Rapid and Sustained Remission of Systemic Juvenile Idiopathic Arthritis-Associated Macrophage Activation Syndrome Through Treatment With Anakinra and Corticosteroids

Brück, Normi; Suttorp, Meinolf; Kabus, Maria; Heubner, Georg; Gahr, Manfred; Peßler, Frank

doi:10.1097/rhu.0b013e318205092d

Cited by 131 publications

(77 citation statements)

References 9 publications

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“…An alternative therapy to etoposide is antithymocyte globulin; however, this treatment is also less commonly used in those with MAS (34). More recently, the IL-1 blocking agent, anakinra, has been successfully used to treat MAS (35); however, it should be noted that some patients with s-JIA have developed MAS while being managed with anakinra (36). Immunoglobulin (i.v.)…”

Section: Cytokinesmentioning

confidence: 99%

Advances in the pathogenesis and treatment of systemic juvenile idiopathic arthritis

Correll

Binstadt

2013

Pediatr Res

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Section: Cytokinesmentioning

confidence: 99%

Advances in the pathogenesis and treatment of systemic juvenile idiopathic arthritis

Correll

Binstadt

2013

Pediatr Res

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“…It has been reported that infliximab, daclizumab, anakinra, vincristine, tocilizumab, and other drugs have been used in HLH salvage therapy. [9][10][11][12][13] One single-center clinical study with 38 subjects showed that initial treatment combined with antithymocyte globulins increased the complete response rate to 73%; however, the early recurrence rate was also high, and there was no superiority in long-term prognosis.…”

Section: Discussionmentioning

confidence: 99%

Multicenter study of combination DEP regimen as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis

Wang

Huang

et al. 2015

Blood

141

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Key Points• A salvage therapy for adults with refractory hemophagocytic lymphohistiocytosis.• Liposomal doxorubicin treatment combined with etoposide and methylprednisolone showed an encouraging overall response and was welltolerated.Hemophagocytic lymphohistiocytosis (HLH) is a refractory immune disorder with a significant risk of death. Although standard therapy has dramatically improved survival in HLH patients, approximately 30%, especially adults, show no response to current treatment strategies. This prospective study aimed to investigate the efficacy of liposomal doxorubicin treatment combined with etoposide and methylprednisolone (doxorubicin-etoposidemethylprednisolone; DEP) as a salvage therapy for adult refractory HLH. Adult patients who did not achieve at least partial response 2 weeks after initial standard HLH therapy were enrolled in this study between June 2013 and June 2014. Response to salvage therapy was assessed at 2 and 4 weeks after initiation of DEP therapy and patients were followed until death or until November 2014. Sixty-three refractory HLH patients were enrolled, including 29 cases of lymphoma-associated HLH, 22 cases of Epstein-Barr virus-associated HLH, and 4 cases of familial HLH. There were 8 cases with unknown underlying diseases. Seventeen cases (27.0%) achieved complete response and 31 cases (49.2%) achieved partial response. The overall response was 76.2% (48/63). Patients who showed no response to DEP died within 4 weeks after salvage therapy. Twenty-nine of the 48 patients who achieved partial or complete response survived to subsequent chemotherapy, allogenic hematopoietic stem cell transplantation, or splenectomy. Our study suggests that DEP regimen is an effective salvage regimen for adult refractory HLH, which can prolong patient survival as we continue to understand the responsible mechanisms and bridge the gap between HLH and its underlying diseases. This study was registered in the Chinese Clinical Trials Registry Platform

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“…Also anakinra, an interleukine-1 receptor antagonist, has been shown to control the inflammatory response. There are case series in which it is successfully used in the treatment of hyperinflammatory syndromes without worsening of cytopenias [12,13].…”

Section: Discussionmentioning

confidence: 99%

Hemophagocytic lymphohistiocytosis in a patient with influenza and invasive pulmonary aspergillosis – A case report

Mgj¹,

Jg²

2017

Clin Case Rep Rev

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Invasive pulmonary aspergillosis can occur secondary to influenza pneumonia and has a high mortality. We present a case of a previously healthy 38-year-old woman suffering from influenza pneumonia complicated by IAA. She also met the criteria for hemophagocytic lymphohistcytosis (HLH), without signs of immunocompromised state. Despite extensive treatment, the patient did not survive. We review the existing literature of HLH in influenza cases.

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Rapid and Sustained Remission of Systemic Juvenile Idiopathic Arthritis-Associated Macrophage Activation Syndrome Through Treatment With Anakinra and Corticosteroids

Cited by 131 publications

References 9 publications

Advances in the pathogenesis and treatment of systemic juvenile idiopathic arthritis

Advances in the pathogenesis and treatment of systemic juvenile idiopathic arthritis

Multicenter study of combination DEP regimen as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis

Hemophagocytic lymphohistiocytosis in a patient with influenza and invasive pulmonary aspergillosis – A case report

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