Treatment of hyperimmunoglobulin D syndrome (HIDS)

HIDS primary affects quality of life and rarely leads to severe physical complications. Thus, the main approach to management is symptomatic treatment of episodes if they significantly impact quality of life and the patient/family wishes to treat. There are no clearly effective treatments for the rare patients with HIDS who develop AA amyloidosis. Continuous therapy with a biologic agent is usually tried in these cases.

NSAID: nonsteroidal antiinflammatory drug.
* Symptomatic treatment is started at the onset of prodromal symptoms (eg, nasal congestion, sore throat, backache, fatigue, vertigo, headache, and behavioral changes) and continued for the anticipated duration of the attack (approximately 4 to 7 days).
¶ Refer to UpToDate topic on management of hyperimmunoglobulin D syndrome for details on dosing options.
Δ An alternative to episodic anakinra is prophylactic therapy with canakinumab.
◊ Options for biologic agents for prophylactic therapy include anakinra, canakinumab, etanercept, and tocilizumab.

Graphic 108431 Version 4.0

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Treatment of hyperimmunoglobulin D syndrome (HIDS)