Scoring Adult-onset Still’s Disease: Table 1.

Mueller, Ruediger B; Sheriff, Ahmed

doi:10.3899/jrheum.100783

Cited by 8 publications

(4 citation statements)

References 7 publications

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“…Major criteria in our patient include a fever greater than 39 C (102.2 F) for at least one week, arthralgias (2 weeks or longer), salmon-colored rash over extremities noted during a febrile episode, and leukocytosis [3, 4]. He also demonstrated several minor criteria including sore throat, elevated liver enzymes and LDH, and negative rheumatoid factor.…”

Section: Discussionmentioning

confidence: 99%

Adult-Onset Still’s Disease Masquerading as Sepsis in an Asplenic Active Duty Soldier

Jaqua

Finger²,

Hawley³

2012

Case Reports in Medicine

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This is a case of a 26-year-old active duty male with a history of idiopathic thrombocytopenic purpura (ITP) and surgical asplenia who presented with a one-week history of fevers, myalgias, arthralgias, and rigors. His evaluation upon presentation was significant for a temperature of 103 degrees F, white blood cell count of 36 K with a granulocytic predominance, and elevated transaminases. He was treated empirically with broad-spectrum antibiotics with concern for a systemic infection with an encapsulated organism. During his stay, he developed four SIRS criteria and was transferred to the progressive care unit for suspected sepsis. He continued to have twice-daily fevers and a faint, salmon-colored centripetal rash was eventually observed during his febrile episodes. After a nondiagnostic microbiologic and serologic workup, he was diagnosed with adult-onset Still's Disease and started on intravenous methylprednisolone with brisk response. He was discharged on oral prednisone and was started on anakinra. Adult-onset Still's disease is a rare condition that presents with varying severity, and this is the first reported case, to our knowledge, of its diagnosis in an asplenic patient. Its management in the setting of asplenia is complicated by the need for antibiotic therapy with each episode of fever.

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Section: Discussionmentioning

confidence: 99%

Adult-Onset Still’s Disease Masquerading as Sepsis in an Asplenic Active Duty Soldier

Jaqua

Finger²,

Hawley³

2012

Case Reports in Medicine

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“…The disease can be asymptomatic for a long time, or it may present a full range of classic symptoms supported with laboratory and imaging study results [ 3 – 6 ]. Often a long time can elapse before a patient is consulted by a rheumatologist.…”

Section: Commentmentioning

confidence: 99%

“…Adult-onset Still's disease can be diagnosed based on the classification criteria established by Yamaguchi et al This approach was used in the reported case with clinical symptoms and results of the laboratory tests considered [ 6 ]. Analyzing the above criteria, the patient met 3 out of 4 main criteria and 2 out of 4 minor criteria, where 5 criteria in total (minimum of 2 major ones) are sufficient for the diagnosis.…”

Section: Commentmentioning

confidence: 99%

Adult-onset Still’s disease as a mask of Hodgkin lymphoma

Dudziec¹,

Pawlak-Buś²,

Leszczyński³

2015

Reumatologia

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Adult-onset Still's disease is a rare disorder, which creates difficulties in making a proper diagnosis. Ambiguous symptoms and results of auxiliary tests, lack of unequivocal diagnostic tests and the need to exclude other causes of the disease are major problems in clinical practice.A case of a 22-year-old woman with dominated recurrent fever, significantly elevated inflammation markers and arthritis is presented. Based on clinical signs after exclusion of infection, hematological and other reasons, the patient was diagnosed with adult-onset Still's disease. Standard treatment, with high doses of glucocorticoids and a disease-modifying drug, was applied, without the anticipated effects. The diagnostic tests were conducted again due to the lack of clinical improvement, increase of inflammatory markers and unusual response to treatment. A new symptom of significance, i.e. mediastinal lymphadenopathy, was found. After the histopathological examination of lymph nodes, Hodgkin's disease was diagnosed and targeted therapy for hematological malignancy was applied.

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“…Sepsis is a life-threatening complex condition that results in a dysregulated host response to severe infection, acute organ dysfunction, and high morbidity and mortality in hospitals ( 6 ); clinical characteristics and symptoms overlap with AOSD, and therefore in some cases it is difficult to differentiate between these conditions. However, the treatment plans for AOSD and sepsis are very different: sepsis may be treated with antimicrobial agents, while AOSD may require immunosuppressive therapy, which can reduce immune resistance and cause or aggravate infection in sepsis patients ( 7 ). Early identification of AOSD is therefore vital for efficient and successful clinical management of this disease.…”

Section: Introductionmentioning

confidence: 99%

Serum Heparin-Binding Protein as a Potential Biomarker to Distinguish Adult-Onset Still’s Disease From Sepsis

et al. 2021

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Adult-onset Still’s disease (AOSD) is a systemic, multifactorial, autoinflammatory disease for which the etiopathogenesis is not well understood. Given the similarities in clinical and laboratory features between this disease and sepsis, and the differences in treatment strategies for these two diseases, specific diagnostic markers are crucial for the correct diagnosis and management of AOSD. Previous studies have shown plasma heparin-binding protein (HBP) is a promising potential biomarker for AOSD; thus, this study aimed to detect serum HBP levels in patients with AOSD or sepsis to assess its potential as a biomarker for differential diagnosis. We found that serum HBP levels were significantly higher in patients with active AOSD than that in those with inactive AOSD. Patients with sepsis had higher serum HBP levels compared with those who had active or inactive AOSD. We calculated the area under the receiver operating characteristic (ROC) curve to assess whether HBP could be used to differentiate active from inactive AOSD; this was 0.811 with sensitivity 0.650, specificity 0.811, and cutoff HBP value of 35.59 ng/ml. The area under the ROC curve for HBP as a biomarker to differentiate AOSD from sepsis was 0.653, with sensitivity 0.759, and specificity 0.552, and cutoff HBP value of 65.1 ng/ml. Taken together, the results of our study suggest that serum HBP could be a useful diagnostic biomarker to evaluate disease activity in patients with AOSD, and to differentiate AOSD from sepsis.

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Scoring Adult-onset Still’s Disease: Table 1.

Cited by 8 publications

References 7 publications

Adult-Onset Still’s Disease Masquerading as Sepsis in an Asplenic Active Duty Soldier

Adult-Onset Still’s Disease Masquerading as Sepsis in an Asplenic Active Duty Soldier

Adult-onset Still’s disease as a mask of Hodgkin lymphoma

Serum Heparin-Binding Protein as a Potential Biomarker to Distinguish Adult-Onset Still’s Disease From Sepsis

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