Predictors of the response to treatment in acute lupus hemophagocytic syndrome

Takahashi, Hidenori; Tsuboi, Hiroto; Kurata, Izumi; Inoue, Seiichiro; Ebe, Hiroshi; Yokosawa, Masahiro; Hagiwara, Shinji; Hirota, Tomoya; Asashima, Hiromitsu; Kaneko, Shunta; Kawaguchi, Haruna; Kurashima, Yuko; Miki, Haruka; Umeda, N.; Kondo, Yuya; Ogishima, Hiroshi; Suzuki, Takeshi; Matsumoto, Isao; Sumida, Takayuki

doi:10.1177/0961203314559086

Cited by 14 publications

(26 citation statements)

References 53 publications

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“…Haematological abnormalities, if relapsing or refractory to steroids, have been shown to respond well to CyA. This is the case of lupus-associated thrombocytopenia [93,94], haemolytic anaemia [95], aplastic anaemia [96], red cell aplasia [97][98][99] and haemophagocytic syndrome [100].…”

Section: Cyclosporine a In Systemic Lupus Erythematosusmentioning

confidence: 99%

The Use of Cyclosporine A in Rheumatology: a 2016 Comprehensive Review

Chighizola

Ong

Meroni

2016

Clinic Rev Allerg Immunol

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Cyclosporine A, an inhibitor of calcineurin, exerts an immunomodulator action interfering with T cell activation. Even though novel therapeutic tools have emerged, CyA still represents a suitable option in several clinical rheumatology settings. This is the case of refractory nephritis and cytopenias associated with systemic lupus erythematosus. Furthermore, CyA is a valued therapeutic tool in the management of uveitis and thrombophlebitis in course of Behçet's disease. Topical CyA has been proven to be beneficial in the dry eye of Sjogren's syndrome, whereas oral treatment with CyA can be considered for the severe complications of adult onset Still's disease. CyA provides a therapeutic option in psoriatic arthritis, being rather effective in skin disease. CyA is currently regarded as a second-line option for patients with inflammatory myopathies refractory to standard regimen. CyA is used even in paediatric rheumatology, in particular in the management of juvenile dermatomyositis and macrophage activation syndrome associated with systemic juvenile idiopathic arthritis. Importantly, CyA has been shown to suppress the replication of HCV, and it can thus be safely prescribed to those patients with chronic hepatitis C. Noteworthy, CyA can be administered throughout the gestation course. Surely, caution should be paid to CyA safety profile, in particular to its nephrotoxicity. Even though most evidence comes from small and uncontrolled studies with few randomised controlled trials, CyA should be still regarded as a valid therapeutic tool in 2016 rheumatology.

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Section: Cyclosporine a In Systemic Lupus Erythematosusmentioning

confidence: 99%

The Use of Cyclosporine A in Rheumatology: a 2016 Comprehensive Review

Chighizola

Ong

Meroni

2016

Clinic Rev Allerg Immunol

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“…However, we could not deny the possibility that the response of ALHS to first IVCY was delayed, second and third IVCY might have contributed to maintaining control of the patient's ALHS, while concomitant IVIG might have had synergistic effects on the activated macrophages. Indeed, our previous study revealed a significant and negative correlation between the serum ferritin and anti-dsDNA antibody levels among reviewed ALHS cases ( 12 ). Moreover, among 32 ALHS patients treated with CsA, the serum ferritin level was significantly higher and the leukocyte count was significantly lower in CsA responders than in non-responders ( 12 ).…”

Section: Discussionmentioning

confidence: 88%

Intractable Hemophagocytic Syndrome Associated with Systemic Lupus Erythematosus Resistant to Corticosteroids and Intravenous Cyclophosphamide That Was Successfully Treated with Cyclosporine A

et al. 2018

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Hemophagocytic syndrome (HPS) associated with systemic lupus erythematosus (SLE), dubbed acute lupus hemophagocytic syndrome (ALHS), is an intractable complication of SLE. A 24-year-old man who had been diagnosed with SLE three months previously, presented with fever, rash, hallucination, and pancytopenia accompanied with hyperferritinemia and bone marrow hemophagocytosis. He was diagnosed with ALHS and neuropsychiatric (NP)-SLE. Although 4 courses of methylprednisolone pulse therapy and 1 course of intravenous cyclophosphamide (IVCY) improved his NP-SLE, his ALHS did not respond. However, the addition of cyclosporine A (CsA) led to a rapid remission from ALHS. This suggests the usefulness of CsA in the treatment of intractable, corticosteroid- and IVCY-resistant ALHS.

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“…HLH is a valid example of the plenty of still-open challenges we are facing in the field of autoimmune and autoinflammatory diseases. The Takahashi et al 10 study results allude to possible different sub-phenotypes of the same disease, which differently respond to a different therapeutic schedule. The identification of predictive markers for the treatment response and disease outcome helped in formulating this hypothesis, which still requires further confirmation.…”

mentioning

confidence: 99%

“…Similarly, between 2005 and 2013 Takahashi et al 10 reported seven patients diagnosed with ALHS, among whom five experienced ALHS as the first manifestation of SLE. Interestingly, cases with low anti-double-stranded DNA (anti-dsDNA) antibody titres tended to have more severe hyperferritinemia, and a negative and significant correlation between anti-dsDNA antibodies and levels of serum ferritin was finally found.…”

mentioning

confidence: 99%