A Case of Successful Treatment of Autoinflammatory Syndrome-Associated Chronic Urticaria with Anakinra
Monday, March 7, 2016
South Exhibit Hall H (Convention Center)
Young-Il Koh, MD, Min-joo Ahn, MD, Ji Eun Yu, MD, Jiung Jeong, MD
Rationale: Autoinflammatory syndrome is characterized by various systemic inflammatory symptoms and cutaneous urticarial rashes. Systemic disease-associated chronic urticaria may be often difficult to treat with usual anti-urticarial medications. We report a case in which autoinflammatory syndrome-associated chronic urticaria was successfully treated with IL-1 receptor antagonist, anakinra.

Methods: Anakinra 100 mg was subcutaneously injected on a daily basis.

Results: A 69-year-old man suffered from pruritic urticarial rash for 9 years. It aggravated episodically and accompanied high fever, arthralgia, leukocytosis, elevated C-reactive protein and erythrocyte sedimentation rate. The episodes lasted over one week. A subpleural nodule with pleural effusion was detected. Neutrophilic and eosinophilic inflammation was found on skin biopsy. The cutaneous lesions were unresponsive to various kinds of anti-histamines, systemic glucocorticoids, colchicine, cyclosporine, dapsone, or methotrexate that have been administered in the recent 3 years. A dramatic response however, has been observed after a daily administration of anakinra. We assume that the case might have adult onset TNF receptor-associated periodic syndrome.

Conclusions: Anakinra could be effective in cases of corticosteroid- and other immunomodulator-refractory chronic urticaria, particularly in urticaria patients with suspected autoinflammatory syndrome.