ANCA-negative Granulomatosis with Polyangiitis: A Difficult Diagnosis

  • Eilis McCarthy Department of General Internal Medicine, University Hospital Limerick, Limerick
  • Muneeb Mustafa Department of General Internal Medicine, University Hospital Limerick, Limerick
  • Mike Watts Department of General Internal Medicine, University Hospital Limerick, Limerick
Keywords: Granulomatosis with polyangitis

Abstract

Granulomatosis with polyangitiis (GPA) is a systemic small and medium vessel vasculitis, commonly associated with anti-neutrophil cytoplasmic antibodies (ANCAs). Presenting signs and symptoms in GPA are varied and patients may present with constitutional, non-specific symptoms, which can delay the diagnosis. Tissue biopsy of the site of active disease can confirm the diagnosis of GPA, in which necrotising granulomatous inflammation is seen. However, surrogate markers may be used for diagnosis without a tissue biopsy. They include upper and lower airway symptoms, signs of glomerulonephritis and a positive ANCA. However, approximately 10–20% of patients with GPA are ANCA negative, allowing for the diagnosis to be overlooked, particularly in those patients with non-specific findings. The reason for the absence of ANCAs is unclear.

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  • Published: 2017-10-30

    Issue: Vol 4 No 8 (view)

    Section: Articles

    How to cite:
    McCarthy, E., Mustafa, M., & Watts, M. (2017, October 30). ANCA-negative Granulomatosis with Polyangiitis: A Difficult Diagnosis. European Journal of Case Reports in Internal Medicine, 4(8). https://doi.org/https://doi.org/10.12890/2017_000625