A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the Literature

Abstract

This paper describes one patient with Antineutrophil Cytoplasmic Antibody- (ANCA-) associated vasculitis who initially presented with multiple ischemic fingers and toes. On further evaluation, the patient was also found to have pulmonary-renal involvement and episcleritis. The diagnosis was supported with a positive cANCA (anti-proteinase 3) and a bronchoscopy consistent with diffuse alveolar hemorrhage. Although the patient refused a tissue biopsy, clinical presentation including nasal ulceration, sinus congestion, and epistaxis and anti-proteinase 3 antibody were more consistent with Granulomatosis with Polyangiitis (GPA) rather than Microscopic Polyangiitis (MPA) or Eosinophilic Granulomatosis with Polyangiitis (EGPA) based on the recently presented ACR/EULAR Provisional 2017 Classification Criteria for GPA (Luqmani et al., 2016). The patient responded well to therapy including high dose steroids and cyclophosphamide, with improvement of all organs involved and had no further digital ischemia or gangrene on follow-up. We include a review of the English literature summarizing presentation, management, and outcome of 16 similar cases.

Rare presentation of granulomatosis with polyangiitis

“…Lau et al presented a case of GPA with digital ischaemia along with a literature review of approximately 16 cases with similar presentation 7. The suspected prevalence of digital ischaemia and gangrene in the GPA population is <1% 7. Given this statistic, along with the presentation of GPA and a wide array of possible organ involvement, this diagnosis can be difficult.…”

An Unusual Presentation of Granulomatosis with Polyangiitis (GPA)

“…Cutaneous manifestations occur in 20% of patients with GPA, which usually includes papulonecrotic, pyoderma gangrenosum and sometimes generalized urticarial lesions [ 1 – 3 ]. There have been only a few cases reported of GPA presenting with digital ischemia as an initial manifestation [ 4 – 6 ].…”

“…Given the paucity of cases reported, the prevalence of digital ischemia and gangrene in the GPA population is suspected to be <1% [ 7 – 10 ]. The pathophysiology of digital ischemia and gangrene is thought to be from destruction of medium-sized vessels due to active vasculitis [ 1 , 2 ]. However, there is also evidence that in situ thrombosis, as a result of active vasculitis, can lead to ischemia and gangrene.…”

“…Granulomatosis with polyangiitis (formerly Wegner’s granulomatosis) is a small-to-medium vessel vasculitis which affects multiple organs. Pathologically, there is a focal necrotizing vasculitis involving the small arteries and veins of the ear, nose, and throat, the lungs, the kidneys, the heart, the nervous system, the eyes, the skin, and, infrequently, other organs [ 1 – 3 ]. Involvement of digital arteries causing ischemia or gangrene is very rare.…”

Acrocyanosis and digital necrosis are associated with poor prognosis in COVID-19

“…Furthermore, acrocyanosis has been described in critically ill patients with COVID-19 because of excessive coagulation status (9). Indeed, acrocyanosis and digital necrosis have previously been reported in many rheumatologic disorders and vasculitis, including ANCA-associated vasculitis, cryoglobulinemia vasculitis, lupus-related vasculitis, vasculopathy caused by APS, and also fingertips necrosis due to scleroderma vascular involvement as well as Raynaud's phenomenon (10)(11)(12). From a physiologic point of view, gangrene may occur by impaired blood flow and insufficient healing process of digital wounds which is associated with the increasing levels of C-reactive protein (CRP) (11).…”