Chronic Inflammatory Demyelinating Polyneuropathy in Patients with Systemic Lupus Erythematosus: Prognosis and Outcome

https://doi.org/10.1016/j.semarthrit.2005.08.008Get rights and content

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.

Section snippets

Methods

Patients with a concurrent diagnosis of CIDP and SLE who were receiving rheumatological health care services from 1 of the coauthors (D.J.W.) between 1994 and 2004 were included in the study. We retrieved clinical and laboratory data, pharmacological treatments, and therapeutic responses from a systematic review of these patients’ charts. For the literature review, the PUBMED database was searched for published case series or reports and review articles of CIDP in patients with SLE in the

Case 1

A black woman, received a diagnosis of SLE in 1994 at the age of 20 years when she presented with a malar rash, photosensitivity, arthritis, myalgias, serositis, and elevated antinuclear antibody (ANA), anti-double-stranded DNA, antiribonucleoprotein (RNP), and anti-Smith (Sm) antibodies. Her lupus had been persistently active despite aggressive therapy with corticosteroids. She noticed increased generalized weakness in November 2001 and, by January 2002, she was unable to turn the key of her

Case Series

Six patients with the diagnoses of both CIDP and SLE were identified from the referral practice database of over 1000 SLE patients from a single rheumatologist specializing in the diagnosis and treatment of SLE (D.J.W.). Cases where there were ambiguities in either diagnosis were deleted from further analysis. All patients were treated with IVIg at some point in their course. Most were started on IVIg due to lack of or minimal response to steroids (patient 1, 2, 3, 4) but others were started on

Discussion

We have described distinct characteristics of 6 patients diagnosed with CIDP and SLE and placed these characteristics in the context of their responses to IVIg management. Patients who respond to IVIg were more likely to have received treatment within 1 year of their CIDP onset, and their improvement occurred more rapidly. In addition, improved patients appear to have minimal discrepancy in weakness between their arms and legs, demonstrate areflexia or hyporeflexia of the upper extremities, and

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