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Nerve and Muscle Biopsy for the Diagnosis of Vasculitis
By Michael Rubin, MD, FRCP(C), Professor of Clinical Neurology, Weill Cornell Medical College. Dr. Rubin reports that he receives grant/research support from Pfizer and is on the speaker's bureau of Athena Diagnostics.
Synopsis: Biopsy of the clinically affected nerve gives the highest yield for the diagnosis of vasculitic neuropathy.
Source: Bennett DL, Groves M, Blake J, et al. The use of nerve and muscle biopsy in the diagnosis of vasculitis: A 5-year retrospective study. J Neurol Neurosurg Psychiatry 2008;79;1376-1381.
Is nerve biopsy, muscle biopsy, or both useful to diagnose vasculitis? To address this question, a retrospective database review was undertaken of all nerve and muscle biopsies performed between January 1999 and August 2005 at the National Hospital for Neurology and Neurosurgery, Queen Square, London, England, to identify those with biopsy-proven peripheral nerve vasculitis. Clinical, physiological, and pathological data were reviewed, compared, and statistically analyzed using the chi square test, Fisher's exact test, or McNemar's test, as appropriate, and the Mann-Whitney rank sum test.
Peripheral nerve vasculitis was documented on biopsy in 53 patients, 31 (58%) having vasculitic neuropathy as part of a multisystem disorder, and 22 (42%) with a disorder restricted to the peripheral nervous system. Women predominated, 30:23, and mean age was 56 years (range 32–79 years). Diagnoses included microscopic polyangiitis (n -4), Churg-Strauss, hepatitis B, polyarteritis nodosa, rheumatoid vasculitis, Wegener's granulomatosis (n = 3 each), hepatitis C, HIV, paraneoplastic (small-cell lung cancer), Sjogren's syndrome, undifferentiated connective tissue disorder (n = 2 each), graft-vs.-host disease, and systemic sclerosis (n = 1 each). Most neuropathies (87%) were painful, presenting either as asymmetric sensorimotor neuropathy (45%), mononeuritis multiplex (20%), or pure, small-fiber, painful, sensory neuropathy (17%). Cranial nerve involvement was seen in only four (8%), and brachial or lumbosacral plexopathy as a presentation was seen in one each. Motor nerves most frequently involved included the peroneal (86%), ulnar (63%), median (58%), tibial (40%), radial (35%), femoral (32%), and musculocutaneous (9%) nerve.
Biopsy of the sural (n = 50), radial (n = 3), or superficial peroneal nerve (one patient had two biopsies) demonstrated definite vasculitis in 19 (36%) and probable vasculitis in 33 (62%). Of 24 patients who also underwent vastus lateralis (n = 23) or peroneus brevis muscle biopsy, three (13%) showed definite and eight (33%) showed probable vasculitis. Vasculitis in muscle but not nerve was demonstrated in only one patient. Vastus lateralis muscle biopsy adds little, other than a scar, to sural nerve biopsy in the diagnosis of vasculitis. If muscle tissue is desired, it is best obtained through the same incision used to obtain the nerve sample.
Extensive review of the literature, using MEDLINE, EMBASE, and Current Contents, indicates that for the evaluation of peripheral neuropathy, nerve biopsy is generally acknowledged as useful to diagnose amyloid, vasculitis, or atypical chronic inflammatory demyelinating polyneuropathy. However, no reliable data indicates when it is likely to be helpful, which nerve is best to sample, when muscle tissue should also be obtained, which nerve or muscle is best to sample, when both should be obtained, and which combination gives the highest yield.1 Presently, common sense dictates that an affected and accessible nerve or muscle would likely give the highest yield and, where necessary, both should ideally be obtained through the same incision.
1. England JD, Gronseth GS, Franklin G, et al. Practice Parameter: Evaluation of distal symmetric polyneuropathy: Role of autonomic testing, nerve biopsy, and skin biopsy (an evidence-based review). Report of the American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Academy of Physical Medicine and Rehabilitation. Neurology 2009:72;185-192.