Improvement of liver involvement in familial Mediterranean fever after introduction of canakinumab: a case report

Massaro, Maria Grazia; Pompili, Maurizio; Sicignano, Ludovico Luca; Pizzolante, Fabrizio; Verrecchia, Elena; Vecchio, Fabio Maria; Rigante, Donato; Manna, Raffaele

doi:10.4084/mjhid.2020.059

Cited by 3 publications

(2 citation statements)

References 47 publications

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“…The FDA has approved canakinumab for the treatment of Still's disease and it is sold under the brand name Ilaris. A study reported that treatment with canakinumab in combination with colchicine showed improvement in hepatic dysfunction due to familial Mediterranean fever [85]. Although it has shown promising effects in the management of cardiovascular disorders, its significant efficacy in the specific management of hepatic disorders has not been observed yet.…”

Section: Canakinumabmentioning

confidence: 99%

Mechanisms of Inflammasome Activation and Involvement in Liver Disease

Baral

2024

JMP

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The liver is a multi-potent organ with important metabolic, immunological and endocrine functions. Hepatic physiology is maintained at a balanced state via the delicate actions of different liver-resident cells. Among several factors that modulate hepatic physiology, the harmony between the activity of pro- and anti-inflammatory cytokines is a crucial determinant. However, initiation of inflammatory activity can be detrimental if it goes unresolved, leading to severe consequences such as hepatitis, hepatic fibrosis, cirrhosis or even hepatocellular carcinoma (HCC). Different physiological processes can modulate the hepatic microenvironment; one such factor is a cytosolic protein complex called the inflammasome. Inflammasome activation is a consequence of the cellular encounter with pathogens or products of cellular damage. Once activated, inflammasomes promote the maturation of interleukin-1 family cytokines such as IL-1β and IL-18 via activation of caspase-1. These cytokines have a very potent role in modulating hepatic physiology. Various lines of reports suggest that inflammasome activation and IL-1 cytokines play critical roles in liver diseases, including hepatitis, hepatic fibrosis and HCC. Conversely, inhibition of inflammasome activation and/or IL-1 signaling prevents such effects. This review summarizes the mechanisms leading to inflammasome activation and the role it plays in hepatic physiology.

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Section: Canakinumabmentioning

confidence: 99%

Mechanisms of Inflammasome Activation and Involvement in Liver Disease

Baral

2024

JMP

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“…Indeed, IL-1 antagonists are the treatment of choice in refractory or colchicine-intolerant cases of FMF, and a large experience with anakinra and canakinumab is now available for thousands of colchicine-resistant patients [40]. Canakinumab seems to have a positive effect on different severe inflammatory symptoms by controlling disease activity and overall inflammation [41]. A better interpretation of FMF mutations in various clinical sceneries has led to coin the umbrella-term "pyrin-associated autoinflammatory diseases", including all non-FMF diseases caused by MEFV-related pyrin defects, such as periodic undefined fevers, periodic fever/aphthosis/ pharyngitis/adenitis syndrome-like pictures and neutrophilic dermatosis [42].…”

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confidence: 99%

The everchanging framework of autoinflammation

Manna

Rigante

2021

Intern Emerg Med

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The innate immunity works as a defence bullwark that safeguards healthy tissues with the power of detecting infectious agents in the human body: errors in the context of innate immunity identify autoinflammatory disorders (AIDs), which arise as bouts of aberrant inflammation with little or no involvement of T and B cells and neither recognized infections, nor associated autoimmune phenomena. Hereditary AIDs tend to have a pediatric-onset heralded by stereotyped inflammatory symptoms and fever, while AIDs without an ascertained cause, such as systemic juvenile idiopathic arthritis, derive from the interaction of genetic factors with environmental noxae and are unevenly defined. A dysregulated inflammasome activation promotes the best-known family of AIDs, as well as several degenerative and metabolic disorders, but also nuclear factor κB- and interferon-mediated conditions have been framed as AIDs: the zenith of inflammatory flares marks different phenotypes, but diagnosis may go unnoticed until adulthood due to downplayed symptoms and complex kaleidoscopic presentations. This review summarizes the main AIDs encountered in childhood with special emphasis on the clinical stigmata that may help establish a correct framework and blueprints to empower young scientists in the recognition of AIDs. The description focuses inflammasomopathies as paradigms of interleukinopathies, nuclear factor-κB -related disorders and interferonopathies. The challenges in the management of AIDs during childhood have been recently boosted by numerous therapeutic options derived from genomically-based approaches, which have led to identify targeted biologic agents as rationalized treatments and achieve more tangible perspectives of disease control.

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Emerging Role of NLRP3 Inflammasome and Pyroptosis in Liver Transplantation

Lucas‐Ruiz

Peñín‐Franch

Pons

et al. 2022

IJMS

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The nucleotide-binding domain leucine-rich repeat-receptor, pyrin domain-containing-3 (NLRP3) inflammasome contributes to the inflammatory response by activating caspase-1, which in turn participates in the maturation of interleukin (IL)-1β and IL-18, which are mainly secreted via pyroptosis. Pyroptosis is a lytic type of cell death that is controlled by caspase-1 processing gasdermin D. The amino-terminal fragment of gasdermin D inserts into the plasma membrane, creating stable pores and enabling the release of several proinflammatory factors. The activation of NLRP3 inflammasome and pyroptosis has been involved in the progression of liver fibrosis and its end-stage cirrhosis, which is among the main etiologies for liver transplantation (LT). Moreover, the NLRP3 inflammasome is involved in ischemia–reperfusion injury and early inflammation and rejection after LT. In this review, we summarize the recent literature addressing the role of the NLRP3 inflammasome and pyroptosis in all stages involved in LT and argue the potential targeting of this pathway as a future therapeutic strategy to improve LT outcomes. Likewise, we also discuss the impact of graft quality influenced by donation after circulatory death and the expected role of machine perfusion technology to modify the injury response related to inflammasome activation.

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Improvement of liver involvement in familial Mediterranean fever after introduction of canakinumab: a case report

Cited by 3 publications

References 47 publications

Mechanisms of Inflammasome Activation and Involvement in Liver Disease

Mechanisms of Inflammasome Activation and Involvement in Liver Disease

The everchanging framework of autoinflammation

Emerging Role of NLRP3 Inflammasome and Pyroptosis in Liver Transplantation

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