Guillain–Barré syndrome occurring synchronously with systemic lupus erythematosus as initial manifestation treated successfully with low-dose cyclophosphamide

Abstract

Systemic lupus erythematous (SLE) is frequently encountered in clinical practice; a widespread immunological response can involve any organ system, sometimes leading to rare and diagnostically challenging presentations. We describe a 38-year-old female who presented with symmetric numbness and tingling of the hands and feet, and cervical pain. Imaging studies were not diagnostic of any serious underlying pathology. The patient developed ascending paresis involving lower extremities and cranial muscles (dysphagia and facial weakness). Guillain–Barré syndrome (GBS) was diagnosed on the basis of electromyography and lumbar puncture showing albuminocytologic dissociation. Intravenous immunoglobulins (IVIG) were administered for 5 days. Supported by anti-dsDNA antibody, oral ulcers, proteinuria of 0.7 g in 24 h, and neurological manifestation, she was diagnosed with lupus. After completion of IVIG, she received pulse-dose corticosteroids and one dose of low-dose cyclophosphamide. Her neurological symptoms improved and she had complete neurological recovery several months after her initial presentation. Literature search provides evidence of co-occurrence of lupus and GBS occurring mostly later in the course of the disease. However, GBS as initial manifestation of SLE is exceedingly rare and less understood. The association of GBS with lupus is important to recognize for rapid initiation of appropriate therapy and for consideration of immunosuppressive therapy which may affect the outcome.

Favorable response to cyclophosphamide in a patient with refractory Guillain–Barre syndrome associated with chronic lymphocytic leukemia

“…Cyclophosphamide is an alkylating agent widely used in anti-cancer therapies [22], including in CLL [1]. It also exerts an immunomodulatory effect on both B-and T-lymphocytes [23] and is used an immunosuppressive agent in a variety of clinical settings [22]. A number of reports demonstrate the utility of cyclophosphamide, at varying doses, in the successful treatment of GBS associated with vasculitic disorders [23][24][25], but its use in GBS associated with CLL has not previously been reported.…”

Fulminant Guillain-Barré syndrome in a patient with systemic lupus erythematosus

“…Review of the literature reveals that cases of GBS in the context of SLE have predominantly presented with acute inflammatory demyelinating polyneuropathy followed by the acute motor axonal neuropathy variant 6–19. Rare cases of acute motor sensory axonal neuropathy,14 20 Miller-Fisher syndrome21 and pharyngeal-cervical-brachial syndrome22 have also been reported.…”

“…Rare cases of acute motor sensory axonal neuropathy,14 20 Miller-Fisher syndrome21 and pharyngeal-cervical-brachial syndrome22 have also been reported. While standard GBS therapies such as plasma exchange and IVIG are often applied, in the majority of cases of concurrent SLE and GBS, additional immunosuppressive or immunomodulatory therapies have been employed, including corticosteroids, cyclophosphamide, azathioprine,14 mycophenolate mofetil17 and/or hydroxychloroquine 13 14 16 18 22. In some cases, patients did not respond to traditional GBS therapies, but had significant clinical improvement following immunosuppressive therapy 10 14 23…”

<p>Guillain&ndash;Barre syndrome as the first manifestation of juvenile systemic lupus erythematosus: a case report</p>

“…When GBS and SLE present concurrently, prednisone therapy is insufficient in about 50% of the cases 15. In this situations, cyclophosphamide along with pulse-dose steroids are usually used for SLE control 16. In our patient, according to American College of Rheumatology and Systemic Lupus International Collaborating Clinics Classification Criteria for SLE, the diagnosis of juvenile SLE was made with the presence of leukopenia, hemolytic anemia, hematuria, proteinuria, high level of ANA, decreased complement level, increased β2 glycoprotein, and positive anti-ds-DNA antibody.…”