Schnitzler syndrome

Gellrich, Frank Friedrich; Günther, Claudia

doi:10.1007/s00105-019-4434-4

Cited by 12 publications

(14 citation statements)

References 34 publications

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“…Multiple features indicate that it is an acquired autoinflammatory disorder, 21 , 22 with elevated levels of proinflammatory cytokine IL-1, including IL-1β, playing a central role in the pathophysiology of the disease. 23 , 24 However, a possible link between autoinflammation and the monoclonal component (monoclonal IgM or IgG gammopathy) remains to be determined, because the level of monoclonal component is highly variable in Schnitzler’s syndrome, which can be very low or very high at once. 25 , 26 Lipsker et al performed immunoblotting on epidermal and dermal skin extracts, and immunoelectron microscopic studies on Lowicryl K4M-embedded skin sections in 3 patients with Schnitzler syndrome.…”

Section: Schnitzler’s Syndromementioning

confidence: 99%

New insights on multigenic autoinflammatory diseases

Efthimiou¹,

Petryna

Nakasato

et al. 2022

Therapeutic Advances in Musculoskeletal

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Autoinflammatory diseases are disorders of the innate immune system, which can be either monogenic due to a specific genetic mutation or complex multigenic due to the involvement of multiple genes. The aim of this review is to explore and summarize the recent advances in pathogenesis, diagnosis, and management of genetically complex autoinflammatory diseases, such as Schnitzler’s syndrome; adult-onset Still’s disease; synovitis, acne, pustulosis, hyperostosis, osteitis syndrome/chronic recurrent multifocal osteomyelitis/chronic non-bacterial osteomyelitis; Adamantiades-Behçet’s disease; Yao syndrome; and periodic fever with aphthous stomatitis, pharyngitis, and adenitis syndrome. The PubMed database was screened for relevant articles using free text words and specific search strings. The search was limited to English-language articles, reporting the results of studies in humans, published through March 2021. Evidence from literature suggest that these rare multigenic autoinflammatory diseases can present with different clinical features and the diagnosis of these diseases can be challenging due to a combination of nonspecific manifestations that can be seen in a variety of other conditions. Diagnostic delays and disease complications may occur due to low disease awareness and the lack of pathognomonic markers. The pathogeneses of these diseases are complex and in some cases precise pathogenesis is not clearly understood. Conventional treatments are commonly used for the management of these conditions, but biologics have shown promising results. Biologics targeting proinflammatory cytokines including IL-1, IL-6, TNF-α, IL-17A and IL-18 have been shown to ameliorate signs and symptoms of different multigenic autoinflammatory diseases.

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Section: Schnitzler’s Syndromementioning

confidence: 99%

New insights on multigenic autoinflammatory diseases

Efthimiou¹,

Petryna

Nakasato

et al. 2022

Therapeutic Advances in Musculoskeletal

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“…Ingen levende, svekkede vaksiner må gis under behandlingen. Hematologisk status, blodprøver, CRP, serumelektroforese og kvantitering av immunglobuliner samt leverprøver er anbefalt kontrollert hver tredje måned (15). Noen få pasienter vil likevel ikke respondere på IL-1-hemmende terapi.…”

Section: Diskusjonunclassified

En mann i 50-årene med tilbakevendende urtikaria, feber og leddsmerter

Simonsen¹,

Berkman²,

Skorpen³

et al. 2022

Tidsskriftet

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er spesialist i indremedisin og i infeksjonssykdommer, fagansvarlig overlege, enhetsleder for leger ved Avdeling for gastromedisin, infeksjon, geriatri og hud og universitetslektor. Forfatteren har fylt ut ICMJE-skjemaet og oppgir ingen interessekonflikter. Bø legekontor Straumsjøen Raimonda Berkman er lege i spesialisering i allmennmedisin og fastlege. Forfatteren har fylt ut ICMJE-skjemaet og oppgir ingen interessekonflikter. Medisinsk avdeling Vesterålen Nordlandssykehuset Stokmarknes Per Kristian Skorpen er spesialist i indremedisin og overlege. Forfatteren har fylt ut ICMJE-skjemaet og oppgir ingen interessekonflikter. Hematologisk seksjon Nordlandssykehuset Bodø Randi Fykse Hallstensen er spesialist i indremedisin og i blodsykdommer og fagansvarlig overlege. Forfatteren har fylt ut ICMJE-skjemaet og oppgir ingen interessekonflikter. Seksjon for klinisk immunologi og infeksjonsmedisin Oslo universitetssykehus, Rikshospitalet og Universitetet i Oslo En mann i 50-årene med tilbakevendende urtikaria, feber og leddsmerter | Tidsskrift for Den norske legeforening

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“…El síndrome de Schnitzler (SS) es un trastorno autoinflamatorio adquirido en la adultez, poco frecuente, asociado principalmente a GM tipo IgM [38][39][40] . La fisiopatología no se conoce del todo, pero, en consecuencia, se produce un aumento de interleuquina (IL)-1 beta e IL-6 38 .…”

Section: Mediadores Inflamatoriosunclassified

“…Clí- nicamente se caracteriza por episodios recurrentes de fiebre, artralgias, adenopatías, hepatoesplenomegalia y un exantema máculo-papular urticariforme que dura menos de 24 h 5,39 . La asociación más común del SS es una GM tipo IgM-kappa, y hasta 25% de los pacientes tendrán niveles bajos de IgG e IgA 40 . Los exámenes de laboratorio pueden mostrar anemia, leucocitosis con neutrofilia, trombocitosis, VHS y PCR elevadas, y aumento de marcadores de remodelamiento óseo anormal y de factor de crecimiento endotelial vascular (VEGF).…”

Section: Mediadores Inflamatoriosunclassified

“…La histopatología de las lesiones cutáneas se caracteriza por un infiltrado de neutrófilos perivascular e intersticial dérmico, asociado a leucocitoclasia sin vasculitis y edema de la dermis 38 . La evolución es crónica, con remisiones y exacerbaciones, y puede evolucionar a MW en 23% a 10-20 años y amiloidosis sistémica secundaria [38][39][40][41] .…”

Section: Mediadores Inflamatoriosunclassified

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