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Autoinflammatory Disorders
Î SS 197. Patients with episodic fever should be screened for other
PIDDs, autoimmune disease, or malignancy. (C)
Cryopyrin-associated periodic syndromes (CAPS)
Î SS 198. CAPS should be suspected in patients presenting with
bouts of systemic inflammation resulting in rash, fevers, arthritis,
neurological deficits, and amyloidosis. (C)
Î SS 199. Patients suspected of having CAPS or FCAS2 should be
screened for persistent systemic signs of inflammation in the absence
of demonstrable infection, autoimmune disease, or malignancy. (C)
Î SS 200. IL-1 inhibitors (anakinra, rilonacept, and canakinumab) should
be given to all patients with CAPS and might be effective for FCAS2. (B)
Deficiency of IL-1 receptor antagonist (DIRA)
Î SS 201. Patients presenting at or soon after birth with a pustular rash,
joint swelling, and profound osteopenia and bone lesions should be
suspected of having DIRA. (C)
Î SS 202. Anakinra or other IL-1 antagonists should be used
therapeutically and can also be helpful in the diagnosis of DIRA. (C)
Blau syndrome
Î SS 203. Blau syndrome should be suspected in patients presenting
with noncaseating granulomas in the skin, eyes, and joints. (C)
Î SS 204. Corticosteroids should be the mainstay of treatment for
patients with Blau syndrome. (C)
Deficiency of IL-36 receptor antagonist (DITRA)
Î SS 205. Patients presenting with generalized pustular psoriasis
should be suspected of having DITRA. (C)
Î SS 206. Retinoids should be the mainstay of treatment for DITRA,
although steroids and IL-1 inhibitors have also been used. (C)
Familial Mediterranean fever (FMF) and TNF receptor-associated
periodic syndrome (TRAPS)
Î SS 207. FMF or TRAPS should be suspected in patients presenting
with recurrent and often prolonged fever attacks associated with
serosal, cutaneous, and synovial manifestations. (C)
Autoinflammatory Disorders
