The introspection on the diagnosis and treatment process of a case of Guillain–Barré syndrome (GBS) attributed to systemic lupus erythematosus (SLE)

Abstract

Rationale:Systemic lupus erythematosus (SLE) is an autoimmune inflammatory connective tissue disorder. It may cause neurologic damage which is mainly characterized by central and mental system, while peripheral sexual damage is relatively rare in which Guillain–Barré Syndrome (GBS) as the first performance is more rare . GBS is an autoimmune peripheral neuropathy usually triggered by an antecedent bacterial or viral infection, with SLE being a rare cause.Patient concerns:A 65-year-old male presented to the hospital with progressive numbness and adynamia in extremities. His vital signs were stable. 5 days later, his condition aggravated and mechanical ventilation was necessitated owing to severe dyspnea.Diagnoses:Based on the clinical symptoms and results of the lumbar puncture and electromyography, he was first diagnosed as GBS, however, after treatment his condition was deteriorate and the blood test showed abnormal immune indices, then renal biopsy was performed, which confirmed the diagnosis of peripheral nervous system in patients with systemic lupus erythematosus (PNS-SLE).Interventions:Firstly he was treated with intravenous immunoglobulin (IVIG) for 5 days. After his condition deterioration, he was conducted endotracheal intubation and, finally, a tracheostomy was performed. Later on he was treated with steroid therapy for several weeks.Outcomes:The patient showed remarkable recovery and was able to walk on his own by the time of discharge.Lessons:PNS-SLE can, by itself, be one of the main causes of morbidity and mortality. Electromyography and renal biopsy should be considered when relevant. Peripheral neuropathy in SLE should be given greater recognition, and rarer forms of presentation should be taken seriously in the differential diagnosis when the clinical picture is atypical. Glucocorticoids may play an important role in the treatment of PNS-SLE.

Case Report: Guillain-Barré Syndrome as Primary Presentation of Systemic Lupus Erythematosus (SLE-GBS) in a Teenage Girl

“…Current treatment recommendations for pediatric (non-SLE) GBS emphasize the use of IVIG over PLE ( 39 , 40 ). However, this approach appears to be inadequate for patients with severe SLE-GBS as inferred from available experience in children and adults ( 5 , 7 , 16 – 18 , 27 , 29 , 37 , 38 ), including our patient.…”

“…No clinical details are available from those cases. The pathophysiology of GBS in SLE patients remains speculative and is thought to involve lupus-induced auto-antibodies against myelin tissue ( 3 , 29 ).…”

“…There are no published clinical trials or contemporary therapeutic guidelines for patients with SLE-GBS. Anecdotally, adult patient were treated with high-dose glucocorticoids, IV or oral cyclophosphamide, PLEX, IVIg, and mycophenolate mofetil, in various combinations ( 6 , 7 , 27 , 29 , 37 , 38 ).…”

High-resolution ultrasound of peripheral neuropathies in rheumatological patients: An overview of clinical applications and imaging findings

“…The most commonly affected nerves are in decreasing order the peroneal nerve, the tibial, and sural nerves, while the ulnar and median nerves involvement is uncommon (54). Although GBS is a rare type of PN in SLE patients, it represent one of the main causes of morbidity and mortality (57,58). The pathogenetic mechanisms of PN directly attributable to SLE neuropathy are not completely understood, but may include auto-antibody-mediated nerve damage and nerve vasculitis.…”

Clinical Manifestations, Pathogenesis, Diagnosis and Treatment of Peripheral Neuropathies in Connective Tissue Diseases: More Diverse and Frequent in Different Subtypes than Expected

“…Although Guillain-Barré Syndrome (GBS) is a rare manifestation of SLE, which falls under the category of PNS-SLE, it was not mentioned in these studies. It is, however, important to remember that GBS can be one of the main causes of morbidity and mortality in PN-SLE patients [15,16].…”