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Chronic Inflammatory Demyelinating Polyneuropathy (CIDP): An Uncommon Manifestation of Systemic Lupus Erythematosus (SLE)

Challenging differential diagnosis, Management of emergency care, Rare disease

Hrudya Abraham, Jose Kuzhively, Syed W. Rizvi

(Division of Rheumatology, Department of Internal Medicine, MacNeal Hospital, Berwyn, USA)

Am J Case Rep 2017; 18:980-983

DOI: 10.12659/AJCR.903541

Published: 2017-09-12


BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an uncommon manifestation of systemic lupus erythematosus (SLE). We report a case of SLE presenting as CIDP and discuss the diagnosis, management, and prognosis of CIDP.
CASE REPORT: A 40-year-old woman with a past medical history of SLE treated with hydroxychloroquine presented with bilateral, progressive, ascending, sensory and motor neuropathy. Physical examination showed weakness and reduced temperature of all extremities, reduced pinprick and vibration sense of the distal extremities, loss of reflexes, and walking with a wide-based unsteady gait. Laboratory investigations showed positive antinuclear antibodies (ANA), anti-(smooth muscle (SM) antibody, anti-RNP antibody, anti-SSA antibody, anti-ds-DNA antibody, and an erythrocyte sedimentation rate (ESR) of 75 mm/hr, low C4, leukopenia, and anemia. Electromyography (EMG) confirmed the diagnosis of CIDP. The patient’s neuropathy and muscle weakness improved on treatment with intravenous immunoglobulin (IVIG) and high-dose steroids.
CONCLUSIONS: The early clinical diagnosis of CIDP, supported by serological autoantibody profiles associated with SLE, can predict a good response to steroids. Most patients with CIDP are treated successfully with steroids if the diagnosis is made early. IVIG, plasmapheresis, or immunosuppressive therapy should be considered if there is no response to steroids.

Keywords: Immunoglobulins, Lupus Vasculitis, Central Nervous System, Methylprednisolone, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, Prednisone



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