Macrophage activation syndrome in systemic lupus erythematosus: a multicenter, case-control study in China

Abstract

The objective of this study was to describe the clinical and laboratory characteristics, precipitating factors, treatment, and outcome of macrophage activation syndrome (MAS) complicating systemic lupus erythematosus (SLE). A multicenter case-control study was performed across six tertiary hospitals from 1997 to 2014. A total of 32 patients with SLE-associated MAS were enrolled. Sixty-four age- and sex-matched SLE patients diagnosed in the same period without MAS episodes were selected as controls. The most frequent clinical feature was fever, followed by splenomegaly. Hyperferritinemia, hypoalbuminemia, and hyper-lactate dehydrogenase (LDH)-nemia were among the most common laboratory abnormalities. Compared with pre-MAS visit, patients at the onset of MAS had greater frequencies of renal involvement, liver dysfunction, and cytopenia. Receiver operating characteristic (ROC) analysis identified optimal cutoff values of ferritin (>662.5 ng/mL) and LDH (>359 U/mL) to predict the occurrence of MAS in SLE. SLE flare and infection were the common triggers of MAS in SLE. Abortion and parturition were recorded as well. The overall mortality rate was 12.5%. All patients received corticosteroids. Cyclosporine A, cyclophosphamide, and etoposide were the three most commonly used immunosuppressants. Rituximab was given to one patient. Intravenous immunoglobulin (IVIG) was added for 46.9% patients. MAS is a potentially fatal complication of SLE. Its occurrence is most frequently associated with active SLE disease or infection. The presentation of unexplained fever, cytopenia, or liver dysfunction, with high levels of ferritin and LDH, in patients with SLE should raise the suspicion of MAS. Corticosteroids with immunosuppressants and IVIG may be an appropriate treatment.

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

“…For this purpose, case-control studies, in which patients with SLE-associated MAS are compared with SLE patients with fever but no MAS, might be appropriate, but these data are still lacking in the literature. The therapeutic strategy for SLE-associated MAS has not yet been established (6,8). In our experience, the combination of corticosteroids with other immunosuppressive medications was beneficial.…”

“…There has been no clear conclusion on which immunosuppressant is preferable for MAS in SLE. Cyclosporin A was the most frequently used in our, as in other series (8), and is generally preferred to other drugs for the lack of bone marrow suppression. Moreover, our data suggest that in most SLE patients the full therapeutic regimen developed for primary HLH (HLH-2004, including etoposide, cyclosporine, and dexamethasone, and intrathecal therapy with methotrexate and corticosteroids in selected patients) may be not necessary.…”

“…Although occasional cases of MAS associated with infections have also been reported in SLE (4 out of 41 cases collected in a recent review of the literature) (5), in the large majority of cases, MAS is therefore linked with active lupus (4)(5)(6)(7)(8). Although in our experience, MAS and SLE were diagnosed simultaneously and this was also the most frequent scenario in some other previous series (4,8), recent data indicate that MAS may arise in patients with long disease duration in up to 50% of the cases (7). 3 and/or a platelet count of <110,000/mm 3 ; °the cut-off suggested for the classification of reactive MAS in the original study was 169 (11).…”

“…The first cases were described in 1991 as acute lupus hemophagocytic syndrome (2). 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).…”

Macrophage activation syndrome: early diagnosis is key

“…The mortality rate varied depending on the centers and the underlying diseases of the patients. Previous reports showed that the mortality rate in SJIA varied between 8% and 23%,10–13 whereas the mortality rate in SLE was ~5%–35% 14–17. The mortality rate was also higher in adults, which was around 50% 18…”