalities. The clinical and pathological features are those of an ischaemic neuropathy caused by a necrotizing vasculitis of small arterioles. These 20 patients had neuropathic symptoms for a median time of 11.5 yrs (range 1-35 yrs). The clinical pattern of neuropathy was that of multiple mononeuropathy in 13, asymmetric neuropathy in 4, distal polyneuropathy in 3, and sensory polyneuropathy in 1. As compared with their initial evaluation, 8 are now worse, 5 are better, 4 are approximately the same, and 3 are dead from unrelated causes. Prednisone was thought to prevent the development of new lesions in some cases. By contrast, of the 41 patients with systemic necrotizing vasculitis whose outcome is known, 12 are dead (median time, 1.5 yrs, range 3 months-8 yrs) and 29 are alive (median time, 6 yrs, range 6 months-22 yrs).