Clinical characteristics and treatment of elderly onset adult-onset Still’s disease

Kishida, Dai; Ichikawa, Takanori; Takamatsu, Ryota; Nomura, Shun; Matsuda, Masayuki; Ishii, Wataru; Nagai, Tatsuo; Suzuki, Sho; Ueno, Kenichi; Tachibana, Naoki; Shimojima, Yasuhiro; Sekijima, Yoshiki

doi:10.1038/s41598-022-10932-3

Cited by 13 publications

(14 citation statements)

References 36 publications

(42 reference statements)

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“…An increased rate of mortality in elderly onset AOSD patients was observed in previous studies [23,24], and Suda et al also reported the more frequent presence of DIC in AOSD patients over 70 years old [42]. Moreover, although the pleuritis was not related to death in previous studies, Maruyama et al and Kishida et al showed an increased frequency of pleuritis in the elderly onset AOSD patients [22,23]. Thus, the elderly onset AOSD patients may characterized with worse prognosis including higher mortality and higher frequency of complications.…”

Section: Discussionmentioning

confidence: 81%

“…The outcome and treatment were not signi cantly different in two groups, however, AOSD patients with MAS had an increased prevalence of relapse. To our knowledge, our research is one of the largest studies to investigate the different clinical features and outcome of elderly onset AOSD patients [22][23][24].…”

Section: Discussionmentioning

confidence: 99%

“…Recently more studies focused on the elderly onset AOSD patients and found the increase of the proportion of them. The rst study in 2021 demonstrated that 33.1% of the of Japanese AOSD patients were older than 60 years [23], and the second study showed that 41.9% of the of Japanese AOSD patients were older than 65 years in 2022 [22]. In Italy, 16.7% AOSD patients had an onset of the disease aged over than 60 years during the last twenty years [24].…”

Section: Discussionmentioning

confidence: 99%

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Clinical characteristics and outcome of elderly onset adult-onset Still’s disease: A 10-year retrospective study

Ying

Bai

et al. 2022

Preprint

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Objective: Our objective was to retrospectively analyze the clinical characteristics and outcome of adult-onset Still’s disease (AOSD) patients with elderly onset. Methods: Retrospective data of patients diagnosed with AOSD in our institute during 2013-2021 were analyzed. The diagnoses were based on the Yamaguchi criteria for AOSD. All long-term follow-up data were collected from medical records and phone calls. Results: In total, 281 AOSD patients were enrolled in this study, with the median follow-up interval of 47 months. Thirty-two (11.4%, ≥65 years) AOSD patients were classified into the elderly onset groups. Compared to the younger onset group, the percentage of patients with skin rash (p=0.047), sore throat (p=0.001), myalgia (p=0.001), splenomegaly (p=0.039), hepatosplenomegaly (p=0.002) and the Pouchot’s score (p=0.002) were significantly lower in the elderly onset group. The death rate (p=0.014) of elderly onset group is higher than younger onset group, and the independent risk factors of mortality in all AOSD patients were age at onset (HR: 1.115, p=0.044), disseminated intravascular coagulation (HR: 391.576, p=0.001) and pleuritis (HR: 23.162, p=0.033). The probability of relapse was significantly increased in the patients with macrophage activation syndrome (MAS) compared with the patients without MAS (p<0.001), though the different age groups of AOSD patients with MAS showed no difference in the probability of relapse (p=0.737). Conclusion: Elderly onset AOSD patients were distinguished by several distinct clinical features compared to younger onset AOSD patients. The frequency of relapse and complications were similar to that of AOSD patients with elderly or younger onset. A higher mortality rate was observed in elderly onset AOSD patients, and the mortality of AOSD patients was related to age at onset, DIC and pleuritis. Furthermore, AOSD patients accompanied with MAS have a higher frequency of relapse, with no difference between age groups.

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Section: Discussionmentioning

confidence: 81%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Clinical characteristics and outcome of elderly onset adult-onset Still’s disease: A 10-year retrospective study

Ying

Bai

et al. 2022

Preprint

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“… 49–51 Further work has been done to highlight the clinical heterogeneity of AOSD, with a study showing they may fall into four distinct phenotypes, each characterised by a single prominent clinical feature (high CRP, high ferritin, high systemic score, low CRP/ferritin), raising the possibility of risk stratification and targeted therapies in future. 52 …”

Section: Adult Onset Still’s Diseasementioning

confidence: 99%

“…[49][50][51] Further work has been done to highlight the clinical heterogeneity of AOSD, with a study showing they may fall into four distinct phenotypes, each characterised by a single prominent clinical feature (high CRP, high ferritin, high systemic score, low CRP/ferritin), raising the possibility of risk stratification and targeted therapies in future. 52 When comparing presentation by age, those that develop AOSD aged 65 or above similarly present with fever, arthralgia, and skin rashes, though are less likely to develop pharyngitis and more likely to develop pleuritis. 53,54 Those with an elderly onset are 3-5-times more likely to develop life-threatening complications such as disseminated intravascular coagulopathy (DIC) and MAS, highlighting the need for prompt recognition and management in this population.…”

Section: Adult Onset Still's Diseasementioning

confidence: 99%

Improving Diagnosis and Clinical Management of Acquired Systemic Autoinflammatory Diseases

Al‐Hakim¹,

Mistry²,

Savic³

2022

JIR

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Systemic autoinflammatory diseases (SAID) are conditions caused by dysregulation or disturbance of the innate immune system, with neutrophils and macrophages the main effector cells. Although there are now more than 40 distinct, genetically defined SAIDs, the genetic/molecular diagnosis remains unknown for a significant proportion of patients with the disease onset in adulthood. This review focuses on new developments related to acquired/late onset SAID, including phenocopies of monogenic disorders, Schnitzler’s syndrome, Adult onset Still’s disease, VEXAS syndrome, and autoinflammatory complications associated with myelodysplastic syndrome.

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Clinical and laboratory features associated with macrophage activation syndrome in Still’s disease: data from the international AIDA Network Still’s Disease Registry

Triggianese,

Vitale,

Lopalco

et al. 2023

Intern Emerg Med

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To characterize clinical and laboratory signs of patients with Still’s disease experiencing macrophage activation syndrome (MAS) and identify factors associated with MAS development. Patients with Still’s disease classified according to internationally accepted criteria were enrolled in the AutoInflammatory Disease Alliance (AIDA) Still’s Disease Registry. Clinical and laboratory features observed during the inflammatory attack complicated by MAS were included in univariate and multivariate logistic regression analysis to identify factors associated to MAS development. A total of 414 patients with Still’s disease were included; 39 (9.4%) of them developed MAS during clinical history. At univariate analyses, the following variables were significantly associated with MAS: classification of arthritis based on the number of joints involved (p = 0.003), liver involvement (p = 0.04), hepatomegaly (p = 0.02), hepatic failure (p = 0.01), axillary lymphadenopathy (p = 0.04), pneumonia (p = 0.03), acute respiratory distress syndrome (p < 0.001), platelet abnormalities (p < 0.001), high serum ferritin levels (p = 0.009), abnormal liver function tests (p = 0.009), hypoalbuminemia (p = 0.002), increased LDH (p = 0.001), and LDH serum levels (p < 0.001). At multivariate analysis, hepatomegaly (OR 8.7, 95% CI 1.9–52.6, p = 0.007) and monoarthritis (OR 15.8, 95% CI 2.9–97.1, p = 0.001), were directly associated with MAS, while the decade of life at Still’s disease onset (OR 0.6, 95% CI 0.4–0.9, p = 0.045), a normal platelet count (OR 0.1, 95% CI 0.01–0.8, p = 0.034) or thrombocytosis (OR 0.01, 95% CI 0.0–0.2, p = 0.008) resulted to be protective. Clinical and laboratory factors associated with MAS development have been identified in a large cohort of patients based on real-life data.

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Clinical characteristics and treatment of elderly onset adult-onset Still’s disease

Cited by 13 publications

References 36 publications

Clinical characteristics and outcome of elderly onset adult-onset Still’s disease: A 10-year retrospective study

Clinical characteristics and outcome of elderly onset adult-onset Still’s disease: A 10-year retrospective study

Improving Diagnosis and Clinical Management of Acquired Systemic Autoinflammatory Diseases

Clinical and laboratory features associated with macrophage activation syndrome in Still’s disease: data from the international AIDA Network Still’s Disease Registry

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