Pierre-Edouard Gavand1, Ilaria Serio2, Laurent Arnaud3, Nathalie Costedoat-Chalumeau4, Julien Carvelli5, Antoine Dossier6, Olivier Hinschberger7, Luc Mouthon8, Véronique Le Guern4, Anne-Sophie Korganow9, Vincent Poindron9, Clément Gourguechon10, Christian Lavigne10, François Maurier11, Guylaine Labro12, Marie Heymonet13, Matthieu Artifoni14, Amélie Brabant Viau15, Cristophe Deligny16, Thomas Sene17, Louis Terriou18, Jean Sibilia3, Alexis Mathian19, Coralie Bloch-Queyrat20, Claire Larroche21, Zahir Amoura19, Thierry Martin9. 1. Service d'immunologie Clinique et médecine interne, CHU de Strasbourg, France. Electronic address: pierreedouard.gavand@chru-strasbourg.fr. 2. Division of Internal Medicine, Department of Medical & Surgical Sciences, University of Bologna, S Orsola-Malpighi Hospital, Bologna, Italy. 3. Service de rhumatologie, CHU Strasbourg, France. 4. AP-HP, Cochin Hospital, Internal Medicine Department, Centre de référence maladies auto-immunes et systémiques rares, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France; INSERM U 1153, Center for Epidemiology and Statistics Sorbonne Paris Cité (CRESS), Paris, Hôpital Cochin, France. 5. Service de médecine interne et d'immunologie clinique, CHU Conception Marseille - APHM, France. 6. Service de médecine interne CHU Bichat, Paris, Université paris VII, France. 7. Service de médecine interne, CH Mulhouse, France. 8. Service de médecine interne, hôpital Cochin, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, DHU Authors (Autoimmune and Hormonal Diseases), Université Paris Descartes, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France. 9. Service d'immunologie Clinique et médecine interne, CHU de Strasbourg, France. 10. Service de médecine interne, CHU Amiens, France. 11. Service de médecine Interne et Immunologie Clinique, HP Metz Site Belle Isle, France. 12. Service de médecine interne, CHU Besançon, France. 13. CHU de Nancy, Internal Medicine and Clinical immunology Department, France. 14. Service de médecine interne, CHU Nantes, France. 15. Service de médecine interne, CHU Reims, France. 16. Service de médecine interne, CHU Fort-de-, France. 17. Service de médecine Interne, Groupe Hospitalier Diaconesses, Croix Saint-Simon, Paris, France. 18. CHU Lille, Département de Médecine Interne et Immunologie Clinique, F-59000 Lille, France. 19. Service de médecine interne 2, Groupement hospitalier La Pitié-Salpétrière, Paris, France. 20. URC CRC Hopital Avicenne, Bobigny, France. 21. Service de médecine interne, CHU Avicennes, Bobigny, France.
Abstract
OBJECTIVES: Macrophage activation syndrome (MAS) is a life-threatening hyperinflammatory syndrome that can occur during systemic lupus erythematosus (SLE). Data on MAS in adult SLE patients are very limited. The aim of this study is to describe the clinical characteristics, laboratory findings, treatments, and outcomes of a large series of SLE-associated MAS. METHODS: We conducted a retrospective study that included 103 episodes of MAS in 89 adult patients with SLE. RESULTS: 103 episodes in 89 adult patients were analyzed. Median age at first MAS episode was 32 (18-80) years. MAS was inaugural in 41 patients (46%).Thirteen patients relapsed. Patients had the following features: fever (100% episodes), increased serum levels of AST (94.7%), LDH (92.3%), CRP (84.5%), ferritin (96%), procalcitonin (41/49 cases). Complications included myocarditis (n=22), acute lung injury (n=15) and seizures (n=11). In 33 episodes, patients required hospitalization in an ICU and 5 died. Thrombocytopenia and high CRP levels were associated independently with an increased risk for ICU admission. High dose steroids alone as first line therapy induced remission in 37/57 cases (65%). Additional medications as first or second line therapies included IV immunoglobulins (n=22), cyclophosphamide (n=23), etoposide (n=11), rituximab (n=3). Etoposide and cyclophosphamide-based regimens had the best efficacy. CONCLUSION: MAS is a severe complication and is often inaugural. High fever and high levels of AST, LDH, CRP, ferritin and PCT should be considered as red flags for early diagnosis. High dose steroids lead to remission in two third of cases. Cyclophosphamide or etoposide should be considered for uncontrolled/severe forms.
OBJECTIVES:Macrophage activation syndrome (MAS) is a life-threatening hyperinflammatory syndrome that can occur during systemic lupus erythematosus (SLE). Data on MAS in adult SLEpatients are very limited. The aim of this study is to describe the clinical characteristics, laboratory findings, treatments, and outcomes of a large series of SLE-associated MAS. METHODS: We conducted a retrospective study that included 103 episodes of MAS in 89 adult patients with SLE. RESULTS: 103 episodes in 89 adult patients were analyzed. Median age at first MAS episode was 32 (18-80) years. MAS was inaugural in 41 patients (46%).Thirteen patients relapsed. Patients had the following features: fever (100% episodes), increased serum levels of AST (94.7%), LDH (92.3%), CRP (84.5%), ferritin (96%), procalcitonin (41/49 cases). Complications included myocarditis (n=22), acute lung injury (n=15) and seizures (n=11). In 33 episodes, patients required hospitalization in an ICU and 5 died. Thrombocytopenia and high CRP levels were associated independently with an increased risk for ICU admission. High dose steroids alone as first line therapy induced remission in 37/57 cases (65%). Additional medications as first or second line therapies included IV immunoglobulins (n=22), cyclophosphamide (n=23), etoposide (n=11), rituximab (n=3). Etoposide and cyclophosphamide-based regimens had the best efficacy. CONCLUSION: MAS is a severe complication and is often inaugural. High fever and high levels of AST, LDH, CRP, ferritin and PCT should be considered as red flags for early diagnosis. High dose steroids lead to remission in two third of cases. Cyclophosphamide or etoposide should be considered for uncontrolled/severe forms.