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Î SS 208. Patients suspected of having FMF or TRAPS should be
screened for persistent systemic signs of inflammation in the absence
of demonstrable infection or autoimmune disease. (C)
Î SS 209. Colchicine should be the mainstay of therapy for FMF. (B)
Î SS 210. Corticosteroids, TNF blockers, and IL-1 antagonists should be
used in therapy for TRAPS. (B)
Mevalonate kinase deficiency (hyper-IgD syndrome [HIDS])
Î SS 211. HIDS should be suspected in patients presenting with fevers
with lymphadenopathy, abdominal pain, diarrhea, vomiting, arthralgia,
rash, aphthous ulcers, and splenomegaly. (C)
Î SS 212. Patients with suspected HIDS should be screened by
measuring serum IgD and urine mevalonic acid levels. (C)
Î SS 213. Therapeutic trials of corticosteroids and inflammatory
cytokine inhibitors should be undertaken for patients with HIDS. (C)
Pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA)
Î SS 214. PAPA syndrome should be suspected in patients presenting
with the characteristic recurrent episodes of severe joint and skin
inflammation. (C)
Î SS 215. Treatment of patients with PAPA syndrome with cytokine
inhibitors should be attempted. (C)
Proteasome catalytic subunit b type 8 (PSMB8 gene) and
transmembrane protein 173 (TMEM173; stimulator of
interferon) defects
Î SS 216. These disorders should be suspected in patients with early-
onset fevers, systemic inflammation, and purpuric plaques caused by
cutaneous leukocytoclastic vasculitis. (C)
Î SS 217. A variety of anti-inflammatory modalities should be tried in
patients with PSMB8 or TMEM173 defects. (C)
Autoinflammatory Disorders
