Role of mean platelet volume in differential diagnosis of adult-onset Still's disease and sepsis

Neutrophils are innate immune phagocytes that play a key role in immune defense against invading pathogens. The main offensive mechanisms of neutrophils are the phagocytosis of pathogens, release of granules, and production of cytokines. The formation of neutrophil extracellular traps (NETs) has been described as a novel defense mechanism in the literature. NETs are a network of fibers assembled from chromatin deoxyribonucleic acid, histones, and neutrophil granule proteins that have the ability to kill pathogens, while they can also cause toxic effects in hosts. Activated neutrophils with NET formation stimulate autoimmune responses related to a wide range of inflammatory autoimmune diseases by exposing autoantigens in susceptible individuals. The association between increased NET formation and autoimmunity was first reported in antineutrophil cytoplasmic antibody-related vasculitis, and the role of NETs in various diseases, including systemic lupus erythematosus, rheumatoid arthritis, and psoriasis, has since been elucidated in research. Herein, we discuss the mechanistic role of neutrophils, including NETs, in the pathogenesis of systemic juvenile idiopathic arthritis (SJIA) and adult-onset Still’s disease (AOSD), and provide their clinical values as biomarkers for monitoring and prognosis.

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Interluekin-6 inhibitors for the treatment of adult-onset Still’s disease

Modern Rheumatology ◽

10.1093/mr/roab004 ◽

2021 ◽

Author(s):

Yuko Kaneko

Keyword(s):

Proinflammatory Cytokines ◽

Cytotoxic T Cells ◽

Adult Onset Still’S Disease ◽

Adult Onset ◽

Still’S Disease ◽

Still's Disease ◽

Systemic Inflammatory Disease ◽

Adult Onset Still's Disease ◽

Multifunctional Cytokine

ABSTRACT Adult-onset Still’s disease is a systemic inflammatory disease characterized by high spiking fever, arthritis, evanescent skin rash, leukocytosis, and hyperferritinemia. The pathogenesis of adult-onset Still’s disease has not been fully understood yet; however, multiple proinflammatory cytokines, such as IL-1β and IL-6, play important roles in the development of adult-onset Still’s disease. IL-6 is a multifunctional cytokine that accelerates the differentiation of macrophages and cytotoxic T-cells and chemotaxis of neutrophils and macrophages. Serum concentrations of IL-6 well correlate with disease activity of adult-onset Still’s disease, and blockade of IL-6 has been proven to be effective in active adult-onset Still’s disease. This review will focus on the recent understanding of the role of proinflammatory cytokines of adult-onset Still’s disease and the efficacy of IL-6 inhibitors for the treatment of adult-onset Still’s disease.

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Acute liver injury in a patient with adult-onset Still’s disease—the challenge of differential diagnosis

Oxford Medical Case Reports ◽

10.1093/omcr/omaa102 ◽

2020 ◽

Vol 2020 (11) ◽

Author(s):

Sabine Weber ◽

Alexander L Gerbes

Keyword(s):

Differential Diagnosis ◽

Liver Injury ◽

Acute Liver Injury ◽

Adult Onset Still’S Disease ◽

Adult Onset ◽

Still’S Disease ◽

Still's Disease ◽

Cardinal Symptoms ◽

Underlying Diseases ◽

Adult Onset Still's Disease

ABSTRACT In addition to the cardinal symptoms of fever, rash and arthralgia, liver involvement in patients with adult-onset Still’s disease (AOSD) has been described. However, acute liver injury in AOSD patients can have various other causes: it can be a result of an AOSD-induced macrophage activation syndrome or be associated to the drugs given for the underlying diseases and symptoms. Differential diagnosis can therefore be challenging. We here present a case of a 32-year-old male with acute liver injury following the initial diagnosis of AOSD to discuss the possible underlying reasons.

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Adult-onset Still's disease: an uncommon differential diagnosis of urticaria and treatment with anakinra

Journal of the European Academy of Dermatology and Venereology ◽

10.1111/j.1468-3083.2008.02746.x ◽

2008 ◽

Vol 0 (0) ◽

pp. 080422063954486-??? ◽

Cited By ~ 1

Author(s):

A Gesierich ◽

J Stoevesandt ◽

C Kneitz ◽

E-B Bröcker ◽

MP Schön

Keyword(s):

Differential Diagnosis ◽

Adult Onset Still’S Disease ◽

Adult Onset ◽

Still’S Disease ◽

Still's Disease ◽

Adult Onset Still's Disease

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Interleukin 18: A Biomarker for Differential Diagnosis Between Adult-onset Still’s Disease and Sepsis

The Journal of Rheumatology ◽

10.3899/jrheum.130575 ◽

2014 ◽

Vol 41 (6) ◽

pp. 1118-1123 ◽

Cited By ~ 37

Author(s):

Roberta Priori ◽

Serena Colafrancesco ◽

Cristiano Alessandri ◽

Antonina Minniti ◽

Carlo Perricone ◽

...

Keyword(s):

Differential Diagnosis ◽

Disease Activity ◽

Serum Levels ◽

Inactive Disease ◽

Adult Onset Still’S Disease ◽

Adult Onset ◽

Still’S Disease ◽

Still's Disease ◽

Yamaguchi Criteria ◽

Adult Onset Still's Disease

Objective.The differential diagnosis between rheumatic diseases and infectious conditions is a great challenge in clinical practice. Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory syndrome that shares several clinical and laboratory variables with sepsis. Interleukin (IL)-18 is overexpressed in AOSD, suggesting a possible role as a disease biomarker. The aim of our study was to detect IL-18 serum levels in a cohort of patients with AOSD and sepsis and to address its possible role as a biomarker for differential diagnosis.Methods.A group of unselected patients with AOSD diagnosed according to the Yamaguchi criteria and consecutive patients with sepsis diagnosed according to the American College of Chest Physicians/Society of Critical Care Medicine Consensus Conference criteria were enrolled. The clinical and laboratory data were collected. In the AOSD group, disease activity was assessed by Pouchot’s and Rau’s criteria. IL-18 serum levels were detected by ELISA.Results.Thirty-nine patients with AOSD and 18 patients with sepsis were enrolled. Two out of 18 patients with sepsis (11.1%) also fulfilled the Yamaguchi criteria. A significant difference was found in IL-18 serum levels between patients with active and inactive disease (p < 0.001), and it positively correlated with disease activity (p = 0.0003), ferritin serum level (p = 0.016), and erythrocyte sedimentation rate (p = 0.041). IL-18 was significantly increased in patients with AOSD when compared with sepsis (p = 0.014). For a cutoff of 148.9 pg/ml, this test had a specificity of 78.3% and a sensitivity of 88.6%.Conclusion.We have demonstrated that IL-18 can be a biomarker for differential diagnosis between AOSD and sepsis.

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Pneumonitis in Adult Onset Still’s Disease: Uncommon or Under Diagnosed?

Acta Médica Portuguesa ◽

10.20344/amp.7177 ◽

2017 ◽

Vol 30 (7-8) ◽

pp. 578 ◽

Cited By ~ 1

Author(s):

Silvia Fernandes ◽

Margarida Almeida ◽

José Alberto Pereira da Silva ◽

José Carlos Romeu

Keyword(s):

Differential Diagnosis ◽

Case Report ◽

Clinical Manifestations ◽

Adult Onset Still’S Disease ◽

Severe Respiratory Failure ◽

Adult Onset ◽

Still’S Disease ◽

Still's Disease ◽

Pulmonary Parenchyma ◽

Adult Onset Still's Disease

The adult onset Still’s Disease is an uncommon entity characterized by multiple clinical manifestations. Pneumonitis, less often considered, deserves particular emphasis given the need for differential diagnosis and because it can progress to severe respiratory failure. With the aim to highlight the pulmonary parenchyma involvement in patients with adult onset Still’s Disease, we present a case report which progresses with pneumonitis.

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