Case Report: An Uncommon Presenting Complaint of GPA

Patients with digital limb ischemia as the presenting symptom of GPA may have delayed diagnosis and treatment. With the rapid progression to multiple-organ involvement, early diagnosis and treatment are imperative in this patient population. Moreover, reported mortality rates of GPA are highest in the first six months of disease, and without correct and prompt treatment, digital limb ischemia can lead to severe disability.¹⁰

In Sum

We describe a 71-year-old woman who presented with digital limb ischemia as the primary complaint for GPA. Digital limb ischemia and gangrene are much more commonly found with a medium vessel vasculitis and have rarely been associated with GPA, with an estimated incidence of <1%. With the mortality rates of GPA being highest in the first six months of diagnosis, patients must be diagnosed and treated promptly. Thus, it is important to bring awareness to digital limb ischemia and gangrene as an associated, but rare, presenting cutaneous symptom of GPA.

Adina Greene, BA, is a third-year medical student at the University of Arizona College of Medicine, Phoenix, and a dermatology research fellow at Mayo Clinic Arizona.

Sabrina Dahak, MD, is a preliminary internal medicine intern at the University of Arizona College of Medicine, Phoenix, and will start her dermatology residency in July 2024 at Washington University in St Louis – Division of Dermatology.

Wesley A. Robertson, MD, graduated medical school from the University of Arizona College of Medicine, Tucson. He received his masters in public health and public policy from the University of Wisconsin. A current third-year resident at the University of Arizona College of Medicine, Phoenix, he is a future gastroenterologist.

Denege Ward-Wright, MD, graduated from Wayne State University School of Medicine, Detroit. She is board certified in internal medicine and a former assistant clinical professor at the University of Michigan, where she practiced primary care and hospital medicine. She is currently the associate clinical professor of internal medicine and associate chair of justice equity diversity and inclusion in the Department of Medicine at the University of Arizona College of Medicine, Phoenix.

Kristen Young, DO, MEd, is a clinical assistant professor of medicine and rheumatologist at the University of Arizona College of Medicine, Phoenix, and Banner-University Medical Center, Phoenix. Her research interests include medical education, interstitial lung diseases, access to care and patient-related outcomes in rheumatology.

References

1. Godman GC, Churg J. Wegener’s granulomatosis: Pathology and review of the literature. AMA Arch Pathol. 1954 Dec;58(6):533–553.
2. Lau RA, Bains R, Suraweera D, et al. A rare case of digital ischemia and gangrene in ANCA-associated vasculitis with review of the literature. Case Rep Rheumatol. 2017;2017:2421760.
3. Hines A, Bello V, Iftikhar A, Zein H. Rare presentation of granulomatosis with polyangiitis. BMJ Case Rep. 2019 Feb 22;12(2):e227218.
4. Pokharel S, Singh S, Shaukat MS. An unusual presentation of granulomatosis with polyangiitis (GPA). Am J Case Rep. 2018 Sep 7;19:1071–1073.
5. Lim SY, Lim JH, Horn C. Upper-extremity digital ischemia in granulomatosis with polyangiitis. J Clin Rheumatol. 2014 Apr;20(3):155–159.
6. DeRemee RA, McDonald TJ, Harrison EG Jr., Coles DT. Wegener’s granulomatosis: Anatomic correlates, a proposed classification. Mayo Clin Proc. 1976;51(12):777–781.
7. Young C, Beynon H, Haskard D. Buerger’s disease (thromboangiitis obliterans): A reversible cause of upper limb digital infarcts. Rheumatology (Oxford). 2000 Apr;39(4):442–443.
8. Modi M, Vats AK, Prabhakar S, et al. Acroosteolysis and mononeuritis multiplex as a presenting symptom of systemic angiitis of Wegener’s type. Indian J Med Sci. 2007 Apr;61(4):212–215.
9. Koike H, Nishi R, Ohyama K, et al. ANCA-associated vasculitic neuropathies: A review. Neurol Ther. 2022 Mar;11(1):21–38. 10. Lin CY, Chen HA, Chang TW, et al. Time-dependent risk of mortality and endstage kidney disease among patients with granulomatosis with polyangiitis. Front Med (Lausanne). 2022 Aug 10;9:817204.