Malignant Peritoneal Mesothelioma Complicating FamilialMediterranean Fever on 18F-FDG PET/CT

Antoine Fayand, MD,* Khaldoun Kerrou, MD,† Dominique Wendum, MD, PhD,‡

Gilles Grateau, MD, PhD,* and Sophie Georgin-Lavialle, MD, PhD*

Abstract:

A 77-year-old man with a personal history of familial Mediterranean fever presented with a slowly enlarging tumefaction of the left abdominal wall and persistent inflammatory syndrome despite good adherence to colchicine. 18F-FDG PET/CT showed a hypermetabolic muscular mass of the abdominal wall along with other hypermetabolic lesions including a peritoneal mass and several subcutaneous soft tissue nodules.

CT-guided needle biopsy led to the diagnosis of a muscular localization of a malignant peritoneal mesothelioma, which is an extremely rare complication of familial Mediterranean fever. Six courses of chemotherapy with carboplatin and pemetrexed allowed an almost complete response.

Key Words: malignant peritoneal mesothelioma, familial Mediterranean fever, autoinflammatory disorders, 18F-FDG PET/CT.

Efficacy of canakinumab for mosaic tumor necrosis factor receptor associated periodic syndrome

Alexandre Terréa,1, Mathieu Vautierb,1, Jean-Emmanuel Kahnc,

Sophie Georgin-Laviallea,2,*, Guilaine Boursierd,2

Introduction:

Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) is one of the first monogenic autoinflammatory diseases to be described [1]. TRAPS is a dominant disease due to mutations in the TNFRSF1A gene and affects patients from all regions of the world [2].

The main clinical features are prolonged recurrent inflammatory flares associated with fever, abdominal pain, myalgia and oedema. Most patients have a germline mutation, but 4 patients with somatic (i.e.post-zygotic) mutations have been described [3–6]. Canakinumab, a monoclonal anti-interleukin-1 antibody, has been reported to be effective in preventing attacks and controlling inflammation in germinal TRAPS. Indeed, canakinumab is the only molecule that has shown efficacy in a randomized trial [7]. One case associated with somatic mutation reported efficacy of canakinumab for somatic TRAPS [4]. Here we report the efficacy of canakinumab for the treatment of 2 patients followed in the French referral center for adult auto-inflammatory disease (CEREMAIA).

https://doi.org/10.1016/j.ejim.2024.01.034

Received 11 December 2023; Received in revised form 22 January 2024; Accepted 25 January 2024

0953-6205/© 2024 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.