Granulomatosis with polyangiitis: seeing the diagnosis
Max J Blumberg1, Cynthia I Tung2, Lindsay A May1
1Department of Ophthalmology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York, USA.
BMJ Case Reports
|May 11, 2017
Summary
A case study reveals granulomatosis with polyangiitis (GPA), a rare autoimmune disease, presenting with eye inflammation and kidney dysfunction. Early recognition and multidisciplinary collaboration are key for timely diagnosis and effective treatment.
Area of Science:
- Nephrology
- Ophthalmology
- Rheumatology
Background:
- Granulomatosis with polyangiitis (GPA) is a rare autoimmune vasculitis affecting small to medium-sized blood vessels.
- It commonly involves the respiratory tract and kidneys, but can affect multiple organ systems.
Observation:
- A 41-year-old woman presented with symptoms initially attributed to ibuprofen use and infection.
- She subsequently developed severe inflammatory eye disease (iritis, conjunctivitis, keratitis) and decreased renal function.
- The patient experienced severe hemoptysis before a definitive diagnosis was established.
Findings:
- Diagnosis of GPA was confirmed via blood tests, imaging, and kidney biopsy.
- Treatment with plasmapheresis, cyclophosphamide, and prednisone led to a positive clinical response.
Implications:
- This case underscores the importance of considering GPA in patients with inflammatory eye disease and renal impairment.
- Multispecialty collaboration, particularly involving ophthalmologists, is crucial for the early diagnosis and management of GPA.
- Prompt recognition and intervention can significantly improve patient outcomes in GPA.
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