Progressive muscle weakness and amyotrophy during pregnancy as the first manifestation of systemic lupus erythematosus: A case report and review of literature

Sci Prog. 2021 Oct;104(4):368504211050276. doi: 10.1177/00368504211050276.

Abstract

Background: Systemic lupus erythematosus is a common autoimmune disease involving multiple systems. Clinical involvement of the central and peripheral nervous systems is not unusual, but peripheral neuropathy in systemic lupus erythematosus with chronic inflammatory demyelinating polyneuropathy is uncommon. Our study aimed to illustrate the clinical features, diagnosis, and treatment of systemic lupus erythematosus combined with chronic inflammatory demyelinating polyneuropathy, and to aid in the identification of peripheral neuropathy in systemic lupus erythematosus.

Methods: This article reports a case of systemic lupus erythematosus with onset in pregnancy, with chronic inflammatory demyelinating polyneuropathy as the first manifestation. We then analyze the identification of common peripheral neuropathy in systemic lupus erythematosus in detail, based on a literature review of confirmed cases of systemic lupus erythematosus combined with chronic inflammatory demyelinating polyneuropathy.

Results: A 34-year-old woman presented progressive muscle weakness and muscular atrophy in the extremities during pregnancy, 3 years previously. At 4 months after onset, she had completely lost the ability to hold objects and walk, and had slight numbness in the limbs, without paresthesia. Her condition was misdiagnosed as "motor neuron disease" at the time. Three years after onset, her condition was revisited because of nephrotic syndrome, and she was diagnosed with nephrotic syndrome and peripheral nerve injury caused by systemic lupus erythematosus. After immunosuppressive treatment with corticosteroids and intravenous cyclophosphamide, her symptoms of muscle weakness were markedly improved. This article summarizes the characteristics of systemic lupus erythematosus combined with chronic inflammatory demyelinating polyneuropathy that have been reported in the literature, from the aspects of morbidity, disease progression, nerve injury, laboratory examinations, and treatment response.

Conclusions: Our identification of a common peripheral neuropathy in systemic lupus erythematosus will help to improve clinicians' understanding of various peripheral neuropathies in systemic lupus erythematosus. It will also aid in the early diagnosis and treatment of such patients, thus improving their long-term prognosis.

Keywords: Systemic lupus erythematosus; chronic inflammatory demyelinating polyneuropathy; muscle weakness; nephrotic syndrome; peripheral neuropathy.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adult
  • Female
  • Humans
  • Lupus Erythematosus, Systemic* / complications
  • Lupus Erythematosus, Systemic* / diagnosis
  • Male
  • Muscle Weakness / etiology
  • Muscular Atrophy
  • Nephrotic Syndrome*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating* / drug therapy
  • Pregnancy