Multi-organ failure in adult onset Still’s disease: a septic disguise

Walker, Emily; Aw, Darren; Marshall, David A.; Villiers, Francois De; Staber, M.

doi:10.1007/s10067-008-1010-1

Cited by 17 publications

(11 citation statements)

References 16 publications

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“…This is not the first report dealing with such confounding conclusion. Indeed, it was described in a febrile patient with AOSD without evidence of infection the presence of serum levels of PCT between 2 and 10 ng/ml [72]. Nonetheless, Chen et al investigated the role of PCT in febrile patients with AOSD achieving different results [73].…”

Section: Procalcitoninmentioning

confidence: 98%

See 1 more Smart Citation

Presentation and diagnosis of adult-onset Still’s disease: the implications of current and emerging markers in overcoming the diagnostic challenge

Colafrancesco¹,

Priori²,

Valesini³

2015

Expert Review of Clinical Immunology

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Adult-onset Still's disease (AOSD) is a known cause of fever of unknown origin. It is characterized by a triad of symptoms: spiking fever (>39°C), salmon-colored rash and arthritis/arthralgia. On a predisposing genetic background, several conditions may act as trigger for disease and among these, infectious agents are the most important. Nowadays, a dichotomous view of AOSD has been introduced which distinguishes this entity in two subsets according to the clinical features and laboratory aspects: systemic or articular. As AOSD is a diagnosis of exclusion, specific biomarkers able to facilitate differential diagnosis are needed. A number of possible biomarkers have been proposed that will be discussed in detail in this review: ferritin, IL-18, procalcitonin, s100 proteins and sCD163.

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Section: Procalcitoninmentioning

confidence: 98%

“…[60] S100A8/A9 11,770 ± 8840 ng/ml 36 [85] 57.11 ± 25.38 ng/ml 25 [84] 0.5-9 ng/ml 18 [35] S100A12 547.9 ± 148.4 ng/ml 37 [86] Procalcitonin 0.09-71 ng/ml 5 [70] 2-10 ng/ml 1 [72] 0-1.4 ng/ml 38 [73] SD: Standard deviations.…”

Section: Biomarkersmentioning

confidence: 99%

Presentation and diagnosis of adult-onset Still’s disease: the implications of current and emerging markers in overcoming the diagnostic challenge

Colafrancesco¹,

Priori²,

Valesini³

2015

Expert Review of Clinical Immunology

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“…[25,28–33] However, it remains unknown whether tocilizumab would be effective for SIRS complicating AOSD. Although SIRS complicating AOSD is extremely rare and mild cases are responsive to high-dose steroids, [8,34] it can also be fatal. [6] We searched for MEDLINE from January 1, 1991 to December 1, 2016 using a search strategy with the terms “Adult onset Still disease” and “Systemic inflammatory response syndrome,” and/or “edema” and/or “shock”.…”

Section: Discussionmentioning

confidence: 99%

Tocilizumab for uncontrollable systemic inflammatory response syndrome complicating adult-onset Still disease

Masui-Ito

Okamoto²,

Ikejiri³

et al. 2017

Medicine

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Rationale:Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown etiology characterized by evanescent salmon-pink rash, fever spikes, arthralgia, and lymphadenopathy. AOSD usually has a good prognosis, but it can sometimes be fatal, especially when it is complicated by systemic inflammatory response syndrome (SIRS) and multiple organ failure.Patient concerns:A previously healthy 26-year-old woman was referred to our hospital for persistent high fever and mild systemic edema. Five days later, the patient presented with dyspnea, hypotension, and anuria. Anasarca developed with massive pleural effusion, ascites, and systemic edema, resulting in an increase of 47 kg in body weight.Diagnoses:The patient was diagnosed as AOSD after infection, malignancy, hematologic disorders, and other autoimmune diseases were excluded.Interventions:We administered tocilizumab, an IL-6 receptor inhibitor, intravenously in addition to cyclosporine, prednisolone, plasma exchange, and continuous hemodiafiltration.Outcomes:The patient's systemic condition improved. After stabilization by all medications, the patient was managed and responded to tocilizumab alone. To the best of our knowledge, this was the first case of severe SIRS complicating AOSD that was successfully treated with an anti- IL-6 receptor antibody.Lessons:SIRS should not be overlooked in a patient with steroid-resistant AOSD and edema. Inhibitors of the IL-6 receptor can be used safely and effectively to control AOSD complicated with severe SIRS.

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“…DIC is one of the most dramatic events in AOSD and is a possible cause of death [2,18,20,22,28] although complete recovery in several patients has been reported [2,14,22,35,42,[45][46][47][48][49][50]. It can follow or be associated with reactive haematophagocytic syndrome (RHS), a further possible life-threatening complication in AODS [15,17,21,22,43,51].…”

Section: Casementioning

confidence: 99%

“…Three other cases of fatal AOSD and ARDS have been described in one case associated with RHS [22,25,26]. Myocarditis [14,16,20,35,46,[53][54][55], and in particular cardiac tamponade [45], can also very rarely complicate the course of AOSD, more frequently appearing in the paediatric form. Indeed, in SoJIA, myocarditis is a well-known complication often associated with pericardial effusion, which can lead to congestive heart failure and arrhythmias.…”

Section: Casementioning

confidence: 99%

Adult-onset Still disease: a rare disorder with a potentially fatal outcome

Priori

Colafrancesco

Gattamelata

et al. 2010

Autoimmun Highlights

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Purpose The aim of this study was to assess the clinical and laboratory features of a cohort of Italian patients with adult-onset Still disease (AOSD) with particular attention on possible life-threatening complications. Methods The clinical charts of 41 consecutive Italian patients with AOSD referred to our rheumatological department over the last 10 years were retrospectively examined. Data regarding clinical manifestations, laboratory features and complications were collected and compared with those reported in literature. Results The most frequent manifestations were: fever (90.2%), arthralgias (80.4%), skin rash (75.6%), sore throat (53.6%), arthritis (51.2%), lymphadenopathy (48.7%), hepatosplenomegaly (41.4%), myalgia (21.9%), fatigue (12%), diarrhoea and vomiting (9.7%), pleural effusion (9.7%), pericardial effusion (4.8%) and abdominal pain (2.4%). In two patients whose cases are described in detail; the course of the disease was complicated by disseminated intravascular coagulopathy, in one patient with a fatal outcome. ESR, CRP and leucocyte count mean values were 69.41 mm/h, 69.05 mg/l and 18,798.5 cell/mm 3 (neutrophils 84.64%), respectively. Serum ferritin levels were increased in 48.7% of patients while transaminases were elevated in 42.6% of patients (71% considering only patients in an active phase of disease). Conclusion The results of this study are in line with those reported for other cohorts of patients. Even if the prognosis of AOSD is considered favourable, the present study indicates that the disease is a troubling condition needing prompt intervention. Occasionally, AOSD may rapidly worsen with life-threatening events.

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Multi-organ failure in adult onset Still’s disease: a septic disguise

Cited by 17 publications

References 16 publications

Presentation and diagnosis of adult-onset Still’s disease: the implications of current and emerging markers in overcoming the diagnostic challenge

Presentation and diagnosis of adult-onset Still’s disease: the implications of current and emerging markers in overcoming the diagnostic challenge

Tocilizumab for uncontrollable systemic inflammatory response syndrome complicating adult-onset Still disease

Adult-onset Still disease: a rare disorder with a potentially fatal outcome

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