Retinal Vasculitis as a Rare Presentation of Microscopic Polyangiitis

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 <i>amaurosis fugax</i>. 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.

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Journal
Cureus
Published
2024-12-28
DOI
https://doi.org/10.7759/cureus.76532
Primary Topic
Vasculitis and related conditions
Type
article
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article

Retinal Vasculitis as a Rare Presentation of Microscopic Polyangiitis

João Chaves, Andreia Matos, C Macedo, P. Mendes
Cureus
Vasculitis and related conditions
article

Retinal Vasculitis as a Rare Presentation of Microscopic Polyangiitis

João Chaves, Andreia Matos, C Macedo, P. Mendes
article en

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 <i>amaurosis fugax</i>. 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.

CureusVol. 16(12)
Escola Superior de Tecnologia da Saúde de Coimbra (PT), Administração Regional de Saúde de Lisboa e Vale do Tejo (PT), University of Coimbra (PT)
Zero hunger
Openalex Percentile: Top 56%
Vasculitis and related conditions
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