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Peripheral ulcerative keratitis as presenting manifestation of systemic microscopic polyangiitis: a case report
David Díaz-Valle1, Blanca Benito-Pascual1, Rosalía Méndez-Fernández1
1Department of Ophthalmology, Hospital Clinico San Carlos, Madrid, Spain.
Abstract:
Microscopic polyangiitis is a rare autoimmune disease of unknown etiology, characterized by inflammation and necrosis of blood vessels. It forms a part of the antineutrophil cytoplasmic antibody-associated vasculitides-a heterogeneous group of disorders characterized by vasculitis. It is a systemic disease affecting multiple organs. The patients may present with a wide variety of symptoms. Ocular manifestations may present as its initial clinical symptoms, necessitating a multidisciplinary approach for reducing the morbidity and mortality. Early diagnosis aids in the formulation of appropriate treatment and prevention of further complications. Aggressive treatment, including surgery, is often necessary to limit structural damage and preserve visual function. We present the case of an 82-year-old woman who initially presented with peripheral ulcerative keratitis that led to the diagnosis of microscopic polyangiitis.
Insights
Microscopic polyangiitis, a rare autoimmune vasculitis, can initially present with eye symptoms like peripheral ulcerative keratitis. Early diagnosis and multidisciplinary treatment are crucial for managing this systemic disease.
Area of Science:
- Rheumatology
- Ophthalmology
- Pathology
Background:
- Microscopic polyangiitis (MPA) is a rare autoimmune disease causing blood vessel inflammation and necrosis.
- It is a subset of antineutrophil cytoplasmic antibody-associated vasculitides, affecting multiple organs.
- Ocular manifestations can be initial presenting symptoms, highlighting the need for a multidisciplinary approach.
Observation:
- An 82-year-old woman presented with peripheral ulcerative keratitis.
- This ocular symptom was the first indication of an underlying systemic disease.
- The presentation necessitated a comprehensive diagnostic workup.
Findings:
- The patient's peripheral ulcerative keratitis led to the diagnosis of microscopic polyangiitis.
- This case underscores the potential for ocular symptoms to be the primary presentation of MPA.
- Early identification of MPA through ocular signs is critical.
Implications:
- Prompt diagnosis of MPA, even when presenting with ocular symptoms, is vital for effective treatment.
- A multidisciplinary approach involving ophthalmology and rheumatology is essential for managing MPA.
- Aggressive treatment, potentially including surgery, can limit damage and preserve visual function in MPA patients.
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