Acute inflammatory demyelinating polyneuropathy as the initial presentation of lupus

Abstract

A 28-year-old woman presented at the emergency department (ED) with acute bilateral leg weakness and lost the ability to walk 1 day after noticing bilateral leg numbness and intermittent pleuritic pain for 3 weeks. Physical examination revealed bilateral decreased muscle strength with score 4 symmetrically in the legs and decreased deep tendon reflex. Laboratory tests indicated lymphopenia but no other grossly remarkable findings. Cerebrospinal fluid analysis demonstrated albuminocytologic dissociation. Electrophysiologic survey also indicated sensory-motor demyelinating polyneuropathy. The clinical picture was compatible with acute inflammatory demyelinating polyneuropathy (AIDP), the most prevalent form of Guillain-Barré syndrome (GBS). Plasma exchange was immediately arranged. After serial examination, pleuritic pain, persistent lymphopenia, positive immunologic findings of anti-DNA, and antinuclear antibodies led to a diagnosis of systemic lupus erythematosus (SLE). Prednisolone was added along with plasma exchange. The patient was able to walk after 2 weeks of therapy. Acute inflammatory demyelinating polyneuropathy presenting as the initial manifestation of SLE is rather rare. The precise mechanism of SLE-related AIDP remains unclear but is probably immune related. Although steroids are not recommended in the management of AIDP or GBS, patients with SLE-related AIDP may benefit from steroid therapy. This case highlights that early initiation of evaluation for SLE by ED physicians may facilitate correct diagnosis and better outcomes in patients presenting with GBS or AIDP in the ED.