all CAPS: A Story of Ice and Fire - Part 1

ByAdam J. Brown, MD

This first episode delves into the clinical aspects of the Cryopyrin-Associated Periodic Syndromes and includes some helpful tips for sorting out some of the autoinflammatory diseases in the spectrum.

Intro :10
Cryopyrin-Associated Periodic Syndromes (CAPS) :25
Outline of Part 1 2:25
What is CAPS? 3:12
Case #1 – a 25-year-old female with urticaria after cold exposure 4:25
Case #2 – a 55-year-old female with progressive deafness 5:45
The index case of familial cold autoinflammatory syndrome 9:00
The final disease in the spectrum 11:00
A lot of names for the same disease 13:50
Genetics prove these three diseases are in the same spectrum 14:22
Over 100 mutations have been found to-date 16:31
Let’s talk about mosaicism 17:03
Disease characteristics of CAPS 18:15
- Neutrophilic infiltrate in chronic urticaria vs. CAPS 20:00
- Schnitzler syndrome 20:45
- Sensorineural hearing loss 21:58
- Joint pain 24:10
Complication: Amyloidosis 25:29
These diseases were very debilitating 28:38
Episode recap 29:46
Part 2 sneak peek 30:38
Come say hi at ACR! 30:51