RESEARCH - Chronic inflammatory demyelinating polyneuropathy in patients with SLE: prognosis and outcome

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Seminars in Arthritis and Rheumatism

Volume 35, Issue 3 , December 2005, Pages 175-184

Chronic Inflammatory Demyelinating Polyneuropathy in Patients with Systemic

Lupus Erythematosus: Prognosis and Outcome

Ernest R. Vina MD, Annie J. Fang MD, J. Wallace MD and H.

Weisman MD

Objectives

To identify clinical characteristics, laboratory features, approaches to

management, and predictors of outcome of chronic inflammatory demyelinating

polyneuropathy (CIDP) in patients with systemic lupus erythematosus (SLE).

Methods

An analysis of 6 adults with the concurrent diagnosis of CIDP and SLE seen

at a SLE Clinic from 1994 to 2004 with a review of 13 patients with SLE and

CIDP reported in the medical literature from 1950 through 2004.

Results

Among our 6 patients with SLE and CIDP, 3 (50%) achieved a substantial

clinical response to intravenous immunoglobulin (IVIg) and the remainder had

a minimal response. The improved patients were more likely to have received

treatment earlier (within 1 year of CIDP onset) and to respond faster (<1 to

3 months) than minimally improved patients. They tended to have CIDP

features of weakness of all extremities, hyporeflexia of the upper

extremities, and slowed nerve conduction velocity of the motor median nerve.

Compared with minimal responders, responders had more serious internal organ

manifestations and multiple autoantibodies associated with SLE. Review of

the literature identified 13 previously reported CIDP patients with SLE.

Many had neurological involvement of all extremities, nerve biopsies showing

demyelination, and serious SLE internal organ manifestations. Most were

treated with steroids, but the 1 treated with IVIg had similar

characteristics to our subset of patients who improved with IVIg.

Conclusions

CIDP is an uncommon, but not rare, manifestation of SLE. Certain

characteristics including early CIDP diagnosis, involvement of all 4

extremities, hyporeflexia of the upper extremities, and slowed motor nerve

conduction velocity of the median nerve in addition to SLE involvement of

critical internal organs and the presence of multiple antibodies associated

with SLE all appear to predict a good response to IVIg.

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Mayo Clinic in Rochester

http://www.mayoclinic.org/rochester

s Hopkins Medicine

http://www.hopkinsmedicine.org