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ANCA-negative Granulomatosis with Polyangiitis: A Difficult Diagnosis
Eilís McCarthy1, Muneeb Mustafa1, Mike Watts1
1Department of General Internal Medicine, University Hospital Limerick, Limerick, Ireland.
European Journal of Case Reports in Internal Medicine
|February 14, 2019
Summary
Granulomatosis with polyangitiis (GPA) can present with non-specific symptoms, delaying diagnosis. A negative anti-neutrophil cytoplasmic antibody (ANCA) test does not rule out GPA, especially in ANCA-negative cases.
Area of Science:
- Rheumatology
- Immunology
- Pathology
Background:
- Granulomatosis with polyangitiis (GPA) is a systemic vasculitis affecting small and medium vessels.
- It is often associated with anti-neutrophil cytoplasmic antibodies (ANCAs).
- Diverse and non-specific symptoms can delay GPA diagnosis.
Purpose of the Study:
- To highlight a case of GPA presenting with non-specific findings.
- To emphasize the diagnostic challenge posed by ANCA-negative GPA.
- To underscore the importance of considering GPA even with negative ANCA results.
Main Methods:
- Case report presentation.
- Review of diagnostic criteria for GPA.
- Discussion of ANCA testing in vasculitis.
Main Results:
- The described case of GPA presented with non-specific symptoms.
- Approximately 10-20% of GPA cases are ANCA-negative.
- Diagnosis can be missed in ANCA-negative patients with non-specific presentations.
Conclusions:
- A negative ANCA result does not exclude a diagnosis of GPA.
- Tissue biopsy remains a crucial diagnostic tool when vasculitis is suspected.
- Further investigations are warranted in suspected GPA, irrespective of ANCA status.
Keywords:
Granulomatosis with polyangiitis
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