Adult-Onset Still's Disease in a Patient With a Previous Diagnosis of Acute Sarcoidosis

Ganhão, Sara; Ferreira, Raquel; Guerra, Miguel; Furtado, André Tosi; Águeda, Ana; Mariz, Eva; Bernardes, Miguel; Costa, Lúcia

doi:10.1097/rhu.0000000000001404

Cited by 3 publications

(2 citation statements)

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“…Similarly, an association between AOSD and other inflammatory diseases has been reported: Crohn's disease [53][54][55], sarcoidosis [56,57] and neutrophilic urticarial dermatosis (NUD) [58,59]. The latter is a rare form of dermatosis with clinical and biological features very similar to AOSD (high fever, skin eruption, arthralgia, biological inflammation, neutrophilic hyperleukocytosis).…”

Section: Aosd As a Said At The Crossroad Of Other (Auto) Inflammatory Disorders And Points Left Unresolvedmentioning

confidence: 88%

Clinical Phenotypes of Adult-Onset Still’s Disease: New Insights from Pathophysiology and Literature Findings

Mitrovic

Fautrel

2021

JCM

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Adult-onset Still’s disease (AOSD) is a non-familial, polygenic systemic autoinflammatory disorder. It is traditionally characterized by four cardinal manifestations—spiking fever, an evanescent salmon-pink maculopapular rash, arthralgia or arthritis and a white-blood-cell count (WBC) ≥ 10,000/mm3, mainly neutrophilic polymorphonuclear cells (PMNs)—but many other manifestations and complications can be associated, making clinical expression very heterogeneous and diagnosis sometimes difficult. The AOSD course can be diverse and is currently impossible to predict. Several clinical phenotypes have been described, either on the basis of the evolution of symptoms over time (monocyclic, polycyclic and chronic evolution) or according to dominant clinical evolution (systemic and arthritis subtypes). However, these patterns are mainly based on case series and not on robust epidemiological studies. Furthermore, they have mainly been established a long time ago, before the era of the biological treatments. Thus, based on our personal experience and on recent advances in the understanding of disease pathogenesis, it appears interesting to reshuffle AOSD phenotypes, emphasizing the continuum between AOSD profiles and other systemic autoinflammatory disorders, eventually proposing a research agenda.

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Section: Aosd As a Said At The Crossroad Of Other (Auto) Inflammatory Disorders And Points Left Unresolvedmentioning

confidence: 88%

Clinical Phenotypes of Adult-Onset Still’s Disease: New Insights from Pathophysiology and Literature Findings

Mitrovic

Fautrel

2021

JCM

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“…К ним можно отнести болезнь Стилла взрослых (БСВ) (пример комплекс ного САВЗ) [297], палиндромный ревматизм [298][299][300], «полимилгический» вариант РА у пожилых людей [301][302][303]. Описаны «перекресты» БСВ с анкилозирующим спондилоартритом и псоратическим артритом [304], семейной средиземноморской лихорадкой [305,306]; ассоциация БСВ c другими ИВЗ, включая болезнь Крона [307][308][309], саркоидоз [310,311], синдром VEXAS (vacuoles, E1 enzyme, X-linked, autoinfl ammatory, somatic) [312][313][314], синдром Шницлера [315,316], нейтрофильный уртикарный дерматоз [317,318], а также неклассифицируемого САВЗ, миелодиспластического синдрома и синдрома VEXAS [319]. У пациентов с серонегативным РА в полости сустава могут выявляться кристаллы мочевой кислоты, которые, активируя NLRP3-инфламмасому, индуци руют синтез ИЛ-1β [320].…”

Section: таблица 3 системные аутовоспалительные заболевания: общая ха...unclassified

The role of interleukin 1 in the development of human diseases: focus on Anakinra (IL-1 receptor antagonist)

Насонов

Samsonov

2022

Naučno-praktičeskaâ revmatologiâ

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According to modern concepts, human immune-mediated inflammatory diseases (IMIDs), depending on the prevailing mechanisms of immunopathogenesis, are divided into two main categories – autoimmune and autoinflammatory.At the same time, both autoimmune and autoinflammatory mechanisms are involved in the pathogenesis of most IMIDs, the complex interaction of which is reflected in the polymorphism of clinical manifestations, course variants, outcomes, and therapy efficacy. It is assumed that hyperproduction of cytokines of the interleukin (IL) 1 family, which is one of the key regulators of innate immunity, determines the “crossover” between the mechanisms of autoinflammation and autoimmunity in IMIDs. Anakinra is currently used in clinical practice to suppress the pathological effects of IL-1. An analysis of the results of the clinical use of Anakinra indicates that treatment with this drug should be considered as a promising direction in the pharmacotherapy of systemic autoinflammatory diseases (SAIDs) and critical conditions in children and adults associated with the development of hyperinflammation. The main directions of the Anakinra clinical research program are presented, including: determining the place of the drug in the implementation of the "Treat to Target" strategy and personalization of therapy, primarily in patients with “resistant” (difficult-to-treat) subtype of rheumatoid arthritis and comorbid pathology, as well as with severe forms of microcrystalline arthritis; the possibility of using Anakinra to improve the early diagnosis of SAIDs in children and adults; creation of the Russian register of patients with SAIDs, who are potentially indicated for treatment with Anakinra.

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Adult Still's disease: New horizons

Насонов

Feist

2021

Naučno-praktičeskaâ revmatologiâ

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Still's disease in children (systemic juvenile idiopathic arthritis - JIA) and adult Still's disease (ASD) are considered as systemic autoinflammatory diseases of unknown etiology, which are based on similar immunopathogenetic mechanisms associated with genetically determined disorders of the mechanisms of innate immunity. ASD was first described 50 years ago by the English rheumatologist Eric George Lapthorne Bywaters. The molecular basis of ASD immunopathogenesis is the activation of innate immunity associated with NLRP3 inflammasome-dependent mechanisms of inflammation, characterized by the overproduction of “pro-inflammatory” cytokines - interleukin (IL) 1 and IL-18, inducing the synthesis of other proinflammatory inflammatory mediators. A review of new data concerning the mechanisms of immunopathology, clinical polymorphism, laboratory biomarkers and the possibilities of ASD pharmacotherapy is presented.Particular attention is paid to the prospects for the use of monoclonal antibodies to IL-1β - canakinumab. The problems associated with the generality of clinical and laboratory disorders, pathogenetic mechanisms and pharmacotherapy of ASD and coronavirus disease 2019 (COVID-19) are considered.

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Adult-Onset Still's Disease in a Patient With a Previous Diagnosis of Acute Sarcoidosis

Cited by 3 publications

References 5 publications

Clinical Phenotypes of Adult-Onset Still’s Disease: New Insights from Pathophysiology and Literature Findings

Clinical Phenotypes of Adult-Onset Still’s Disease: New Insights from Pathophysiology and Literature Findings

The role of interleukin 1 in the development of human diseases: focus on Anakinra (IL-1 receptor antagonist)

Adult Still's disease: New horizons

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