Prognostic factors of macrophage activation syndrome, at the time of diagnosis, in adult patients affected by autoimmune disease: Analysis of 41 cases collected in 2 rheumatologic centers

Ruscitti, Piero; Cipriani, Paola; Ciccia, Francesco; Masedu, Francesco; Liakouli, Vasiliki; Carubbi, Francesco; Berardicurti, Onorina; Guggino, Giuliana; Benedetto, Paola Di; Bartolomeo, Salvatore Di; Valenti, Marco; Triolo, Giovanni; Giacomelli, Roberto

doi:10.1016/j.autrev.2016.09.016

Cited by 68 publications

(41 citation statements)

References 52 publications

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“…The patient had some negative prognostic factors for mortality in MAS according to 2 previous papers, [20,21] that is thrombocytopenia, elevated aspartate aminotransferase and increased serum ferritin levels. However, the main potentially fatal complication that the patient developed was fulminant myocarditis.…”

Section: Discussionmentioning

confidence: 89%

Refractory adult-onset Still disease complicated by macrophage activation syndrome and acute myocarditis

et al. 2017

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Rationale:Myocarditis is a rare but potentially fatal complication of Still's disease (about 7% of total cases).Patient concerns:A 42-year-old woman was admitted to our ward with high-grade fever, rash and polyarthralgia, lasting since 4 weeks and rapidly complicated by MAS and acute heart failure.Diagnoses:Adult Onset Still's Disease rapidly developping macrophage activation syndrome and disseminated intravascular coagulopathy, further complicated by iperacute myocarditis with cardiac arrest.Interventions:After failure of conventional therapies (steroids plus cyclosporine and then biological therapy with Anakinra 100 mg/day), the patient was treated with anakinra 100 mg sc 1 fl 4 times a day.Outcomes:Fast clinical and laboratoristic improvement and subsequent disease remission with complete recovery of cardiac function.Lessons:This is the first case report in which high doses of Anakinra have been used to treat a refractory AOSD complicated by MAS and myocarditis. In AOSD complicated by life-threatening conditions, probably we need to consider aggressive therapeutic approaches with higher doses of Il-1 receptor blocker to switch off the hyper-inflammation.

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Section: Discussionmentioning

confidence: 89%

Refractory adult-onset Still disease complicated by macrophage activation syndrome and acute myocarditis

et al. 2017

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“…Our patients showed peripheral cytopenias associated with high levels of both inflammatory markers and serum ferritin. All these patients were treated with a steroid pulse therapy [43, 45, 46]. Of note, it has been proposed that AOSD and MAS may be part of the same disease spectrum, in which AOSD should be considered the milder form [14], and the occurrence of MAS might be misdiagnosed due to the immunosuppressive treatments used to control an AOSD flare [14, 15].…”

Section: Discussionmentioning

confidence: 99%

Adult-onset Still’s disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers

Ruscitti

Cipriani

Masedu

et al. 2016

BMC Med

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110

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BackgroundAdult-onset Still’s disease (AOSD) is rare inflammatory disease of unknown etiology that usually affects young adults. The more common clinical manifestations are spiking fevers, arthritis, evanescent rash, elevated liver enzymes, lymphadenopathy, hepatosplenomegaly, and serositis. The multi-visceral involvement of the disease and the different complications, such as macrophage activation syndrome, may strongly decrease the life expectancy of AOSD patients.MethodsThis study aimed to identify the positive and negative features correlated with the outcome of patients. A retrospective analysis of AOSD patients prospectively admitted to three rheumatologic centers was performed to identify the clinical features present at the time of diagnosis and to predict the possible outcome. Furthermore, we investigated the as yet to be validated prognostic value of the systemic score previously proposed.ResultsOne hundred consecutive AOSD patients were enrolled. The mean systemic score showed that the majority of patients had a multi-organ involvement. Sixteen patients showed different complications, mainly the macrophage activation syndrome. A strong increase of inflammatory markers was observed. All patients received steroids at different dosages, 55 patients in association with immunosuppressive drugs and 32 in association with biologic agents. Sixteen patients died during the follow-up. Regression analysis showed that the higher values of the systemic score and the presence of AOSD-related complications, assessed at the time of diagnosis, were significantly correlated with patient mortality. A prognostic impact of the systemic score of ≥ 7.0 was reported.ConclusionsOur study showed that a higher systemic score and the presence of AOSD-related complications at the time of diagnosis were significantly associated with mortality. Of note, a cut-off at 7.0 of the systemic score showed a strong prognostic impact in identifying patients at risk of AOSD-related death.

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“…In our experience, no patient died because of MAS, but a mortality of 9.8% among 61 cases of SLE-associated MAS, identified through a review of the literature, was described (6), clearly indicating that this may be a potentially fatal condition. A very high mortality (42.5%) was reported in a series of 41 cases of MAS associated with autoimmune diseases in a study from 2 Italian rheumatology centers, but cases of SLE were not separately analyzed (9). Moreover, in this study, older age and higher levels of serum ferritin were associated with mortality, and it has been reported that SLE patients are younger, and with lower levels of ferritin as compared with other adult patients with autoimmune disease-associated MAS (6).…”

Section: Original Papermentioning

confidence: 99%

“…Afterwards, several small case series were described, with an estimated prevalence of MAS among SLE patients ranging from 0.9% to 4.6% (3)(4)(5)(6)(7)(8). To our knowledge, only one paper described adult SLE-associated MAS cases collected in Italy (9). However, in this study 9 SLE cases recruited from 2 rheumatology centers were presented together with 32 cases with other autoimmune diseases, and SLE cases were not separately analyzed (9).…”

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confidence: 99%

“…To our knowledge, only one paper described adult SLE-associated MAS cases collected in Italy (9). However, in this study 9 SLE cases recruited from 2 rheumatology centers were presented together with 32 cases with other autoimmune diseases, and SLE cases were not separately analyzed (9). Diagnostic criteria for MAS secondary to adult autoimmune diseases suffer from substantial limitations.…”

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confidence: 99%

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Macrophage activation syndrome in adult systemic lupus erythematosus: report of seven adult cases from a single Italian rheumatology center

et al. 2018

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SUMMARYThe aim was to describe the macrophage activation syndrome (MAS), a life-threatening syndrome characterized by excessive immune activation that can be triggered by conditions affecting immune homeostasis, in a cohort of adult Italian patients with systemic lupus erythematosus (SLE). This was a monocentric retrospective evaluation. The utility of the H-score, developed to estimate the individual risk of having reactive MAS in adult patients, was assessed. Among 511 patients with SLE, 7 cases (1.4%) of MAS (all females) were identified and their medical records reviewed. In all cases, MAS was simultaneous to the onset of SLE. All patients had fever, lymphadenopathy, hematological involvement, and high titer of anti-dsDNA antibodies. Workup for infections and malignancies was negative. In all cases, the H-score was higher than the cut-off suggested for the classification of reactive MAS. All cases required hospital admission, and 2 patients were admitted to the intensive care unit. Most patients were treated successfully with high doses of corticosteroids and with immunosuppressive drugs, whereas the full therapeutic regimen developed for primary hemophagocytic lymphohistiocytosis HLH was used only in one case. No death from MAS was observed. MAS is a rare and severe disorder that complicated the onset of SLE in our cohort. The H-score may be useful in the classification of these patients.

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Prognostic factors of macrophage activation syndrome, at the time of diagnosis, in adult patients affected by autoimmune disease: Analysis of 41 cases collected in 2 rheumatologic centers

Cited by 68 publications

References 52 publications

Refractory adult-onset Still disease complicated by macrophage activation syndrome and acute myocarditis

Refractory adult-onset Still disease complicated by macrophage activation syndrome and acute myocarditis

Adult-onset Still’s disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers

Macrophage activation syndrome in adult systemic lupus erythematosus: report of seven adult cases from a single Italian rheumatology center

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