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Response to: ‘Efficacy and improved tolerability of combination therapy with interleukin-1 blockade and MAPK pathway inhibitors for the treatment of Erdheim-Chester disease’ by Campochiaro et al
  1. Fleur Cohen-Aubart1,
  2. Neila Benameur2,
  3. Zahir Amoura1,
  4. Julien Haroche1
  1. 1Sorbonne Université, Assistance Publique Hôpitaux de Paris, Service de Médecine Interne 2, Hôpital de la Pitié-Salpêtrière, Centre National de Référence Lupus Systémique et Histiocytoses, Paris, France
  2. 2Assistance Publique Hôpitaux de Paris, Département de Pharmacie, Hôpital de la pitié-Salpêtrière, Paris, France
  1. Correspondence to Dr Fleur Cohen-Aubart, AP-HP, Hôpital de la Pitié-Salpêtrière, Service de Médecine Interne 2, Sorbonne Université Faculté de Médecine, Paris, Île-de-France 75013, France; fleur.cohen{at}

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We read with interest the letter by Campochiaro et al1 that reported the efficacy of a combination regimen including anakinra and targeted therapy for the treatment of Erdheim-Chester disease (ECD), a rare multisystem inflammatory histiocytosis. Based on the involvement of inflammatory cytokines, including tumour necrosis factor-alpha and interleukin-1, in the pathophysiology and the clinical manifestations of ECD, previous reports demonstrated a variable efficacy with a good tolerance of daily subcutaneous anakinra and infliximab for the treatment of ECD.2 3 Among 262 patients with ECD who have been seen at our institution until 2019, 31 (12%) (including 12 described in a previous analysis2) were treated with 100 mg (n=27) or 200 mg (n=4) daily of anakinra, with a mean duration of 29 months (range, 1–132). Patients were followed on a regular basis and evaluated as previously described.4 The clinical and molecular details are provided in table 1. Patients treated with anakinra had more osseous and less central nervous system (CNS) involvements. The patients were treated with anakinra alone or in combination with steroids in two patients, and interferon-alpha in one patient. In the …

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