Schnitzler syndrome: Treatment failure to rituximab but response to anakinra

Eiling, Elisabeth; Möller, Maike; Kreiselmaier, Inga; Brasch, Jochen; Schwarz, Thomas

doi:10.1016/j.jaad.2007.03.036

Cited by 69 publications

(48 citation statements)

References 11 publications

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“…In contrast, anakinra resulted in a rapid improvement sometimes within hours and a complete remission in disease activity within days. 46 Table 1 lists autoinflammatory diseases that, for the most part, respond to blocking IL-1 activity. …”

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confidence: 99%

Interleukin-1 in the pathogenesis and treatment of inflammatory diseases

Dinarello

2011

Blood

1,815

1,479

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More than any other cytokine family, the IL-1 family of ligands and receptors is primarily associated with acute and chronic inflammation. The cytosolic segment of each IL-1 receptor family member contains the Toll-IL-1-receptor domain. This domain is also present in each Tolllike receptor, the receptors that respond to microbial products and viruses. Since Toll-IL-1-receptor domains are functional for both receptor families, responses to the IL-1 family are fundamental to innate immunity. Of the 11 members of the IL-1 family, IL-1␤ has emerged as a therapeutic target for an expanding number of systemic and local inflammatory conditions called autoinflammatory diseases. For these, neutralization of IL-1␤ results in a rapid and sustained reduction in disease severity. Treatment for autoimmune diseases often includes immunosuppressive drugs whereas neutralization of IL-1␤ is mostly anti-inflammatory. Although some autoinflammatory diseases are due to gain-of-function mutations for caspase-1 activity, common diseases such as gout, type 2 diabetes, heart failure, recurrent pericarditis, rheumatoid arthritis, and smoldering myeloma also are responsive to IL-1␤ neutralization. This review summarizes acute and chronic inflammatory diseases that are treated by reducing IL-1␤ activity and proposes that disease severity is affected by the anti-inflammatory members of the IL-1 family of ligands and receptors. (Blood. 2011;117(14):3720-3732) IntroductionSince the 1996 publication in Blood of "Biologic Basis for Interleukin-1 in Disease," 1 there have been several major advances in understanding a role for IL-1 in the pathogenesis of disease. Because of its property as a hematopoietic factor, IL-1 was administered to patients to improve recovery after BM transplantation (human responses to IL-1 were reviewed in detail in 1996). 1 Effective in reducing the duration of thrombocytopenia and leukopenia, recipients of IL-1 therapy experienced unacceptable signs and symptoms of systemic inflammation, including hypotension. Therefore, attention was initially focused on blocking IL-1 activity in sepsis with the use of the naturally occurring IL-1 receptor antagonist (IL-1Ra), now known by its generic name anakinra. There were 3 controlled trials of anakinra in human sepsis. Although in each trial there was a reduction in 28-day all-causes mortality compared with placebo-treated patients, the reductions did not reach statistical significance. 2 The failure of blocking IL-1 to significantly reduce mortality in septic shock is not unusual, because most anticytokines and anti-inflammatory agents have also failed in sepsis trials (reviewed in Eichacker et al 3 ).Subsequently, attention focused on blocking IL-1 in noninfectious, chronic inflammatory conditions, such as rheumatoid arthritis. Anakinra is approved for reducing the signs and symptoms of rheumatoid arthritis and slows the progressive joint destructive characteristics of the disease. Anakinra has also been administered to patients with smoldering/indolent myeloma at high risk o...

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confidence: 99%

Interleukin-1 in the pathogenesis and treatment of inflammatory diseases

Dinarello

2011

Blood

1,815

1,479

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“…Its pathogenesis is related to excessive interleukin-1 (IL-1) (8, 9) which is further evidenced by the good and rapid clinical response to IL-1 receptor blockade (7,10,11). IL-1 is a pro-inflammatory cytokine that is mainly released by cells of the innate immune system.…”

Section: Discussionmentioning

confidence: 99%

“…Monotherapy with the IL-1 receptor antagonist anakinra is a highly effective therapeutic option for Schnitzler syndrome (7,10,11). Other IL-1 inhibiting agents, such as rilonacept and canacinumab have also demonstrated efficacy in short term studies, further studies are needed to evaluate long term efficacy (4).…”

Section: Discussionmentioning

confidence: 99%

Interleukin-1 receptor antagonist (anakinra) for Schnitzler syndrome

Sönnichsen

Saulīte

Mangana

et al. 2016

Journal of Dermatological Treatment

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Schnitzler syndrome is a rare autoinflammatory disease, which is defined by the presence of two major criteria: chronic urticaria and monoclonal IgM or IgG gammopathy, in combination with at least two additional minor criteria: recurrent fever, leukocytosis and/or elevated CRP, objective signs of abnormal bone remodeling and a neutrophilic infiltrate in skin biopsy.We present a 68-year old female patient with a 10 year medical history of chronic urticaria, recurrent fever, severe arthralgia and increased CRP. Over the years multiple diagnostic investigations were performed without conclusive findings, and therapeutic attempts with anti-histamines and several immunosuppressive agents had failed.The decision to initiate monotherapy with interleukin-1 receptor antagonist was based on immunohistochemical detection of abundance of IL-1β positive cells in the patient's skin biopsy. After starting treatment with anakinra, disappearance of symptoms could be observed within 24 hours. Discontinuation of the treatment resulted in a rapid relapse of the symptoms. . Finally, already after the initiation of therapy with anakinra, the suspected diagnosis of Schnitzler syndrome could be confirmed by detection of IgMgammopathy that was initially absent.

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“…Heyes ve ark ise tedaviye dirençli elefantiyazik tiroid dermopatisi olan bir olgunun rituksimab ve plazmaferez tedavisine yanıt verdiğini göstermişlerdir. Ruiz ve ark ise 5 vitiligolu hastada 6 ay boyunca rituksimab tedavisi uyguladıklarında 3 olguda düzelme, 1 olguda kısmi düzelme ve 1 olguda hiç düzelmeme saptamışlardır (132)(133)(134)(135)(136)(137)(138)(139)(140)(141)(142)(143)(144)(145)(146).…”

Section: I-i̇mmunglobulin G4 İlişkili Hastalıklarunclassified

Dermatolojide Rituksimab Kullanımı

2014

Dermatoz

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Schnitzler syndrome: Treatment failure to rituximab but response to anakinra

Cited by 69 publications

References 11 publications

Interleukin-1 in the pathogenesis and treatment of inflammatory diseases

Interleukin-1 in the pathogenesis and treatment of inflammatory diseases

Interleukin-1 receptor antagonist (anakinra) for Schnitzler syndrome

Dermatolojide Rituksimab Kullanımı

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