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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References

  • Levine SM, Stone JH. Pathogenesis of granulomatosis with polyangitiis and related vasculitides. http://cursoenarm.net/UPTODATE/contents/mobipreview.htm?9/20/9537 (accessed 5 January 2017).
  • Falk RJ, Jennette JC. ANCA small-vessel vasculitis. J Am Soc Nephrol 1997;8:314–122.
  • Tracy CL, Papadopoulos PJ. Granulomatosis with polyangiitis – overview. http://emedicine.medscape.com/article/332622 (accessed 6 January 2017).
  • Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 1990;33:1101–1107.m/art
  • Fauci AS, Haynes BF, Katz P. The spectrum of vasculitis: clinical, pathologic, immunologic, and therapeutic considerations. Ann Intern Med 1978;89:660–676.

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    Published: 2017-10-30
    Issue: Vol. 4 No. 8 (view)

    How to cite:
    1.
    McCarthy E, Mustafa M, Watts M. ANCA-negative Granulomatosis with Polyangiitis: A Difficult Diagnosis. EJCRIM 2017;4 doi:10.12890/2017_000625.

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