Autoinflammatory syndromes by Dr Dan Kastner at #ACR22 !
1️⃣FMF🏖️
🧬AR, pyrin mutation, evolutionary resistance to Y pestis
🌡️Fever 1-3 days
😣Peritonitis (severe)
🫁Pleurisy unilateral
✊Mono or oligoarthritis
2%-->Amyloidosis
⚕️ colchicine, anti IL1 💉
#MedTwitter #MedEd
1️⃣FMF🏖️
🧬AR, pyrin mutation, evolutionary resistance to Y pestis
🌡️Fever 1-3 days
😣Peritonitis (severe)
🫁Pleurisy unilateral
✊Mono or oligoarthritis
2%-->Amyloidosis
⚕️ colchicine, anti IL1 💉
#MedTwitter #MedEd
2️⃣TRAPS
No ethnic predisposition
🧬AD, TNGRSF1A- p55
🌡️Fever 4-6 weeks
😣Serositis
♨️Migratory rash proximal -->distal
💪fasciitis
👁️ conjunctivitis, periorbital edema
Amyloidosis occasionally
❌response to colchicine
⚕️Steorids ✅ anti IL1✅
No ethnic predisposition
🧬AD, TNGRSF1A- p55
🌡️Fever 4-6 weeks
😣Serositis
♨️Migratory rash proximal -->distal
💪fasciitis
👁️ conjunctivitis, periorbital edema
Amyloidosis occasionally
❌response to colchicine
⚕️Steorids ✅ anti IL1✅
3️⃣HIDS/MKD
Northern europeans
🌡️3-7 days
😣 Serositis
🦵✊Arthritis
♨️Diffuse MP rash
Cervical adenopathy
Aphthous ulcers
⚕️Canakinumab
Northern europeans
🌡️3-7 days
😣 Serositis
🦵✊Arthritis
♨️Diffuse MP rash
Cervical adenopathy
Aphthous ulcers
⚕️Canakinumab
4️⃣CAPS
🧬 AD, NLRP3, cryopyrin
FCAS 🌡️ urticaria 2 hrs after 🥶
MWS 🌡️ limb pain urticaria SNHL amyloidosis 25%
NOMID 🌡️ urticaria, papilledema, epiphyseal swelling, SNHL, neurologic features
🧬 AD, NLRP3, cryopyrin
FCAS 🌡️ urticaria 2 hrs after 🥶
MWS 🌡️ limb pain urticaria SNHL amyloidosis 25%
NOMID 🌡️ urticaria, papilledema, epiphyseal swelling, SNHL, neurologic features
5️⃣DADA2
🌡️ Recurrent strokes livedoid rash
PAN like presentation
Lesser the ADA2, more likely to have bone marrow features
⚕️TNFis✅
🌡️ Recurrent strokes livedoid rash
PAN like presentation
Lesser the ADA2, more likely to have bone marrow features
⚕️TNFis✅
• • •
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