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Abstract Number: 2512

French Multicenter Retrospective Study of Patients with Cocaine- and/or Levamisole-induced Vasculitis and Vasculopathy

Kevin Chevalier1, François Chasset2, Yoann Zerbib3, Pierre-André Jarrot4, Romain Muller4, Thomas Pires5, Saroumadi Adavane6, Didier Bessis7, Alban Deroux8, Vincent Descamps9, Fanny Domont10, Joëlle Faddoul10, Pierre-Edouard gavand11, Tiphaine Goulenok12, Baptiste Gramont13, Vincent Jachiet14, Gaëlle Leroux15, Matthieu Mahevas16, Julie Merindol17, Loïc Meudec18, Martin Michaud19, Morgane Mourguet20, Héloïse Munos pons21, Yann Nguyen22, Bénédicte Oules23, Thomas Papo24, Jacques Pouchot25, Sébastien Rivière14, Oscar Robert26, Charles ronsin27, Benjamin Rossi28, Delphine Staumont-salle29, Benjamin Terrier30, Nathalie Tieule31, Vollmer Olivier32, Pierre Wolkenstein33, Xavier Puéchal34, Jean-David Bouaziz35, Nicolas Dupin23, Gilles Peytavin36, Jean-Claude Alvarez37, Luc Mouthon38 and Alexis Régent34, 1Department of Internal Medicine, National Reference Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France, Paris, France, 2Dermatology, Hôpital Tenon, Paris, France, 3Medical Intensive Care Unit and MP3CV-EA7517, Amiens University Hospital, Amiens, France, Amiens, France, 4Service de Médecine Interne et Immunologie Clinique, Hôpital de La Conception, Marseille, Marseille, France, 5Department of Internal Medicine, CHU Bordeaux, Bordeaux, France., Bordeaux, France, 6Service de Cardiologie, Hôpitaux Saint-Antoine and Tenon, AP-HP, 75012 Paris, France., Paris, France, 7Department of Dermatology and Reference Center for Rare Skin Diseases (MAGEC), Saint-Eloi Hospital, University of Montpellier and INSERM U1058, Montpellier, France., Montpellier, France, 8Clinique Universitaire de Médecine Interne, Department of Internal Medicine, Grenoble University Hospital, Grenoble, France, 9Department of Dermatology, APHP, Bichat Hospital, Paris, France., Paris, France, 10AP-HP. Centre de Référence des Maladies Auto-Immunes Systémiques Rares, Centre de Référence des Maladies Auto-Inflammatoires et de l'Amylose Inflammatoire, F-75013 Paris, France., Paris, France, 11Department of Internal Medicine, Clinique Rhena, Strasbourg, France., Strasbourg, France, 12Assistance Publique Hôpitaux de Paris, Paris, France, 13Internal Medicine Departement, Saint Etienne, Saint Etienne, France, 14Service de médecine interne, AP-HP, Hôpital Saint Antoine, Sorbonne Universite, Paris, France., Paris, France, 15Service de Médecine Interne, AP-HP Hôpital Pitié-Salpêtrière, Paris, France., Paris, France, 16Department of Internal Medicine, APHP, Mondor Hospital, Paris, France., Créteil, France, 17Internal Medicine Department, University Hospital of Nice, Côte d'Azur University, Nice, Provence-Alpes-Côte d'Azur, France., Nice, France, 18Department of Rheumatology, Université Paris-Saclay, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Le Kremlin Bicêtre, France., Kremlin Bicêtre, France, 19Department of Internal Medicine, Clinique Saint-Exupery, Toulouse, France., Toulouse, France, 20service de médecine interne CHU Toulouse RANGUEIL, Toulouse, France, 21Department of Internal Medicine, Saint-Etienne University Hospital, Saint-Etienne, France., Saint Etienne, France, 22Université Paris Saclay, Clichy, Ile-de-France, France, 23Dermatology Department, Cochin Hospital, Paris, France., Paris, France, 24Université Paris Cité, Paris, France, 25Service de médecine Interne, Centre National de Référence des syndromes drépanocytaires majeurs de l'adulte, AP-HP, Hôpital Européen Georges Pompidou, Paris, France, Paris, France, 26Groupe Hospitalier Pitié Salpêtrière, Assistance Publique Hopitaux de Paris, Service de Pneumologie et Réanimation Médicale, Paris, France; [email protected]., Paris, France, 27cHD Vendée, La Roche sur Yon, La Roche sur Yon, France, 28Service de médecine interne, maladies infectieuses, Centre hospitalier intercommunal de Robert Ballenger, Aulnay-sous-bois, France., Aulnay-sous-bois, France, 29Service de dermatologie, CHU de Lille, université de Lille, 59000 Lille, France., Lille, France, 30Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France, 31Rhumatologie, Centre Hospitalier Universitaire Nice, Hôpital Pasteur, Nice, France, 32Department of Immunology and Internal Medicine, Strasbourg University Hospital, Strasbourg, France., Strasbourg, France, 33Department of Dermatology, National Referral Center for Neurofibromatosis, Henri Mondor University Hospital, Assistance Publique- Hôpitaux de Paris, Creteil, France., Créteil, France, 34National Referral Center For Rare Systemic Autoimmune Diseases, Paris, France, 35Dermatology, Hopital Saint-Louis, Paris, Île-de-France, France., Paris, France, 36Service de Pharmacologie, Université Paris Cité, INSERM, IAME, UMR 1137, AP-HP, Hôpital Bichat-Claude Bernard, Paris, France., Paris, France, 37Service de Pharmacologie‑Toxicologie, Hôpital Raymond Poincaré, Groupe Hospitalier Universitaires AP-HP.Paris-Saclay, Garches, 92380, France, Paris, France, 38Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes et Autoinflammatoires Rares d'Ile de France de l’Est et de l’Ouest, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France

Meeting: ACR Convergence 2024

Keywords: ANCA, skin, Vasculitis

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Session Information

Date: Monday, November 18, 2024

Title: Vasculitis – Non-ANCA-Associated & Related Disorders Poster III

Session Type: Poster Session C

Session Time: 10:30AM-12:30PM

Background/Purpose: Levamisole, an imidazothiazole formerly used as an anthelmintic, is also used as a cutting agent to enhance the effects and duration of cocaine. In recent years, levamisole has been implicated in cases of vasculitis and/or thrombotic vasculopathy, particularly in the context of adulterated cocaine use. In addition, cocaine itself has significant vascular effects. We decided to describe a large series of patients with vasculopathy/vasculitis induced by levamisole and/or cocaine.

Methods: We conducted a retrospective multicenter observational study in France. Patients were recruited by a call for observations on behalf of the French Vasculitis Study Group (FVSG) and by screening data from toxicology laboratories.

Patients were included if they had 1) a documented or reported cocaine use regardless of the type of use and 2) vasculitis and/or vasculopathy symptoms after exclusion of other causes, ANCA positivity was not an exclusion criterion. We collected data using a standardized form, including age, sex, past medical history, clinical manifestations, biological and immunological characteristics, pathological data, treatment and outcome.

Results: A total of 63 patients were included, most of them male (58.7%), with a median age of 40 [35-47] years. The main characteristics of the patients are shown in Table 1. The main manifestations were skin (n=34, 54%) with purpura (n=13, 38.2%), necrosis (n=17, 50%) and erythematous plaques (n=14, 41.2%) mainly localized to the extremities, ear-nose and throat (ENT) symptoms (n=40, 63.5%) including sinusitis (n=27, 67.5%), nasal crusts (n=27, 67.5%), epistaxis (n=16, 40%) and saddle nasal deformity (n=28, 70%).

Patients with a positive ANCA test by ELISA (ANCA+) including 16 patients with PR3-ANCA, 3 with MPO-ANCA and 7 with both, were more likely to have ENT involvement than patients with a negative ANCA test (ANCA-) (Table 2). Of the 40 patients (63.5%) with histological results, ENT (n=20, 50%) and skin (n=18, 45%) were the preferred biopsy sites. Necrosis (n=13, 65%) and granuloma (n=6, 20%) were commonly described in ENT biopsies, whereas vasculitis (n=9, 50%) and thrombosis (n=6, 33%) were commonly identified in skin biopsies.

At disease onset, 24 patients (38.1%) were able to stop using cocaine. Of the 63 patients, 24 patients (38.1%) received glucocorticoids, 9 patients (14.3%) received methotrexate and 8 patients (12.7%) received rituximab. Treatment details and therapeutic sequences are summarized in Figure 1.

At the last follow-up, 37 patients (58.7%) were in remission and 7 (11.1%) had died. Twenty-four patients (38.1%) had relapsed. Patients who had been weaned from cocaine were more likely to be in remission (p< 0.0001) and had significantly fewer relapses (p=0.04) than other patients.

Conclusion: Cocaine/levamisole-induced vasculitis/vasculopathy frequently affects the skin (with a histological pattern of vasculitis and thrombosis) and ENT (with a histological pattern of necrosis). ANCA positivity does not appear to influence the clinical phenotype or the clinical course of the disease. Cocaine withdrawal significantly improves the prognosis of these patients.

Supporting image 1

Table 1 – Clinical characteristics of patients with cocaine/levamisole induced vasculitis/vasculopathy at the time of diagnosis

Supporting image 2

Table 2 – Comparison of main characteristics between patients with ANCA in ELISA (ANCA+) and patients without ANCA (ANCA-)

Supporting image 3

Figure 1 – Sunburst representation of treatment combinations at disease onset (baseline) or at first and second relapse according to disease severity
Patients’ manifestations were classified according to their functional or life-threatening consequences as minor organ involvement (i.e. skin, ear-nose-throat or musculoskeletal) or major organ involvement (i.e. lung, kidney, peripheral or central nervous system and heart).
DMARD stands for disease-modifying anti-rheumatic drugs and includes azathioprine, methotrexate, and mycophenolate mofetil. IS stands for immunosuppressive treatments and includes cyclophosphamide and rituximab.
GC: glucocorticoids


Disclosures: K. Chevalier: None; F. Chasset: AstraZeneca, 1, 2, 4, 5, Biogen, 12, National coordinator of clinical trial, BMS, 6, 12, National coordinator of clinical trial, GSK, 1, 4, 5, HorizonTherapeutics, 1, 4, Merck, 1, 4, PrincipaBio, 1; Y. Zerbib: None; P. Jarrot: None; R. Muller: None; T. Pires: None; S. Adavane: None; D. Bessis: None; A. Deroux: None; V. Descamps: None; F. Domont: None; J. Faddoul: None; P. gavand: None; T. Goulenok: None; B. Gramont: None; V. Jachiet: None; G. Leroux: None; M. Mahevas: None; J. Merindol: None; L. Meudec: None; M. Michaud: None; M. Mourguet: None; H. Munos pons: None; Y. Nguyen: None; B. Oules: None; T. Papo: None; J. Pouchot: None; S. Rivière: None; O. Robert: None; C. ronsin: None; B. Rossi: None; D. Staumont-salle: None; B. Terrier: AstraZeneca, 2, GlaxoSmithKline, 2, Novartis, 2, Vifor Pharma, 2; N. Tieule: None; V. Olivier: None; P. Wolkenstein: None; X. Puéchal: None; J. Bouaziz: None; N. Dupin: None; G. Peytavin: None; J. Alvarez: None; L. Mouthon: None; A. Régent: None.

To cite this abstract in AMA style:

Chevalier K, Chasset F, Zerbib Y, Jarrot P, Muller R, Pires T, Adavane S, Bessis D, Deroux A, Descamps V, Domont F, Faddoul J, gavand P, Goulenok T, Gramont B, Jachiet V, Leroux G, Mahevas M, Merindol J, Meudec L, Michaud M, Mourguet M, Munos pons H, Nguyen Y, Oules B, Papo T, Pouchot J, Rivière S, Robert O, ronsin C, Rossi B, Staumont-salle D, Terrier B, Tieule N, Olivier V, Wolkenstein P, Puéchal X, Bouaziz J, Dupin N, Peytavin G, Alvarez J, Mouthon L, Régent A. French Multicenter Retrospective Study of Patients with Cocaine- and/or Levamisole-induced Vasculitis and Vasculopathy [abstract]. Arthritis Rheumatol. 2024; 76 (suppl 9). https://acrabstracts.org/abstract/french-multicenter-retrospective-study-of-patients-with-cocaine-and-or-levamisole-induced-vasculitis-and-vasculopathy/. Accessed .
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