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

Table 1

Literature review of digital ischemia and gangrene associated with GPA in adults.

Age/sex	Onset after initial GPA symptoms	cANCA	Biopsy status	Other organ systems involved	Management	Outcome	Reference
30/M	3-4 months	Positive	Skin: nonspecific chronic inflammation; Renal: segmental necrotizing GN	Sinusitis, epistaxis, arthralgias, painless oral ulcers, active urine sediment, mild renal insufficiency (creat. 2.3 mg/dL), and left upper lung lobe fibrotic infiltrates	Cyclophosphamide and steroids	Gangrene formation with autoamputation	Handa and Wali [7]
80/M	1-2 years	Positive	Skin: leukocytoclastic vasculitis	Multiple lung opacities with incomplete cavitation, renal insufficiency (creat. 1.5 mg/dL), microscopic hematuria, sensorineural hearing loss, and nasal ulcers	Cyclophosphamide and steroids	Gangrene formation, improvement of reversible tissue ischemia	La Civita et al. [8]
68/M	2 years	Not available	Sural nerve: vasculitis with secondary demyelination; Lung: nodular necrotizing granulomatous inflammation with vasculitis	Purulent sinusitis, renal insufficiency (creat. 1.7 mg/dL), right peroneal neuropathy, otitis media, right 7th nerve palsy, pulmonary nodules, left pleural effusion, and pyoderma gangrenosum	Cyclophosphamide and steroids	Gangrene formation with surgical debridement and amputation	Phillips and Twiest [9]
26/F	1-2 years	Positive	Renal: focal crescentic GN; Nasopharyngeal: small artery vasculitis	Secretory otitis, conjunctivitis, tonsillitis, arthritis, hematuria, mild renal insufficiency, mucosal ulcerations, and palpable purpura	Not specified	Gangrene formation	Karjalainen and Hakala [10]
48/M	3 months	Positive	Renal: pauci immune GN	Acute kidney injury (creat. 4.6 mg/dL), nasal crusting, hemoptysis, right sided pleuritic chest pain, and active urine sediment	Cyclophosphamide, steroids, heparin, aspirin, calcium channel blockers, nitroglycerin ointment, and plasmapheresis	Gangrene formation with autoamputation	Lim et al. [11]
24/F	On presentation	Positive	Femoral artery: normal	Pulmonary infiltrates, splenic infarcts, hemoptysis, and nodular masses in bilateral lungs with diffuse interstitial lung disease	Cyclophosphamide, steroids, heparin, and thrombectomy	Gangrene formation with surgical amputation	Bessias et al. [12]
55/M	3-4 months	Negative	Nasopharyngeal: epithelial ulceration with vasculitis	Epistaxis, polyarthralgia, bilateral lung nodular densities with cavitations, and small pleural effusion	Cyclophosphamide, steroids, warfarin, thrombectomy, and bypass	Improvement of reversible tissue ischemia	Maia et al. [13]
58/M	Unclear	Not available	Renal: 30% crescents	ENT, sinusitis, bronchiectasis, lung infiltrates, renal insufficiency (creat. 4.7 mg/dL), eye, and joint pain	Immunosuppression (not specified)	Gangrene formation	Pinching et al. [14]
40/M	Unclear	Not available	Renal: granuloma and vasculitis with 30% crescents; Respiratory tract: vasculitis	ENT, lung infiltrates, renal failure requiring hemodialysis (creat. 18.43 mg/dL), joint pain, eye, vasculitic skin rash, and mononeuritis multiplex	Immunosuppression (not specified)	Gangrene formation	Pinching et al. [14]
39/F	On presentation	Positive	Lung: patchy nodular fibrotic changes with hemosiderin laden macrophages and diffuse alveolar hemorrhage; Left 4th gangrene digit: vasculitic changes with no thrombotic phenomenon	Hemoptysis, decline in respiratory function, and bilateral pulmonary infiltrates	Cyclophosphamide, steroids, and plasmapheresis	Gangrene formation with surgical amputation, improvement of reversible tissue ischemia	Leung et al. [15]
45/M	2 years	Positive	Renal: necrotizing GN	Polyneuropathy, renal injury, arthralgia, mild bloody nasal discharge, and skin vasculitis	Cyclophosphamide, steroids, heparin, aspirin, and iloprost	Improvement of reversible tissue ischemia	Schmidt et al. [16]
41/M	2 years	Positive	Sinus: granulomatous vasculitis	Bloody nasal discharge, inner ear granuloma, polyneuropathy, pulmonary infiltrates, and destructive sinusitis	Cyclophosphamide, steroids, heparin, aspirin, and iloprost	Improvement of reversible tissue ischemia	Schmidt et al. [16]
49/M	On presentation	Positive	Sural nerve: axonal loss	Mononeuritis multiplex with left foot drop, and cavitary nodule in the lung	Cyclophosphamide, steroids	Acroosteolysis, improvement of reversible ischemia	Modi et al. [17]
61/F	4 months	Positive	Renal: focal segmental necrotizing GN	Joint pain, Raynaud’s, and acute kidney injury	Cyclophosphamide, steroids	Gangrene formation with autoamputation	Bartsch et al. [18]
46/F	2 months	Positive	Skin: leukocytoclastic vasculitis	Polyarthralgias, epistaxis, sinusitis, mouth ulcers, episcleritis, mononeuritis multiplex, multiple nodules in right lung, and active urine sediment	Cyclophosphamide, steroids, and iloprost	Gangrene formation	Kejriwal et al. [19]
26/M	On presentation	Positive	Skin: superficial dermal blood vessel necrosis	Oligoarthritis, polydipsia, polyuria, lung nodules, and mucosal thickening of maxillary sinus	Cyclophosphamide, steroids, and azathioprine	Gangrene formation with autoamputation	Agarwal and Khan [20]
62/M	On presentation	Positive		Nasal ulceration and congestion, epistaxis, episcleritis, hemoptysis, diffuse alveolar hemorrhage, pulmonary infiltrates, joint pain, renal insufficiency (creat. 1.7), and active urine sediment	Cyclophosphamide, steroids	Improvement of reversible ischemia, gangrene formation with autoamputation	Current case

M: male; F: female; ENT: ear, nose, and throat; GN: glomerulonephritis; IV: intravenous.