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Microscopic polyangitis presenting with temporal arteritis and multiple cranial neuropathies

Akiyoshi Morinaga1, Kenjiro Ono, Kiyonobu Komai

  • 1Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, Takaramachi 13-1, Kanazawa, Japan. amorinag@med.kanazawa-u.ac.jp

Journal of the Neurological Sciences
|March 24, 2007
PubMed

Insights

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis can affect large and small vessels simultaneously. This case highlights ANCA vasculitis presenting with temporal arteritis and kidney disease, emphasizing its diverse vascular involvement.

Area of Science:

  • Rheumatology
  • Neurology
  • Nephrology

Background:

  • Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) encompasses a group of systemic autoimmune diseases characterized by inflammation of small-to-medium-sized blood vessels.
  • Neurological complications, including cranial neuropathies, can occur in AAV, but simultaneous large vessel involvement is less commonly reported.

Observation:

  • A 77-year-old male presented with dysphagia, hoarseness, dysgeusia, gait instability, and temporal pain, alongside multiple cranial neuropathies.
  • Investigations revealed elevated perinuclear ANCA, severe inflammation, mild proteinuria, and dural enhancement on brain MRI.
  • Biopsies confirmed necrotizing glomerulonephritis and temporal arteritis without giant cells.

Findings:

  • The patient was diagnosed with microscopic polyangiitis manifesting as temporal arteritis and cranial nerve involvement.
  • Pathological confirmation of AAV lesions in both the temporal artery (large vessel) and the kidney (microvasculature) was established.
  • Treatment with predonisolone led to symptom resolution and normalization of laboratory markers.

Implications:

  • This case demonstrates that AAV can affect large arteries, such as the temporal artery, concurrently with microvasculature in organs like the kidney and nerves.
  • It underscores the importance of considering AAV in patients presenting with cranial neuropathies and systemic inflammation, even with atypical large vessel involvement.
  • The findings expand the understanding of the vascular distribution in AAV, suggesting a broader spectrum of potential organ system engagement.

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