Jean-Christophe Lega1, Raphaèle Seror2, Thomas Fassier3, Olivier Aumaître4, Isabelle Quere5, Jacques Pourrat6, Brigitte Gilson7, Agnes Sparsa8, Denis Wahl9, Claire Le Jeunne2, Olivier Decaux10, Luc Mouthon2, Alfred Mahr2, Pascal Cohen2, Loïc Guillevin2, Christian Pagnoux11. 1. Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, Paris, France; Department of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, 69495 Pierre-Bénite, Lyon, France. Electronic address: jean-christophe.lega@chu-lyon.fr. 2. Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, Paris, France. 3. Department of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, 69495 Pierre-Bénite, Lyon, France. 4. Department of Internal Medicine, Hôpital Gabriel-Montpied, CHU de Clermont-Ferrand, Clermont-Ferrand, France. 5. Department of Internal and Vascular Medicine, Hôpital Saint-Eloi, CHU de Montpellier France. 6. Department of Nephrology, Hôpital Rangueil, CHU de Toulouse, Toulouse, France. 7. Department of Internal Medicine, Centre Hospitalier de Verdun, Verdun, France. 8. Department of Dermatology, Hôpital Dupuytren, CHU de Limoges, Limoges, France. 9. Division of Internal and Vascular Medicine and Regional Competence Center for Rare Vascular And Systemic Autoimmune Diseases, CHU de Nancy; INSERM UMR_S 1116; Université de Lorraine, Nancy, France. 10. Department of Internal Medicine, Hôpital Sud, CHU de Rennes, Rennes, France. 11. Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, Paris, France; Department of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada.
Abstract
OBJECTIVES: To describe the prevalence, characteristics, and outcome of cutaneous ischemia, and whether it can occur in systemic necrotizing vasculitides (SNVs), i.e., polyarteritis nodosa, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis. METHODS: We conducted a retrospective analysis of all patients with SNV who were included in the French Vasculitis Study Group cohort database between March 1963 and September 2007. We compared characteristics and outcomes for patients with and without cutaneous ischemia (digital necrosis and/or isolated necrotic cutaneous ulcers). RESULTS: Among the 1304 patients with a diagnosis of SNVs, 40 (3.1%) had digital necrosis and 25 (1.9%) had isolated necrotic cutaneous ulcers, with an equal distribution among SNVs. Presence of cutaneous ischemia was associated with past and/or current smoking [odds ratio (OR), 1.73; 95% confidence interval (95% CI), 1.02-2.95] and history of coronary artery disease (2.40; 1.01-6.00), as well as with other cutaneous manifestations (6.54; 3.21-8.67), gastrointestinal tract perforations (4.29; 1.41-13.07), and arthralgias (1.84; 1.10-3.07) during diagnosis. Ten patients with digital necrosis underwent extremity amputation, but no patient with isolated necrotic cutaneous ulcers (p = 0.007) underwent it. Smoking was the main risk factor of amputation (OR, 9.1; 1.7-48.9). At a mean follow-up of 10 years, cutaneous ischemia was identified as an independent predictor of vasculitis relapse (hazard ratio, 1.47; 95% CI, 1.05-2.05) and all-cause death (1.66; 1.01-2.74). CONCLUSIONS: Cutaneous ischemia is a rare manifestation of SNVs but is associated with an increased risk of relapse and mortality. Tobacco use is associated with amputation, which emphasizes the importance of managing conventional cardiovascular risk factors in SNV patients.
OBJECTIVES: To describe the prevalence, characteristics, and outcome of cutaneous ischemia, and whether it can occur in systemic necrotizing vasculitides (SNVs), i.e., polyarteritis nodosa, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis. METHODS: We conducted a retrospective analysis of all patients with SNV who were included in the French Vasculitis Study Group cohort database between March 1963 and September 2007. We compared characteristics and outcomes for patients with and without cutaneous ischemia (digital necrosis and/or isolated necrotic cutaneous ulcers). RESULTS: Among the 1304 patients with a diagnosis of SNVs, 40 (3.1%) had digital necrosis and 25 (1.9%) had isolated necrotic cutaneous ulcers, with an equal distribution among SNVs. Presence of cutaneous ischemia was associated with past and/or current smoking [odds ratio (OR), 1.73; 95% confidence interval (95% CI), 1.02-2.95] and history of coronary artery disease (2.40; 1.01-6.00), as well as with other cutaneous manifestations (6.54; 3.21-8.67), gastrointestinal tract perforations (4.29; 1.41-13.07), and arthralgias (1.84; 1.10-3.07) during diagnosis. Ten patients with digital necrosis underwent extremity amputation, but no patient with isolated necrotic cutaneous ulcers (p = 0.007) underwent it. Smoking was the main risk factor of amputation (OR, 9.1; 1.7-48.9). At a mean follow-up of 10 years, cutaneous ischemia was identified as an independent predictor of vasculitis relapse (hazard ratio, 1.47; 95% CI, 1.05-2.05) and all-cause death (1.66; 1.01-2.74). CONCLUSIONS:Cutaneous ischemia is a rare manifestation of SNVs but is associated with an increased risk of relapse and mortality. Tobacco use is associated with amputation, which emphasizes the importance of managing conventional cardiovascular risk factors in SNV patients.