Complete remission in 3 of 3 anti-IL-6–treated patients with Schnitzler syndrome

Krause, Karoline; Feist, Eugen; Fiene, Michael; Kallinich, Tilmann; Maurer, Marcus

doi:10.1016/j.jaci.2011.10.031

Cited by 68 publications

(53 citation statements)

References 11 publications

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“…TCZ has also shown promise in several other indications, including amyloidosis [97][98][99][100], FMF [101][102][103], Schnitzler's syndrome [104,105], and polychondritis [106][107][108][109]. Results of the doubleblind, randomized, placebo-controlled, phase 3 TAKT trial in 36 patients with Takayasu arteritis demonstrated a trend toward relapse suppression in favor of TCZ-SC over placebo [110].…”

Section: Other Diseasesmentioning

confidence: 99%

A Review of Recent Advances Using Tocilizumab in the Treatment of Rheumatic Diseases

et al. 2018

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Tocilizumab (TCZ) is the first humanized antiinterleukin-6 (IL-6) receptor monoclonal antibody approved for the treatment of patients with rheumatoid arthritis (RA), Castleman's disease, polyarticular and systemic juvenile idiopathic arthritis, and, most recently, giant cell arteritis as well as for the treatment of chimeric antigen receptor T cell therapy-induced cytokine release syndrome. The global clinical development program for TCZ provides a wealth of clinical data on intravenous TCZ, and more recent studies in patients with RA have provided evidence characterizing the role of intravenous TCZ as monotherapy in early disease and led to the introduction of a subcutaneous formulation of TCZ. In addition, recently published open-label extension and observational studies continue to support the longterm efficacy and safety of TCZ in both clinical trial and real-world settings. Given the involvement of IL-6-mediated signaling in inflammatory disorders, TCZ is also being investigated in other immunological diseases. In particular, a phase 2 trial on the safety and efficacy of subcutaneous TCZ in adults with systemic sclerosis shows clinically relevant improvements in skin sclerosis and lung function in these patients. Another anti-IL-6 receptor agent, sarilumab, targeting the IL6 receptor alpha subunit, was recently approved for the treatment of patients with RA, although longterm data for this biologic are not yet published. In this article we review the placement of TCZ in current treatment guidelines; recent clinical trial data, including quality of life in patients with RA; recent updates to the TCZ safety profile; recent investigations of TCZ in other immunological diseases; and the clinical development of other novel IL-6-targeted agents. Keywords: Interleukin-6; Rheumatoid arthritis; Tocilizumab TOCILIZUMAB: THE FIRST INTERLEUKIN-6 RECEPTOR-NEUTRALIZING BIOLOGICInterleukin-6 (IL-6) is a multifunctional cytokine that plays an important role in both acute and chronic inflammatory responses. Consequently, the dysregulated or persistent production of IL-6 can lead to the development of inflammatory disorders [1]. Elevated levels of IL-6 in serum, synovial fluid, and various tissues have been correlated with disease activity in patients with rheumatoid arthritis (RA) [2], juvenile idiopathic arthritis (JIA) [3,4], Castleman's disease [5,6], systemic sclerosis (SSc) [7], giant cell arteritis (GCA) [8,9], adult-onset Still's disease (AOSD) [10], familial Mediterranean fever (FMF) [11,12], Schnitzler's syndrome [13,14], polychondritis [15], and amyloidosis [1]. These associations suggest a pathogenetic role for IL-6 in multiple inflammatory conditions and form the basis of the rationale for the development of anti-IL-6 therapies.Tocilizumab (TCZ) is the first humanized monoclonal antibody targeting the IL-6 receptor subunit alpha (IL-6Ra) [16], and its mechanism of action has been described in detail in previous reviews [17,18]. Briefly, TCZ targets both membrane-bound and soluble IL-6Ra, which prevents t...

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Section: Other Diseasesmentioning

confidence: 99%

A Review of Recent Advances Using Tocilizumab in the Treatment of Rheumatic Diseases

et al. 2018

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“…Response to glucocorticoids is variable in different autoinflammatory disorders. Cryopyrin-associated periodic syndrome and Schnitzler's syndrome, but not isolated chronic urticaria, respond poorly (or not at all) to glucocorticoids except at high doses of the order of a mg per kg (45). In HIDS, TRAPS, AOSD and soJIA however, oral glucocorticoids can be quite effective (7,34,40,46).…”

Section: How To Distinguish Isolated Chronic Urticaria From Autoinflamentioning

confidence: 99%

How not to miss autoinflammatory diseases masquerading as urticaria

Krause

Grattan

Bindslev‐Jensen

et al. 2012

Allergy

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Urticarial skin reactions are one of the most frequent problems seen by allergists and clinical immunologists in daily practice. The most common reason for recurrent wheals is spontaneous urticaria. There are, however, several less common diseases that present with urticarial rash, such as urticarial vasculitis and autoinflammatory disorders. The latter include cryopyrin-associated periodic syndrome and Schnitzler's syndrome, both rare and disabling conditions mediated by increased interleukin-1 secretion. Apart from the urticarial rash, patients are suffering from a variety of systemic symptoms including recurrent fever attacks, arthralgia or arthritis and fatigue. Autoinflammatory diseases are often associated with a diagnostic delay of many years and do not respond to antihistamines and other treatments of urticaria. Also, the chronic inflammation may lead to longterm complications such as amyloidosis. It is therefore important not to miss these diseases when diagnosing and treating patients with chronic recurrent urticarial rash. Here, we present clinical clues and tips that can help to identify autoinflammatory disorders in patients presenting with chronic urticarial rash and discuss their clinical picture and management.

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“…[157][158][159][160] Psoralen combined with ultraviolet A therapy and IL-6 blockade with tocilizumab may be effective, respectively. 161,162 Other treatments that have been used with variable success include TNF-alfa inhibition, thalidomide, colchicine, systemic steroids, and interferon alfa-2b. [163][164][165][166][167][168][169][170][171] Systemic-onset juvenile idiopathic arthritis Systemic-onset juvenile idiopathic arthritis (SOJIA) is a childhood-onset, relapsing inflammatory disorder with spiking fevers, an evanescent morbilliform rash occurring daily, and polyarticular arthritis.…”

Section: Schnitzler Syndromementioning

confidence: 99%

Autoinflammation: From monogenic syndromes to common skin diseases

Nguyen

Cowen

Leslie

2013

Journal of the American Academy of Dermatology

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Autoinflammation is characterized by aberrant regulation of the innate immune system and often manifests as periodic fevers and systemic inflammation involving multiple organs, including the skin. Mutations leading to abnormal behavior or activity of the interleukin 1 beta (IL-1ß)-processing inflammasome complex have been found in several rare autoinflammatory syndromes, for which anticytokine therapy such as IL-1 or tumor necrosis factor-alfa inhibition may be effective. It is becoming clear that features of autoinflammation also affect common dermatoses, some of which were previously thought to be solely autoimmune in origin (eg, vitiligo, systemic lupus erythematosus). Recognizing the pathogenetic role of autoinflammation can open up new avenues for the targeted treatment of complex, inflammatory dermatoses.

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Complete remission in 3 of 3 anti-IL-6–treated patients with Schnitzler syndrome

Cited by 68 publications

References 11 publications

A Review of Recent Advances Using Tocilizumab in the Treatment of Rheumatic Diseases

A Review of Recent Advances Using Tocilizumab in the Treatment of Rheumatic Diseases

How not to miss autoinflammatory diseases masquerading as urticaria

Autoinflammation: From monogenic syndromes to common skin diseases

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