Coexistence of sarcoidosis and adult onset Still disease

Semiz, Hüseyin; Kobak, Şenol

doi:10.1016/j.reuma.2017.04.004

Cited by 12 publications

(7 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…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: 87%

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

Mitrovic

Fautrel

2021

JCM

View full text Add to dashboard Cite

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.

show abstract

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

confidence: 87%

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

Mitrovic

Fautrel

2021

JCM

View full text Add to dashboard Cite

show abstract

“…Despite robust evidence on the role of IL-6 in the pathogenesis of sarcoidosis, data on the use of anti-IL-6 agents in sarcoidosis are scarce. Previous case reports showed clinical improvement under TCZ in patients with chronic sarcoidosis associated with Castleman's disease (29) and Still's disease (30). In a case series of four patients with refractory chronic sarcoidosis the authors reported dramatic responses to TCZ with improved symptoms and organ function allowing steroid tapering (31).…”

Section: Interleukin-6 Blockingmentioning

confidence: 89%

Emerging Molecular Targets for the Treatment of Refractory Sarcoidosis

et al. 2020

View full text Add to dashboard Cite

Sarcoidosis is a multisystem granulomatous disease of unknown origin that has variable clinical course and can affect nearly any organ. It has a chronic course in about 25% of patients. Corticosteroids (CS) are the cornerstone of therapy but their long-term use is associated with cumulative toxicity. Commonly used CS-sparing agents include methotrexate, cyclophosphamide, azathioprine, and mycophenolate mofetil. Twenty to forty percentage of sarcoidosis patients are refractory to these therapies or develop severe adverse events. Therefore, additional and targeted CS-sparing agents are needed for chronic sarcoidosis. Macrophage activation, interferon response, and formation of the granuloma are mainly mediated by T helper-1 responses. Different pro-inflammatory cytokines such as interleukin (IL)-8, IL-12, IL-6, and tumor necrosis factor-alpha (TNF-α) have been shown to be highly expressed in sarcoidosis-affected tissues. As a result of increased production of these cytokines, Janus kinase-signal transducer and activator of transcription (JAK-STAT) signaling is constitutively active in sarcoidosis. Several studies of biological agents that target TNF-α have reported their efficacy and appear today as a second line option in refractory sarcoidosis. Some case series report a positive effect of tocilizumab an anti-IL-6 monoclonal antibody in this setting. More recently, JAK inhibition appears as a new promising strategy. This review highlights key advances on the management of chronic refractory sarcoidosis. Novel therapeutic strategies and treatment agents to manage the disease are described.

show abstract

“…As SD diagnosis is based on the exclusion of multiple differential diagnoses, some signs or symptoms associated with vasculitis may overlap with those of SD. In addition, lymph node enlargement may also challenge the diagnosis [ 41 ]. Very recently, a new entity with vasculitis, skin, and joint involvement has been added to the list of differential diagnoses of SD, namely VEXAS syndrome [ 42 ].…”

Section: Discussionmentioning

confidence: 99%

Still’s Disease Mortality Trends in France, 1979–2016: A Multiple-Cause-of-Death Study

Borciuch

Fauvernier

Gerfaud‐Valentin

et al. 2021

JCM

View full text Add to dashboard Cite

Still’s disease (SD) is often considered a benign disease, with low mortality rates. However, few studies have investigated SD mortality and its causes and most of these have been single-center cohort studies. We sought to examine mortality rates and causes of death among French decedents with SD. We performed a multiple-cause-of-death analysis on data collected between 1979 and 2016 by the French Epidemiological Center for the Medical Causes of Death. SD-related mortality rates were calculated and compared with the general population (observed/expected ratios, O/E). A total of 289 death certificates mentioned SD as the underlying cause of death (UCD) (n = 154) or as a non-underlying causes of death (NUCD) (n = 135). Over the study period, the mean age at death was 55.3 years (vs. 75.5 years in the general population), with differences depending on the period analyzed. The age-standardized mortality rate was 0.13/million person-years and was not different between men and women. When SD was the UCD, the most frequent associated causes were cardiovascular diseases (n = 29, 18.8%), infections (n = 25, 16.2%), and blood disorders (n = 11, 7.1%), including six cases (54%) with macrophage activation syndrome. As compared to the general population, SD decedents aged <45 years were more likely to die from a cardiovascular event (O/E = 3.41, p < 0.01); decedents at all ages were more likely to die from infection (O/E = 7.96–13.02, p < 0.001).

show abstract

Coexistence of sarcoidosis and adult onset Still disease

Cited by 12 publications

References 12 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

Emerging Molecular Targets for the Treatment of Refractory Sarcoidosis

Still’s Disease Mortality Trends in France, 1979–2016: A Multiple-Cause-of-Death Study

Contact Info

Product

Resources

About