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Granulomatosis With Polyangiitis: A Clinical Case.
Filipa Rodrigues1, Ana Isabel Oliveira Sá1, Marta Mendes1
1Internal Medicine, Hospital of Braga, Braga, PRT.
Cureus
|January 13, 2023
Summary
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, a condition affecting small blood vessels, can impact multiple organs. Early diagnosis and immunosuppressive treatment, including cyclophosphamide and rituximab, are crucial for a positive prognosis in patients with granulomatosis with polyangiitis.
Area of Science:
- Rheumatology
- Nephrology
- Pulmonology
Background:
- Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis involves small and medium blood vessel inflammation.
- It predominantly affects the lungs and kidneys, with a complex pathogenesis.
Observation:
- A 55-year-old woman presented with constitutional symptoms, hearing loss, cough, and hemoptysis.
- Physical exam revealed tachypnea and diminished lung sounds; labs showed elevated inflammatory markers, acute kidney injury, and hematuria.
- Pulmonary CT identified peribronchial thickening, and ANCA-PR3 antibodies were positive.
Findings:
- The patient was diagnosed with granulomatosis with polyangiitis (GPA), a type of ANCA-associated vasculitis.
- Treatment involved corticosteroids, cyclophosphamide, and rituximab, leading to significant clinical and laboratory improvement.
Implications:
- This case highlights the importance of early clinical suspicion for GPA.
- Prompt initiation of immunosuppressive therapy is vital for achieving a favorable prognosis in ANCA-associated vasculitis.
- Multisystemic involvement (pulmonary, renal, cutaneous, ear) underscores the need for comprehensive patient evaluation.
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