Author: Dálifer Freites Núñez
Brown et al. (0280) characterized the largest cohort of patients suffering from Susac Syndrome, a rare autoimmune condition causing microvascular occlusions in the brain, retina and inner ear leading to the characteristic triad of encephalopathy, branch retinal artery occlusion (BRAO) and sensorineural hearing loss potentially leading to permanent disability and death if untreated. 60 patients met the European Susac Consortium criteria. The triad of encephalopathy with MRI changes of the corpus callosum, BRAO and hearing loss documented with audiometry were present in 65% of cases. The average age was 33 years of age and females 73% of patients. They conclude flares of disease were common over the first two years of follow up. These preliminary data suggest Rituximab may be superior to MMF in maintaining remission.
Kuemmerle-Deschner et al. (0275) investigated in a real-world setting the infections and infection rates in patients with cryopyrin-associated periodic syndromes, familial Mediterranean fever, hyper-IgD syndrome/mevalonate kinase deficiency and TNF receptor-associated periodic syndrome on Canakinumab. Data from a total of n=232 patients including n=101 (44%) pediatric patients under 18 years diagnosed with autoinflammatory diseases enrolled in the RELIANCE registry. During the study, infections occurred in 54.5% of patients (55 patients). Interim data of the RELIANCE study confirm that in the paediatric cohort the risk of infections including upper respiratory tract infections does not accumulate over 4 years under CAN treatment.
Allen et al. (0760) presented the treatment patterns and outcomes in patients with MAS secondary to Stills disease and treated with Emapalumab. A retrospective medical chart review was conducted across 33 hospitals. Of the 105 patients, 10 had Stills disease. The majority of patients achieved normal levels of ferritin (5/10), fibrinogen (6/10), platelets (8/10), alanine transaminase (8/10), absolute neutrophil count (9/10), and absolute lymphocyte count (9/10). The median time to first normalization of these laboratory parameters ranged from 7 to 46 days. Overall survival and 12-month survival probability following Emapalumab initiation was 90% for patients with Stills disease.
López et al. (1154) evaluated the clinical characteristics of patients diagnosed with IgG4-RD in a University Hospital as well as compared it with other large series. 12 patients (8 females), mean age 62 years. The organs affected at diagnosis were: aorta (n=5), pleura/lung (n=5), lymph nodes (n=4), salivary glands (n=2), retroperitoneum (n=2), pericardium (n=2), lacrimal glands (n=1), bile duct (n=1), kidney (n=1), orbit (n=1), subglottis (n=1), mesentery (n=1), maxillary sinuses (n=1). The pancreas was one of the most frequently involved. In contrast, in our series, aortic involvement and lung/pleura were the most frequent.
ABOUT THE AUTHOR
Dálifer Freites Núñez
Dálifer Freites Núñez, Rheumatologist at Hospital Clínico San Carlos, Madrid- Spain and PhD candidate at Complutense University of Madrid.
Dálifer is a member of the EMEUNET Social Media committee with a genuine interest and enthusiasm in research on Immune-mediated inflammatory diseases and its treatments.