Successful use of short-term add-on tocilizumab for refractory adult-onset still’s disease with macrophage activation syndrome despite treatment with high-dose glucocorticoids, cyclosporine, and etoposide

Ohmura, Shin-Ichiro; Uehara, Koji; Yamabe, Toru; Tamechika, Shinya; Maeda, Shinji; Naniwa, Taio

doi:10.1080/24725625.2020.1741073

Cited by 17 publications

(5 citation statements)

References 30 publications

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“…IL‐1 and IL‐6 inhibitors have proven to be very helpful in the treatment of systemic JIA and MAS (82–84). Some voting panelists noted that monotherapy may not be sufficient for severely ill patients (83).…”

Section: Results/recommendationsmentioning

confidence: 99%

2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Oligoarthritis, Temporomandibular Joint Arthritis, and Systemic Juvenile Idiopathic Arthritis

Onel

Horton

Lovell

et al. 2022

Arthritis Care & Research

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Objective To provide updated guidelines for pharmacologic management of juvenile idiopathic arthritis (JIA), focusing on treatment of oligoarthritis, temporomandibular joint (TMJ) arthritis, and systemic JIA with and without macrophage activation syndrome. Recommendations regarding tapering and discontinuing treatment in inactive systemic JIA are also provided. Methods We developed clinically relevant Patient/Population, Intervention, Comparison, and Outcomes questions. After conducting a systematic literature review, the Grading of Recommendations Assessment, Development and Evaluation approach was used to rate the quality of evidence (high, moderate, low, or very low). A Voting Panel including clinicians and patients/caregivers achieved consensus on the direction (for or against) and strength (strong or conditional) of recommendations. Results Similar to those published in 2019, these JIA recommendations are based on clinical phenotypes of JIA, rather than a specific classification schema. This guideline provides recommendations for initial and subsequent treatment of JIA with oligoarthritis, TMJ arthritis, and systemic JIA as well as for tapering and discontinuing treatment in subjects with inactive systemic JIA. Other aspects of disease management, including factors that influence treatment choice and medication tapering, are discussed. Evidence for all recommendations was graded as low or very low in quality. For that reason, more than half of the recommendations are conditional. Conclusion This clinical practice guideline complements the 2019 American College of Rheumatology JIA and uveitis guidelines, which addressed polyarthritis, sacroiliitis, enthesitis, and uveitis. It serves as a tool to support clinicians, patients, and caregivers in decision‐making. The recommendations take into consideration the severity of both articular and nonarticular manifestations as well as patient quality of life. Although evidence is generally low quality and many recommendations are conditional, the inclusion of caregivers and patients in the decision‐making process strengthens the relevance and applicability of the guideline. It is important to remember that these are recommendations. Clinical decisions, as always, should be made by the treating clinician and patient/caregiver.

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Section: Results/recommendationsmentioning

confidence: 99%

2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Oligoarthritis, Temporomandibular Joint Arthritis, and Systemic Juvenile Idiopathic Arthritis

Onel

Horton

Lovell

et al. 2022

Arthritis Care & Research

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“…26 Tocilizumab, which blocks IL-6 and can inhibit the cytokine storm seen in HLH as well as T-cell expansion has been shown to be an effective adjunct for the management of HLH in adult onset Still's disease. 27 A tailored treatment approach may be the most efficacious; a recent case series of post-LT HLH in pediatric patients described a treatment algorithm which began with cessation of immunosuppression and treatment of underlying infections, with escalation to steroids and etoposide among patients with clinical and laboratory deterioration. The overall mortality rate was only 25%, which is lower than in previous reports.…”

Section: Discussionmentioning

confidence: 99%

Hemophagocytic lymphohistiocytosis occurring after liver transplantation: A case series and review of the literature

Chesner

Schiano

Fiel

et al. 2021

Clinical Transplantation

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Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease characterized by excessive inflammation and tissue destruction due to a dysregulated immune response. Its secondary form is most commonly triggered by viral infection or malignancy. There have previously been 11 cases of acquired HLH described following liver transplantation in adult transplant recipients, most occurring within the first year following transplantation. Herein, we describe two cases of HLH in liver transplant recipients that both occurred remotely following transplantation. In the first case, HLH was thought to be triggered by the development of a post-transplant lymphoproliferative disorder in a patient who was initially diagnosed with recurrent autoimmune hepatitis.In the second, it was thought to be triggered by a newly acquired human herpesvirus-8 infection. In both cases, the syndrome was not recognized until treatment for the initial putative diagnoses was unsuccessful. Despite treatment, both patients unfortunately died from multiorgan failure. HLH in the post-liver transplant setting is likely under-recognized and has a high mortality; early diagnosis and intervention may lead to improved outcomes. K E Y W O R D Scomplications of liver transplantation, hemophagocytic lymphohistiocytosis, human herpesvirus-8, liver transplantation, post-transplant lymphoproliferative disorder 1 INTRODUCTION Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by pathologic macrophage activation, which results in excessive cytokine release, inflammation, tissue destruction, and eventual multi-system organ failure. 1 Primary HLH is an inherited condition caused by autosomal recessive defects in genes triggering macrophage activation, while secondary HLH may be triggered by infection, malignancy, rheumatologic disorders, and immunodeficiencies. 2,3 Regardless of the causative agent, multiorgan failure often results from pervasive organ infiltration of activated monocytes, macrophages, and cytotoxic T-cells. Clinical features are

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“…Biologics such as tocilizumab, etanercept and anakinra have demonstrated good outcomes, especially in refractory EOSD or in case of complications [22,23,28,31,35,38,58,59]. These treatments need to be prescribed carefully in EOSD because of serious adverse events including opportunistic infections, anaphylactic reactions or biologic-associated macrophage activation syndrome [26,31].…”

Section: Discussionmentioning

confidence: 99%

Elderly-onset adult Still’s disease

et al. 2021

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Adult-onset Still's disease is a rare inflammatory disorder usually affecting young adults. Elderly-onset Still's disease (EOSD) is reported in some cases, commonly in Japan, the USA and Europe. One of the most commonly used criteria for diagnosing EOSD is Yamaguci criteria. In elderly patients more severe course of the disease and more complications may be expected than in the younger group of patients with Still's disease. The lungs involvement is rather rare manifestation of this disease. In our article we discuss the problem of both the development of Still's disease in the elderly and interstitial lung changes in the course of the disease, based on available literature and own cases from one centre.

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Successful use of short-term add-on tocilizumab for refractory adult-onset still’s disease with macrophage activation syndrome despite treatment with high-dose glucocorticoids, cyclosporine, and etoposide

Cited by 17 publications

References 30 publications

2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Oligoarthritis, Temporomandibular Joint Arthritis, and Systemic Juvenile Idiopathic Arthritis

2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Oligoarthritis, Temporomandibular Joint Arthritis, and Systemic Juvenile Idiopathic Arthritis

Hemophagocytic lymphohistiocytosis occurring after liver transplantation: A case series and review of the literature

Elderly-onset adult Still’s disease

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