Fulminant myocarditis and macrophage activation syndrome secondary to adult-onset Still’s disease successfully treated with tocilizumab

Savage, Eimear; Wazir, Tasheen; Drake, Mary; Cuthbert, R. J. G.; Wright, Gary

doi:10.1093/rheumatology/keu019

Cited by 42 publications

(21 citation statements)

References 7 publications

(6 reference statements)

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“…Patient number 6 had fulminant myocarditis, but he continued to do well on TCZ as monotherapy after recovery from a cardiac arrest. Hence, TCZ was believed to be effective [ 26 ].…”

Section: Discussionmentioning

confidence: 99%

Successful Tocilizumab Therapy for Macrophage Activation Syndrome Associated with Adult-Onset Still’s Disease: A Case-Based Review

Watanabe

Sugawara

Yamashita

et al. 2016

Case Reports in Medicine

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We report the case of a 71-year-old Japanese woman with adult-onset Still's disease (AOSD) in whom macrophage activation syndrome (MAS) developed despite therapy with oral high-dose prednisolone and intravenous methylprednisolone pulse therapy twice. She was successfully treated with tocilizumab (TCZ). Soon afterward, her fever ceased and high levels of both ferritin and C-reactive protein levels decreased. Her course was complicated by disseminated intravascular coagulation, cytomegalovirus infection, and Pneumocystis jirovecii pneumonia. After these were resolved, AOSD-associated MAS was well controlled. She was discharged on hospital day 87. Although biologics such as TCZ are becoming established for the treatment of AOSD, there is no recommended therapy for AOSD-associated MAS. Several biologics have been tried for this complication, but their efficacy and safety remain controversial. We reviewed reported cases of AOSD-associated MAS successfully treated with various biologics. TCZ initiation after adequate nonselective immunosuppressive therapy, such as methylprednisolone pulse therapy or a prednisolone-based combination of immunosuppressants, can be an effective treatment for AOSD-associated MAS. On the other hand, biologics given after insufficient immunosuppressive therapy may cause MAS. A strategy combining adequate immunosuppression and a biologic could be safe if special attention is given to adverse events such as opportunistic infections or biologic-associated MAS.

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“…Patient number 6 had fulminant myocarditis, but he continued to do well on TCZ as monotherapy after recovery from a cardiac arrest. Hence, TCZ was believed to be effective [ 26 ].…”

Section: Discussionmentioning

confidence: 99%

Successful Tocilizumab Therapy for Macrophage Activation Syndrome Associated with Adult-Onset Still’s Disease: A Case-Based Review

Watanabe

Sugawara

Yamashita

et al. 2016

Case Reports in Medicine

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“…Investigational drugs including NI-0501/anti-IFNγ monoclonal antibody and recombinant human IL-18 binding protein were discussed but ultimately were not included in the EBG because they were not FDA approved at the time of the workgroup meetings and thus were difficult to obtain outside of a clinical trial [31, 44]. Literature on the use of tocilizumab for HLH/MAS was reviewed by the EBG workgroup, including the impact of IL-6 on NK cell function [24, 25], rates of MAS in sJIA patients treated with tocilizumab [9, 50, 51], use of tocilizumab to treat MAS in sJIA and Adult-onset Still’s disease (AOSD) [52, 53], and efficacy of tocilizumab in cytokine release syndrome secondary to T cell engaging oncologic therapies [54, 55]. The reviewed phase III clinical studies demonstrated that MAS can occur in patients with sJIA while on tocilizumab, even with adequate sJIA disease control [9].…”

Section: Resultsmentioning

confidence: 99%

Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome

et al. 2019

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Background Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) were historically thought to be distinct entities, often managed in isolation. In fact, these conditions are closely related. A collaborative approach, which incorporates expertise from subspecialties that previously treated HLH/MAS independently, is needed. We leveraged quality improvement (QI) techniques in the form of an Evidence-Based Guideline (EBG) to build consensus across disciplines on the diagnosis and treatment of HLH/MAS. Methods A multidisciplinary work group was convened that met monthly to develop the HLH/MAS EBG. Literature review and expert opinion were used to develop a management strategy for HLH/MAS. The EBG was implemented, and quality metrics were selected to monitor outcomes. Results An HLH/MAS clinical team was formed with representatives from subspecialties involved in the care of patients with HLH/MAS. Broad entry criteria for the HLH/MAS EBG were established and included fever and ferritin ≥500 ng/mL. The rheumatology team was identified as the “gate-keeper,” charged with overseeing the diagnostic evaluation recommended in the EBG. First-line medications were recommended based on the acuity of illness and risk of concurrent infection. Quality metrics to be tracked prospectively based on time to initiation of treatment and clinical response were selected. Conclusion HLH/MAS are increasingly considered to be a spectrum of related conditions, and joint management across subspecialties could improve patient outcomes. Our experience in creating a multidisciplinary approach to HLH/MAS management can serve as a model for care at other institutions. Electronic supplementary material The online version of this article (10.1186/s12969-019-0309-6) contains supplementary material, which is available to authorized users.

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“…Studies regarding effective therapies are limited to case series and case reports. High dose corticosteroids with or without cyclosporine A was reported as first‐line therapy, followed by combinations with anakinra or tocilizumab as other options to improve clinical outcomes . IL‐1B is a proinflammatory cytokine, generated through cleavage of pro‐IL1B via the inflammasome, and plays a clear role in AOSD/MAS .…”

Section: Discussionmentioning

confidence: 99%

Refractory macrophage activation syndrome in the setting of adult‐onset Still disease with hemophagocytic lymphohistiocytosis detected on skin biopsy treated with canakinumab and tacrolimus

et al. 2019

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A 19-year-old Caucasian female with adult-onset Still disease (AOSD) presented for evaluation of an acute clinical decompensation and atypical annular papules and plaques with purpura on the lower extremities. A punch biopsy demonstrated histiocytes with engulfed degenerated erythrocytes and lymphocytes, consistent with hemophagocytic lymphohistiocytosis (HLH). HLH, clinically referred to as macrophage activation syndrome, is a rare complication of AOSD and is life-threatening.Relevant clinical, laboratory, and histologic features of this diagnosis are reviewed. K E Y W O R D Sadult-onset Still disease, canakinumab, hemophagocytic lymphohistiocytosis, histopathology, macrophage activation syndrome, skin biopsy, tacrolimus

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Fulminant myocarditis and macrophage activation syndrome secondary to adult-onset Still’s disease successfully treated with tocilizumab

Abstract: IL-6 blockade in a case resistant to TNF neutralization should prompt a second diagnostic workup. Rheumatology key message. Neutralization of IL-6 by tocilizumab induces complete clinical remission and controls the growth of popliteal angiomatoid malignant fibrous histiocytoma.

Cited by 42 publications

References 7 publications

Successful Tocilizumab Therapy for Macrophage Activation Syndrome Associated with Adult-Onset Still’s Disease: A Case-Based Review

Successful Tocilizumab Therapy for Macrophage Activation Syndrome Associated with Adult-Onset Still’s Disease: A Case-Based Review

Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome

Refractory macrophage activation syndrome in the setting of adult‐onset Still disease with hemophagocytic lymphohistiocytosis detected on skin biopsy treated with canakinumab and tacrolimus

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