Lambert-Eaton myasthenic syndrome and follicular thymic hyperplasia in systemic lupus erythematosus

Lupus. 2011 Jun;20(7):745-8. doi: 10.1177/0961203310393769. Epub 2011 Mar 22.

Abstract

Fatigue is a prominent feature of systemic lupus erythematosus (SLE), usually ascribed to various factors, such as muscle or joint involvement, anaemia or depression. The Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoantibody-mediated disorder of neuro-muscular transmission. We report on a well-defined LEMS associated with thymus hyperplasia in a SLE patient. An African 41 years-old SLE patient presented with persisting fatigue, myalgia and dyspnea, abolished reflexes and a bilateral ptosis. Neuromuscular electrodiagnostic study showed a clear-cut potentiation that was typical of a pre-synaptic neuromuscular junction disease. Anti-calcium gated channels antibodies were disclosed in serum and a diagnosis of LEMS was made. A total body CT-scan revealed an antero-superior mediastinal mass, compatible with thymoma. The tumour was surgically removed with a final diagnosis of follicular thymic hyperplasia. In conclusion, our observation provides a new example of entangled organ-specific and systemic autoimmunity in the context of thymus pathology. Potentiation study during electromyography should be performed systematically to rule out LEMS in patients with SLE and muscle weakness.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Fatigue / diagnosis
  • Fatigue / etiology
  • Humans
  • Lambert-Eaton Myasthenic Syndrome / complications*
  • Lambert-Eaton Myasthenic Syndrome / diagnosis
  • Lupus Erythematosus, Systemic / complications*
  • Male
  • Mauritania
  • Thymoma / diagnosis
  • Thymoma / surgery
  • Thymus Hyperplasia / complications*
  • Thymus Hyperplasia / diagnosis
  • Thymus Neoplasms / diagnosis
  • Thymus Neoplasms / surgery
  • Tomography, X-Ray Computed
  • Whole Body Imaging