A paraneoplastic vasculitis may occur that primarily affects nerves and muscles. The patient develops a microvasculitis mediated by CD8+ T cells. Affected nerves develop axonal degeneration. A similar process affecting small blood vessels can be identified in muscle biopsies.


Causative tumors include:

(1) small cell lung cancer

(2) malignant lymphoma

(3) renal cell carcinoma

(4) cholangiocarcinoma

(5) gastric carcinoma

(6) prostate carcinoma if male


The condition tends to occur in older males.


Clinical findings:

(1) painful neuropathy

(1a) symmetric sensorimotor polyneuropathy

(1b) asymmetric sensorimotor polyneuropathy

(1c) multiple mononeuropathy

(2) The onset may precede diagnosis of the underlying tumor.

(3) The condition is subacute and progressive.

(4) The ESR is increased.

(5) The protein in the CSF is elevated.

(6) Elevation of muscle enzymes.

(7) Some patients have anti-Hu antibodies.


The neuropathy improves following a combination of cytotoxic chemotherapy and corticosteroids.


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