Schnitzler syndrome with IgG gammopathy and elevated IL-1β and IL-17 in skin biopsy

Villarreal, R.; VandenBoom, Timothy G.; González-González, Francisco J.; Carter, Roderick G.; Peters, Neill T.; Peters, Anju T.; Chiarella, Sergio E.

doi:10.1016/j.anai.2017.10.006

Cited by 2 publications

(5 citation statements)

References 10 publications

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“…5,9 The dramatic response to IL-1 blockade indicates that Schnitzler syndrome is driven by IL-1, which is supported by overproduction of IL-1 in peripheral blood and at the skin lesions. 10,11 tivation in these patients. 2 Intriguingly, gain-of-function mutations and somatic NLRP3 mosaicism were found in some patients with Schnitzler-like syndrome.…”

Section: Skin Biopsy Of the Urticaria Shows Features Of Neutrophilic ...mentioning

confidence: 74%

Schnitzler syndrome—An underdiagnosed adult‐onset autoinflammatory disease

Chu

2023

Int J of Rheum Dis

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Section: Skin Biopsy Of the Urticaria Shows Features Of Neutrophilic ...mentioning

confidence: 74%

Schnitzler syndrome—An underdiagnosed adult‐onset autoinflammatory disease

Chu

2023

Int J of Rheum Dis

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“…Intriguingly, the production of cytokines, such as IL-4, interferon-g, and IL-17A after stimulation of PBMC by anti-CD3 and anti-CD28 antibodies, was suppressed in untreated Schnitzler syndrome patients and this suppression is reversed in patients treated by anakinra [42] . In contrast, IL-17 is found to be highly expressed along with IL-1 in the lesional skin in one case of Schnitzler syndrome although the cellular source in the skin for IL-17 was not identified [44] . The significance of diminished T cell response to T cell receptor stimulation is not clear.…”

Section: Classical Schnitzler Syndromementioning

confidence: 88%

“…[81] Anti-IL-17 therapy IL-17A is found in the lesional skin of patients with Schnitzler syndrome. [30,44] The cellular source in the skin is neutrophils. [30] IL-17A is a potent neutrophil chemoattractant.…”

Section: Rituximabmentioning

confidence: 99%

“…[ 42 ] In contrast, IL-17 is found to be highly expressed along with IL-1 in the lesional skin in one case of Schnitzler syndrome although the cellular source in the skin for IL-17 was not identified. [ 44 ] The significance of diminished T cell response to T cell receptor stimulation is not clear. Further investigation is required to determine whether it contributes to the pathogenesis of Schnitzler syndrome or it is an artificial in vitro experimental phenomenon.…”

Section: Classical Schnitzler Syndromementioning

confidence: 99%

“…IL-17A is found in the lesional skin of patients with Schnitzler syndrome [30,44] . The cellular source in the skin is neutrophils [30] .…”

Section: Classical Schnitzler Syndromementioning

confidence: 99%

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Schnitzler syndrome and Schnitzler-like syndromes

Chu

2022

Chinese Medical Journal

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Schnitzler syndrome is a rare disease of adult-onset with main features including chronic urticarial rash, recurrent fever, arthralgia or arthritis, monoclonal gammopathy of undetermined significance (MGUS), and marked systemic inflammation. Schnitzler syndrome is often underdiagnosed. Patients with Schnitzler syndrome may present to dermatologists and allergists for urticaria, hematologists for MGUS, or rheumatologists for arthritis. It is important to recognize Schnitzler syndrome for its remarkable response to interleukin (IL)-1 blockade. Besides, many cases of Schnitzler-like syndromes do not meet the diagnostic criteria of classical Schnitzler syndrome but display excellent response to IL-1 inhibitors. The overly produced IL-1 is the result of a somatic mosaic gain of function mutation of NLRP3 (nucleotide-binding oligomerization domain [NOD]-like receptor [NLR] family pyrin domain containing 3) gene in some patients with Schnitzler-like syndromes. Inflammasome activation is evident in patients with classical Schnitzler syndrome although no NLRP3 gene mutation is identified. Collectively, Schnitzler syndrome and Schnitzler-like syndromes represent a spectrum of IL-1 mediated adult-onset autoinflammatory diseases.

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Schnitzler syndrome with IgG gammopathy and elevated IL-1β and IL-17 in skin biopsy

Cited by 2 publications

References 10 publications

Schnitzler syndrome—An underdiagnosed adult‐onset autoinflammatory disease

Schnitzler syndrome—An underdiagnosed adult‐onset autoinflammatory disease

Schnitzler syndrome and Schnitzler-like syndromes

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