A Case of Adult-onset Still's Disease with Thrombotic Microangiopathy-like Disorder Successfully Treated by Plasma Exchange and Anti-TNF Therapy.

Seri, Yu; Shoda, Hirofumi; Iwasaki, Yukiko; Okamoto, Akiko; Hagino, Noboru; Kawahata, K.; Yamamoto, Kazuhiko

doi:10.2169/naika.100.1963

Cited by 2 publications

(6 citation statements)

References 10 publications

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“…The most recent literature review was published in 2009 and included a total of 15 cases . An additional five cases have been published since this review 8‐12. Our patient's clinical presentation was similar to what had been previously reported.…”

Section: Discussionsupporting

confidence: 78%

“…Just like our patient, most patients were treated with a combination of immunosuppression (mainly, corticosteroids) and TPE. In a few cases, IL‐1 receptor antagonist (Anakinra), anti‐TNF‐α (etanercept), and anti‐IL‐6 (tosilizumab) were also used. To our knowledge, this is the first report of using eculizumab to treat TMA associated with AOSD.…”

Section: Discussionmentioning

confidence: 99%

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Thrombotic microangiopathy in a patient with adult‐onset Still's disease

et al. 2014

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Work-up of a new fragmentation hemolytic anemia and thrombocytopenia should include careful review of past history, including medications, as well as relevant laboratory investigations with aim to establish a correct diagnosis. Occasionally, the correct diagnosis is not the obvious one and there could be multiple contributors to the pathogenesis. Establishing diagnosis is important for counseling patient on disease prognosis and to guide treatment.

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Section: Discussionsupporting

confidence: 78%

Section: Discussionmentioning

confidence: 99%

Thrombotic microangiopathy in a patient with adult‐onset Still's disease

et al. 2014

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“…the use of biologics (83.33%) report either death 36 or PR with permanent complications, while only one reported complete remission. 30 These findings are important in the context of drug-induced TMA as 5/6 patients who used biologics had poor outcomes, emphasizing Mitrovic and Fautrel's 56 recommendations.…”

Section: Discussionmentioning

confidence: 94%

“…57 In our review, we not only analyzed studies involving standard therapies for AOSD, but also explored the use of specific interventions such as anakinra, 27 etanercept/tocilizumab, 29 eculizumab, 35,37 and infliximab. 30,36 Of these studies, 5/6 who report T A B L E 1 Characteristics of patients with AOSD and concurrent TMA. the use of biologics (83.33%) report either death 36 or PR with permanent complications, while only one reported complete remission.…”

Section: Discussionmentioning

confidence: 99%

“…In our review, we not only analyzed studies involving standard therapies for AOSD, but also explored the use of specific interventions such as anakinra, 27 etanercept/tocilizumab, 29 eculizumab, 35,37 and infliximab 30,36 . Of these studies, 5/6 who report the use of biologics (83.33%) report either death 36 or PR with permanent complications, while only one reported complete remission 30 . These findings are important in the context of drug‐induced TMA as 5/6 patients who used biologics had poor outcomes, emphasizing Mitrovic and Fautrel's 56 recommendations.…”

Section: Discussionmentioning

confidence: 99%

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Adult‐onset Still's disease with concurrent thrombotic microangiopathy: Observations from pooled analysis for an uncommon finding

Ananthaneni,

Shimkus,

Weis

et al. 2023

European J of Haematology

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BackgroundAdult‐onset Still's disease (AOSD) is a rare systemic inflammatory disorder that is characterized by quotidian fevers, arthritis, and an evanescent rash. Occurrence of concurrent thrombotic microangiopathy (TMA) in AOSD is rare. The treatment aspects of TMA in AOSD are actively being debated.MethodsMedline search using MeSH terms and snowballing yielded a total of 29 articles with co‐occurrence of AOSD and thrombotic thrombocytopenic purpura (TTP) including our own. Pooled data were synthesized for descriptive analysis.ResultsMedian age was 35 years with a majority of females (68.96%). A majority of these studies/patients were either Asian (34.48%) or Caucasian (31.03%). Concurrent TMA at the time of AOSD diagnosis was seen in 65.51% patients. Only 3/29 patients had ADAMTS13 level less than 10%, consistent with TTP and 3/29 were diagnosed with hemolytic uremic syndrome (HUS). The remainder were diagnosed clinically. Complication rate was high, and 15/29 (51.72%) patients died or had permanent neurological/renal/vision/gangrenous complications. Median and mean ferritin peak was observed to be higher (7458 and 12 349, respectively) in patients who either died/had partial remission, compared to those who had complete response (3257 and 10 899, respectively), p = .829.ConclusionsA majority of patients with AOSD‐associated TMA either died or had permanent complications. TMA was diagnosed alongside AOSD in 65% patients, while the rest developed TMA during the course of their disease. Blurred vision may precede TMA and could help risk‐stratify high‐risk AOSD patients clinically. Glycosylated ferritin remains low several weeks to months after disease remission and may be used to monitor severity of disease process. Further studies are necessary to confirm the existing vascular endothelial growth factor hypothesis in AOSD‐associated TMA.

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A Case of Adult-onset Still's Disease with Thrombotic Microangiopathy-like Disorder Successfully Treated by Plasma Exchange and Anti-TNF Therapy.

Cited by 2 publications

References 10 publications

Thrombotic microangiopathy in a patient with adult‐onset Still's disease

Thrombotic microangiopathy in a patient with adult‐onset Still's disease

Adult‐onset Still's disease with concurrent thrombotic microangiopathy: Observations from pooled analysis for an uncommon finding

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