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

Abstract

Background: Systemic lupus erythematosus (SLE) is an autoimmune disease which involves multiple organs, including peripheral nervous system. Case presentation: We describe a 12-year-old boy with progressively worsening neurological symptoms as first manifestation. Legs pain, loss of balance, and lower extremity weakness were the reason for his admission in neurologic ward. The patient was started on intravenous immunoglobulin therapy due to the possibility of Guillain–Barre syndrome and acute inflammatory demyelinating polyneuropathy (AIDP). However, there was no appropriate response and he developed recurrent attacks of polyneuropathy again with diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Then, he received intravenous pulse of methylprednisolone for 5 consecutive days followed by oral prednisolone for 3 months. One month after withdrawal of corticosteroid he admitted again with the same manifestations. Rheumatologic workup revealed the presence of leukopenia, hemolytic anemia, hematuria, proteinuria, positive antinuclear antibodies, and ds-DNA antibodies. On the basis of the American College of Rheumatology and Systemic Lupus International Collaborating Clinics Classification Criteria for SLE, the patient had underlying diagnosis of SLE. Eventually, he was treated by the pulse of methylprednisolone and cyclophosphamide, and oral hydroxychloroquine and prednisolone. His neurological and physical symptoms improved and complete neurological recovery occurred several months later. Conclusion: SLE and AIDP/CIDP are different entities, but ADP/CIDP can be part of the neurologic manifestations of the SLE. Although the association between AIDP/CIDP and SLE is very rare especially as a first manifestation of SLE, it should be early recognized for rapid appropriate treatment.

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

“…She eventually improved after the second dose of rituximab. Javadi Parvaneh et al described a 12-year-old Iranian boy with GBS, unresponsive to IVIg ( 17 ). He experienced a relapsing course with only partial improvement following high-dose glucocorticoid treatments.…”

“…Interesting, anti-nuclear (ANA) and anti-dsDNA antibodies were noted at initial GBS presentation. He only achieved lasting GBS remission after commencing IV cyclophosphamide therapy in addition to IVMP pulses and hydroxychloroquine ( 17 ). The two cases differ from the scenario described in a singular earlier report of a 13-year-old Japanese teenager with “late onset SLE-GBS,” more than 6 years after the diagnosis of SLE and four months after a lupus flare, with ongoing cutaneous manifestations.…”

“…CNS symptoms rarely precede the onset of typical features of SLE ( 5 ). Among the few reported cases of SLE with GBS as initial presentation, only two were in the pediatric age group ( 16 , 17 ). In another pediatric case of SLE-GBS ( 18 ), the neurological complication developed 6 years after the lupus diagnosis.…”

Chronic Inflammatory Demyelinating Polyradiculoneuropathy in Association With Concomitant Diseases: Identification and Management

“…CIDP can appear before, after, or at the same time as SLE, and the causes of the disease are varied and are not clear. In addition to the typical clinical manifestations of CIDP, SLE patients with CIDP may also present recurrent episodes of Guillain–Barré syndrome-like symptoms ( 85 , 86 ), which may range from weeks to months of the disease course. At this point, distinguishing the two is extremely difficult, and nerve conduction studies must be performed, which is especially important for identifying the type of neuropathy.…”

Nutraceuticals against Oxidative Stress in Autoimmune Disorders

“…Genetics, environment, drugs, pregnancy, and infection are only part of the complex disease mosaic [88]. It is a multiorgan disorder: mucosa, joints, kidneys, lungs, hearts, and particularly nerve cells in the peripheral nervous system are particularly targeted [89]. Among the risk factors for SLE is oxidative stress.…”