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Granulomatosis With Polyangiitis in a Young Lady: Challenges in Achieving Remission
Nurul Ibtisam Mohammad1, Shakiran Gunaseelan2, Sangeeta Kuganasan2
1Ophthalmology, Universiti Kebangsaan Malaysia Medical Centre (UKMMC), Kuala Lumpur, MYS.
Cureus
|January 6, 2025
Summary
Granulomatosis with polyangiitis (GPA) requires aggressive treatment. This case highlights the need for tailored immunomodulatory therapy to manage relapsing ANCA-associated vasculitis (AAV) effectively.
Area of Science:
- Rheumatology
- Ophthalmology
- Otolaryngology
Background:
- Granulomatosis with polyangiitis (GPA) is a severe form of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
- Aggressive immunosuppression is typically necessary for inducing remission in GPA.
- ANCA-associated vasculitis can affect multiple organ systems, including the eyes and sinuses.
Observation:
- A young Malay female presented with recurrent ANCA-positive nodular anterior scleritis.
- Initial treatment with corticosteroids and methotrexate failed to prevent relapses.
- The patient developed epistaxis, confirmed as granulomatous vasculitis via sino-nasal biopsy.
Findings:
- Despite cyclophosphamide and methylprednisolone for optic neuropathy, the patient experienced further relapses.
- Rituximab was ultimately required to achieve disease control.
- This case underscores the complex and relapsing nature of GPA, even with standard treatments.
Implications:
- Optimal and individualized immunomodulatory strategies are essential for managing GPA.
- Early recognition and aggressive treatment are crucial to prevent irreversible organ damage.
- Multidisciplinary care is vital for patients with refractory or relapsing AAV.
