Successful Use of Higher-Dose Etanercept for Multirefractory Systemic Flare of Adult-Onset Still’s Disease with Liver Failure with No Response to Tocilizumab Therapy

Naniwa, Taio; Tamechika, Shinya; Iwagaitsu, Shiho; Maeda, Shinji; Togawa, H.

doi:10.1155/2013/923497

Cited by 7 publications

(11 citation statements)

References 12 publications

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“…Anti-interleukin 1 (IL-1Ra and anakinra) [ 38 ], anti-interleukin 6 (tocilizumab) [ 53 ], anti- IL1β (canakinumab) [ 54 ], and TNF-α inhibitors (infliximab, etanercept, and adalimumab) [ 55 ] are other newly developed biological treatments which have been shown to be effective in refractory AOSD [ 6 , 7 ]. Overall, either monotherapy or combination therapy can be used to treat AOSD based on the disease course and symptoms severity.…”

Section: Discussionmentioning

confidence: 99%

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Adult-Onset Still’s Disease: Still a Serious Health Problem (a Case Report and Literature Review)

et al. 2017

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Patient: Female, 53Final Diagnosis: Adult-onset Still’s DiseaseSymptoms: Abdominal pain • feverMedication: —Clinical Procedure: —Specialty: RheumatologyObjective:Rare diseaseBackground:Adult-onset Still’s Disease (AOSD) is a rare systemic inflammatory disease accompanied by a triad of spiking fever, maculopapular exanthema, and arthralgia. To date, there is no definite laboratory or imaging test available for diagnosing AOSD, and the diagnosis is one of exclusion, which can be very challenging.Case Report:We report on the case of a 53-year-old female who presented with fever, arthralgia, and abdominal pain. Her initial laboratory tests showed elevated AST and ALT, and normal leukocytes with bandemia. During her hospitalization, we evaluated the patient for other potential differential diagnoses. After an extensive workup, the patient was diagnosed with AOSD based on Yamaguchi criteria. Her serum ferritin levels were measured and found to be markedly elevated, which is a non-specific finding in AOSD patients.Conclusions:This case highlights the important role of a detailed history and physical examination for timely diagnosis of AOSD to prevent complications and improve patient’s prognosis.

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Section: Discussionmentioning

confidence: 99%

“…Intravenous immunoglobulins (IVIGs) could be used to treat an acute AOSD presentation [ 5 ]. Anti TNF-α and anti-interleukins have also been shown to be beneficial in treatment of refractory AOSD [ 6 , 7 ].…”

Section: Introductionmentioning

confidence: 99%

Adult-Onset Still’s Disease: Still a Serious Health Problem (a Case Report and Literature Review)

et al. 2017

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“…AOSD usually has a good prognosis, but can be life-threatening, [ 5 ] especially when it is complicated with multiple organ failure and DIC caused by SIRS, [ 6 – 10 ] hemophagocytic syndrome, [ 11 – 13 ] thrombotic thrombocytopenic purpura, [ 5 ] acute respiratory distress syndrome, [ 14 ] diffuse alveolar hemorrhage, [ 15 ] pulmonary arterial hypertension, [ 16 ] and liver failure. [ 17 ] Therefore, these severe complications should not be overlooked when we see patients with AOSD.…”

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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“…Six patients were classified in the MAS group and 8 in the non-MAS group. From the start of tocilizumab treatment, 4 developed MAS features after approximately 1 week (range 0.7-2.1 weeks, early-onset MAS) 13,16,17 and the others after 20.6 and 61 weeks (late-onset MAS). These patients had no other obvious triggers or underlying disease for MAS, such as malignancy or infection.…”

Section: Clinical Characteristics Of the Study Patientsmentioning

confidence: 99%

“…10,11 Similar to sJIA patients, MAS or HLH cases have been reported in AOSD patients during IL-6 inhibitor therapy. [12][13][14][15][16][17] In addition, there have been reports of AOSD patients who developed MAS immediately after starting tocilizumab treatment, suggesting that IL-6 inhibition may have induced this severe complication. 13,15,16,17 On the other hand, early diagnosis of MAS in patients receiving IL-6 inhibitors may be challenging because IL-6 inhibition alters key clinical and laboratory features of MAS, 11 and the incidence of this serious complication may be underestimated.…”

Section: Introductionmentioning

confidence: 99%

Baseline clinical features predicting macrophage activation syndrome in patients with systemic adult‐onset Still’s disease receiving interleukin‐6 inhibitor treatment

et al. 2022

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Aim Macrophage activation syndrome (MAS), a severe complication of systemic adult‐onset Still’s disease (AOSD), has been reported to occur during interleukin‐6 (IL‐6) inhibitor treatment. However, predictors for MAS development are unknown. Therefore, this study investigated predictive features for MAS development after starting IL‐6 inhibitor treatment in systemic AOSD patients. Method In a single‐center retrospective study involving systemic AOSD patients who were refractory to high‐dose glucocorticoids with immunosuppressants and started IL‐6 inhibitor treatment between April 2008 and March 2020, we compared the baseline clinical features between patients who developed AOSD flare with MAS features (MAS group) and those who did not (non‐MAS group) during IL‐6 inhibitor treatment. Results Only tocilizumab was used as an IL‐6 inhibitor. Six of 14 refractory systemic AOSD patients developed AOSD flares with MAS features during tocilizumab treatment, including 4 who developed them shortly after initiation. The MAS group had significantly lower neutrophil counts, fibrinogen, and higher IL‐18/C‐reactive protein (CRP) ratio at starting tocilizumab (baseline) than the non‐MAS group. Before starting tocilizumab, neutrophil counts were trending downward and upward in the MAS and non‐MAS groups, respectively, with significant differences in changes. Receiver operating characteristic analysis showed that baseline neutrophil counts and fibrinogen and their changes before tocilizumab treatment and baseline IL‐18/CRP ratio had significant discriminatory abilities for subsequent MAS development. Conclusion We identified baseline laboratory features associated with MAS development after initiating an IL‐6 inhibitor in refractory systemic AOSD patients. These features may reflect the suppression of IL‐6 signaling, and further suppression of IL‐6 signaling might trigger early‐onset MAS.

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Successful Use of Higher-Dose Etanercept for Multirefractory Systemic Flare of Adult-Onset Still’s Disease with Liver Failure with No Response to Tocilizumab Therapy

Cited by 7 publications

References 12 publications

Adult-Onset Still’s Disease: Still a Serious Health Problem (a Case Report and Literature Review)

Adult-Onset Still’s Disease: Still a Serious Health Problem (a Case Report and Literature Review)

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

Baseline clinical features predicting macrophage activation syndrome in patients with systemic adult‐onset Still’s disease receiving interleukin‐6 inhibitor treatment

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