Auto-inflammatory Syndromes - Twitter summary from #CSACI19 meeting

Dr. Ellis @DrAnneEllis: Dr. Marcus Shaker "Auto-inflammatory Syndromes".

Recurrent fever - common things are more common! Recurrent viral illness (think day care) recurrent bacterial infection (think immunodeficiency), pseudo fever explain most.

Cryopyrinopathies are rare but described association with celiac disease.

Cryopyrin Associated Periodic Syndromes (CAPS) include Familial Cold Autoinflammatory syndrome (FCAS), Muckle-Wells Syndrome and Neonatal onset multisystem inflammatory disease (NOMID).

Canakinumab (human monoclonal antibody targeted at interleukin-1 beta) led to a 98% response rate for CAPS in the pivotal @NEJM publication.

Autoinflammatory syndromes are issues with the innate immune syndrome rather than adaptive.

TRAPS presents with recurrent fevers, serositis, abdominal pain, rash, myalgias and amyloidosis. Treatment is steroids and anti-Il1.

Shaker: Sometimes discussing auto-inflammatory conditions can feel like Alphabet Soup.

No comments:

Post a Comment