Publications by authors named "E Bodar"
BACKGROUND IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory disease potentially affecting every part of the human body. Because of variability in clinical presentation, IgG4-RD can be challenging to diagnose. Untreated disease can lead to irreversible organ damage such as fibrosis.
View Article and Find Full Text PDFBackground: Mevalonate kinase deficiency (MKD) is a hereditary autoinflammatory syndrome marked by recurrent attacks of fever and inflammation. Severe enzyme deficiency results in mevalonic aciduria (MA) and milder deficiency in hyperimmunoglobulin D syndrome (HIDS). Treatment remains a challenge.
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- A study investigated the oral histone deacetylase inhibitor ITF2357 in eight patients with different autoinflammatory syndromes over 90 days.
- Some patients with Schnitzler syndrome and a TRAPS patient showed partial remission, while four patients had no improvement, and HIDS patients experienced worse symptoms.
- The results suggest that ITF2357 might help a little in Schnitzler syndrome but was ineffective for HIDS patients.
Unlabelled: A 62-year-old patient had suffered from severe cold intolerance with an urticarial rash and oropharyngeal angio-oedema upon cold exposure since early childhood. This could be provoked by the ice cube test and by exposure in a cold room. Her family history was negative, and she did not carry any mutations in the NRLP3 gene.
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