Schnitzler syndrome: response to anakinra in two cases and a review of the literature

Abstract

Based on published data, monotherapy with anakinra is currently the most promising treatment for Schnitzler syndrome, because it is able to induce complete remission of symptoms.

Autoinflammatory Skin Disease: A Review of Concepts and Applications to General Dermatology

“…CAPS shares many phenotypic features with the multisystemic inflammatory diseases such as systemic-onset juvenile idiopathic arthritis, adult-onset Still's disease, and Schnitzler's syndrome such as febrile attacks, urticarial eruptions, bone pain, and elevated inflammatory markers [15] . As these diseases share phenotypic similarities and all are responsive to IL-1 blockade, they have been termed collectively 'IL-1 opathies'.…”

Role of Interleukin 1 in the Development of Human Diseases

“…Ус-тановлено, что лимфоциты, выделенные из крови пациен-тов с синдромом Шницлера, синтезируют избыточное ко-личество ИЛ1 (а также ИЛ6) при стимуляции липополиса-харидом [122,123], что обосновывает причины эффектив-ности ингибиторов ИЛ1 при этом заболевании [118,120,121,[125][126][127][128][129][130]. Это представляется весьма важным с учетом данных о роли ИЛ1 в развитии миеломной болезни, ослож-няющей течение синдрома Шницлера.…”

Schnitzler syndrome with cold-induced urticaria

“…Recurrent fever of about 40°C occurs in 90% of patients but is not related to timing or presentation of urticaria. 4 Lymphadenopathy is found in 50% of patients. The nodes measure 2-3 cm and are usually located at the axillary and inguinal sites, although the cervical chain may be involved.…”

“…1,2 It is a rare condition with approximately 90-100 cases reported in the literature. [1][2][3][4] Thorough history and examination are necessary to rule out other diseases, especially those of hematologic origin. To our knowledge, this is the first reported case of cold-induced physical urticaria with Schnitzler syndrome.…”