Vasculitis Presenting as Autonomic Failure

Neurologist. 2021 Jul 6;26(4):117-121. doi: 10.1097/NRL.0000000000000327.

Abstract

Introduction: Granulomatosis with polyangiitis (GPA) is a vasculitic process that can cause neurological dysfunction in addition to characteristic sinus and pulmonary manifestations. This case report highlights the spectrum of nervous system manifestations and includes rarely reported autonomic and pituitary involvement.

Case report: A 62-year-old woman presented with orthostatic intolerance, tachycardia, dry mouth, and temperature sensitivity; subsequent autonomic reflex study demonstrated widespread postganglionic sympathetic sudomotor, cardiovagal, and cardiovascular adrenergic impairment reflective of autonomic neuropathy and overall autonomic failure. Additional progressive symptoms included dysarthria, dysphagia, bilateral hearing loss, voice hoarseness, and right-sided facial numbness with multiple cranial neuropathies identified on neurological examination. The diagnosis of central diabetes insipidus was also confirmed. Pachymeningitis was present on brain magnetic resonance imaging. Pathologic review of the dural biopsy specimen revealed necrotizing granulomatous vasculitis consistent with GPA. She was treated with intravenous methylprednisolone and rituximab. Over the next 2 months, she had near-complete resolution of her symptoms with normalization on repeat autonomic testing.

Conclusions: This is a unique GPA case presenting with autonomic failure and pituitary dysfunction with conclusive objective findings of autonomic dysfunction. Autonomic dysfunction and other disease manifestations were responsive to immunosuppressive therapy.

Publication types

  • Case Reports

MeSH terms

  • Cranial Nerve Diseases*
  • Female
  • Granulomatosis with Polyangiitis* / complications
  • Granulomatosis with Polyangiitis* / diagnosis
  • Granulomatosis with Polyangiitis* / drug therapy
  • Humans
  • Meningitis*
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Rituximab

Substances

  • Rituximab
  • Methylprednisolone