Retinal Vasculitis as a Rare Presentation of Microscopic Polyangiitis
Cristiane P Macedo1, Andreia M Matos1, João Chaves2
1Internal Medicine, Unidade Local de Saúde de Coimbra, Coimbra, PRT.
Abstract:
Microscopic polyangiitis (MPA) is a rare, autoimmune, small-vessel vasculitis usually described with the presence of perinuclear antineutrophil cytoplasmic antibodies (p-ANCA). It encompasses a broad spectrum of clinical features, including fatigue, weight loss, fever, arthralgia, skin lesions, and involvement of the lungs or kidneys. Ocular manifestations, however, are extremely rare. We report a case of a 60-year-old Caucasian male who presented with sudden left-hand weakness, fever, and amaurosis fugax. He also complained of fatigue and stocking-glove paresthesia over the preceding two months. Neurological evaluation excluded acute stroke, and ophthalmologic examination, supported by fluorescein angiography, revealed mild right-sided temporal retinal vasculitis without other retinal changes. Laboratory investigations demonstrated inflammatory anemia, elevated erythrocyte sedimentation rate, mild proteinuria, autoimmune hypothyroidism, and positive p-ANCA-myeloperoxidase (MPO) with a nuclear-dense anti-nuclear antibodies (ANA) pattern. Renal biopsy confirmed crescentic vasculitis, while nerve biopsy indicated small-vessel vasculitis. Treatment with prednisolone and azathioprine resulted in clinical improvement. This case highlights retinal vasculitis as a rare manifestation of MPA.
Insights
Microscopic polyangiitis (MPA), a rare autoimmune vasculitis, can present with unusual ocular symptoms. This case highlights retinal vasculitis as an extremely rare manifestation of MPA, emphasizing the need for comprehensive evaluation.
Area of Science:
- Rheumatology
- Ophthalmology
- Neurology
Background:
- Microscopic polyangiitis (MPA) is a rare autoimmune small-vessel vasculitis, typically associated with perinuclear antineutrophil cytoplasmic antibodies (p-ANCA).
- Clinical manifestations commonly involve fatigue, fever, weight loss, arthralgia, skin lesions, and kidney or lung involvement.
- Ocular manifestations are exceptionally rare in MPA.
Observation:
- A 60-year-old male presented with sudden left-hand weakness, fever, and transient vision loss (amaurosis fugax).
- He reported preceding fatigue and stocking-glove paresthesia.
- Ophthalmologic examination revealed mild temporal retinal vasculitis, confirmed by fluorescein angiography.
Findings:
- Laboratory results showed inflammatory anemia, elevated ESR, mild proteinuria, autoimmune hypothyroidism, and positive p-ANCA-myeloperoxidase (MPO) antibodies.
- Renal and nerve biopsies confirmed crescentic and small-vessel vasculitis, respectively.
- The patient tested positive for anti-nuclear antibodies (ANA) with a nuclear-dense pattern.
Implications:
- This case underscores retinal vasculitis as a rare, yet possible, ocular manifestation of microscopic polyangiitis.
- Prompt diagnosis and treatment with immunosuppressants like prednisolone and azathioprine led to clinical improvement.
- Highlights the importance of considering MPA in patients presenting with unexplained neurological and ocular symptoms, even without typical systemic involvement.
