Successful treatment with plasma exchange in adult-onset Still’s disease with hyper-IL-18-naemia and hyperallergic state

Ito, Tomoki; Ozaki, Yoshio; Shimamoto, Keiko; Amuro, Hideki; Tanijiri, Tsutomu; Yokoi, Takashi; Son, Yonsu; Tajima, Kenichirou; Fukuhara, Shirou

doi:10.1007/s10165-008-0065-9

Cited by 9 publications

(7 citation statements)

References 8 publications

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“…It has a marked corticosteroid-sparing effect and a good tolerance profile [ 48 , 49 ]. Plasma exchange and intravenous immunoglobulins are other treatment options in refractory AOSD patients [ 50 , 51 ].…”

Section: Discussionmentioning

confidence: 99%

Adult Onset Still’s Disease: A Review on Diagnostic Workup and Treatment Options

Gopalarathinam

Orlowsky

Kesavalu³

et al. 2016

Case Reports in Rheumatology

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Adult onset Still's disease (AOSD) is a rare systemic inflammatory disease of unknown etiology and pathogenesis that presents in 5 to 10% of patients as fever of unknown origin (FUO) accompanied by systemic manifestations. We report an interesting case of a 33-year-old African-American male who presented with one-month duration of FUO along with skin rash, sore throat, and arthralgia. After extensive workup, potential differential diagnoses were ruled out and the patient was diagnosed with AOSD based on the Yamaguchi criteria. The case history, incidence, pathogenesis, clinical manifestations, differential diagnoses, diagnostic workup, treatment modalities, and prognosis of AOSD are discussed in this case report.

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Section: Discussionmentioning

confidence: 99%

Adult Onset Still’s Disease: A Review on Diagnostic Workup and Treatment Options

Gopalarathinam

Orlowsky

Kesavalu³

et al. 2016

Case Reports in Rheumatology

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“…Plasma exchange is an extracorporeal blood purification technique designed to remove various toxic and inflammatory mediators and to replenish essential compounds via the replacement plasma, which is known also to decrease ferritin levels [16]. It is a successful therapy in all four clinical conditions discussed, although in the case of the AOSD, there are only anecdotal cases [59,73-75,77,78,81,85-88]. …”

Section: Clinical and Laboratory Features In Mas Aosd Caps And Septmentioning

confidence: 99%

The Hyperferritinemic Syndrome: macrophage activation syndrome, Still’s disease, septic shock and catastrophic antiphospholipid syndrome

Rosário

Zandman‐Goddard

Meyron-Holtz

et al. 2013

BMC Med

416

361

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BackgroundOver the last few years, accumulating data have implicated a role for ferritin as a signaling molecule and direct mediator of the immune system. Hyperferritinemia is associated with a multitude of clinical conditions and with worse prognosis in critically ill patients.DiscussionThere are four uncommon medical conditions characterized by high levels of ferritin, namely the macrophage activation syndrome (MAS), adult onset Still’s disease (AOSD), catastrophic antiphospholipid syndrome (cAPS) and septic shock, that share a similar clinical and laboratory features, and also respond to similar treatments, suggesting a common pathogenic mechanism. Ferritin is known to be a pro-inflammatory mediator inducing expression of pro-inflammatory molecules, yet it has opposing actions as a pro-inflammatory and as an immunosuppressant. We propose that the exceptionally high ferritin levels observed in these uncommon clinical conditions are not just the product of the inflammation but rather may contribute to the development of a cytokine storm.SummaryHere we review and compare four clinical conditions and the role of ferritin as an immunomodulator. We would like to propose including these four conditions under a common syndrome entity termed “Hyperferritinemic Syndrome”.

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“…Plasma exchange has been reported to be effective for AOSD-associated MAS (35)(36)(37). Ito et al demonstrated the prompt improvement of hypercytokinemia due to IL-6 and IL-18 using plasma exchange and a high-dose corticosteroid (37).…”

Section: Discussionmentioning

confidence: 99%

“…Ito et al demonstrated the prompt improvement of hypercytokinemia due to IL-6 and IL-18 using plasma exchange and a high-dose corticosteroid (37). The HLH 2004 protocol recommends the use of CyA concomitantly from an early stage in the cases of AOSD-associated MAS (38).…”

Section: Discussionmentioning

confidence: 99%

Two Cases of Adult-onset Still's Disease Treated with Tocilizumab that Achieved Tocilizumab-free Remission

et al. 2015

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There have been many previously reported cases of adult-onset Still's disease (AOSD) which were successfully treated with tocilizumab (TCZ). However, the efficacy and safety of TCZ therapy for AOSDassociated macrophage activation syndrome (MAS), and the optimal duration of TCZ therapy, remain unclear. We herein report two cases of refractory AOSD, one of which was associated with MAS. These two patients were treated with TCZ, and the withdrawal of TCZ was planned according to the serum interleukin-6 level, which resulted in TCZ-free remission.

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Successful treatment with plasma exchange in adult-onset Still’s disease with hyper-IL-18-naemia and hyperallergic state

Cited by 9 publications

References 8 publications

Adult Onset Still’s Disease: A Review on Diagnostic Workup and Treatment Options

Adult Onset Still’s Disease: A Review on Diagnostic Workup and Treatment Options

The Hyperferritinemic Syndrome: macrophage activation syndrome, Still’s disease, septic shock and catastrophic antiphospholipid syndrome

Two Cases of Adult-onset Still's Disease Treated with Tocilizumab that Achieved Tocilizumab-free Remission

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