Small vessel vasculitis and relapsing panniculitis in tumour necrosis factor receptor associated periodic syndrome (TRAPS)

Lamprecht, Peter; Moosig, Frank; Adam‐Klages, Sabine; Mrowietz, Ulrich; Csernok, Elena; Kirrstetter, M; Ahmadi‐Simab, K; Schröder, Johann O.; Gross, W L

doi:10.1136/ard.2003.016733

Cited by 70 publications

(36 citation statements)

References 11 publications

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“…Es kodiert für den p55-TNF-Rezeptor (TNFR1) und verlieh dem Syndrom seinen heutigen Namen [12,28]. [15]. Eine AA-Amyloidose wird in bis zu 25% der betroffenen Familien beobachtet und kann zu einer deutlichen Beeinträchtigung der Nierenfunktion führen.…”

Section: Tumornekrosefaktor-rezeptor-1-assoziiertes Fiebersyndromunclassified

Autoinflammatorische Erkrankungen als Ursache von Wundheilungsstörungen

et al. 2011

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Ulcerations of the skin and mucosal membranes are a common feature of autoinflammatory diseases. They can give raise to chronic wound healing defects and should be considered in the differential diagnosis of chronic skin ulcers. The increased activation of the innate immune system in the absence of an apparent provocation for inflammation is a hallmark of autoinflammatory diseases. Mutations and alterations of signaling pathways regulating the innate immune response to physical trauma/tissue damage result into an unrestrained activation of the inflammasome, which leads to increased activation of Interleukin-1. Uncontrolled recruitment and activation of myeloid effector cells within the wound site lead to the release of potent proteases that cause the degradation of structural components of the skin. The majority of these diseases respond well to immunosuppressive and immunomodulatory treatment regimes. Therapeutic resistance converts the acute inflammatory response into a chronic and non-resolving inflammatory process that leads to tissue degeneration. In this article we will focus on the review of those autoinflammatory diseases that often display ulcerative cutaneous and aphthous lesions including pyoderma gangrenosum, Behçet disease, PAPA syndrome and hyperimmunoglobulinemia D with periodic fever syndrome (HIDS). Furthermore, the article will be complemented by an overview of those inflammatory diseases that are associated with non-ulcerative cutaneous manifestations.

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Section: Tumornekrosefaktor-rezeptor-1-assoziiertes Fiebersyndromunclassified

Autoinflammatorische Erkrankungen als Ursache von Wundheilungsstörungen

et al. 2011

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“…Even unusual manifestations such as non-suppurative panniculitis and small-vessel vasculitis were controlled by etanercept in 2 adult patients (22). In a recently reported case series of 7 patients affected with TRAPS who were treated with etanercept for 24 weeks, although the drug did not completely eliminate inflammatory attacks, it was able to reduce disease activity, also in terms of acute-phase response, and allowed for reduction of corticosteroid dose administration in all cases (23).…”

Section: Treatment Strategiesmentioning

confidence: 99%

Role of Etanercept in the Treatment of Tumor Necrosis Factor Receptor-Associated Periodic Syndrome: Personal Experience and Review of the Literature

Cantarini

Rigante

Lucherini

et al. 2010

Int J Immunopathol Pharmacol

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Tumor necrosis factor-α receptor (TNFRl)—associated periodic syndrome (TRAPS) is the most common autosomal-dominant autoinflammatory condition and is caused by mutations in the TNFRSF1A gene. TRAPS is characterized by recurrent attacks of fever typically lasting from 1 to 3 weeks; in addition to fever, common clinical features include mainly periorbital oedema, conjunctivitis, a migratory erythematous plaque simulating erysipela with underlying myalgia, and arthritis or arthralgia; serosal membrane inflammation is also possible. The identification of TNFRSF1A mutations as the genetic cause of TRAPS coincided with the wider use of biological agents in medicine and raised the possibility that blocking TNF could potentially represent the primary therapeutic goal in TRAPS, thus disclosing new treatment choices for this complex disease. In the past few years, isolated reports and case-series have been published suggesting that inhibition of TNF-α might represent a promising therapeutic approach in TRAPS. We present here our experience with etanercept in the treatment of patients affected with TRAPS, and we also add a review of the literature.

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“…В настоящее время в доступной литературе представле-но три случая успешного применения иФНОα при ИПВК, но все эти пациенты имели узловатую форму болезни [6][7][8]. Это обстоятельство дает право утверждать, что наше наблю-дение является первым описанием случая успешного приме-нения иФНОα при инфильтративной форме болезни, отли-чающейся наибольшей торпидностью к терапии.…”

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