Macrophage Activation Syndrome as the Primary Presentation of Pediatric Systemic Lupus Erythematosus: A Case Report and Review of the Literature

Macrophage activation syndrome (MAS), in its secondary form, often complicates rheumatic diseases but rarely constitutes a mode of revelation. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unknown etiology that primarily affects women in adulthood. MAS is a serious condition that may be the first presentation of SLE. Here, we report the case of a 4-year-old female with MAS as the primary manifestation of Systemic Lupus Erythematosus (SLE). In this case, we outline the characteristics of a complex case of SLE that was initially accompanied with MAS, and also review the literature to discuss the clinical, biological, and therapeutic aspects of this condition.

Macrophage Activation Syndrome in a Context of Pre-B Type Lymphoblastic Acute Leucemia: A Case Report

Macrophage activation syndrome (MAS) is linked to inappropriate stimulation of macrophage cells in the bone marrow and lymphoid system, resulting in abnormal phagocytosis of figurative blood elements and the release of pro-inflammatory cytokines. It is a rare and serious hyper-inflammatory condition of diagnostic and therapeutic emergency. MAS is characterized by non-specific clinical and laboratory signs associated with images of hemophagocytosis. MAS is either “primary” (familial or pediatric forms), or “secondary/reactive” to infection, neoplasia, or autoimmune disease. Hemopathies dominate MAS secondary to neoplasia. B-type acute lymphoblastic leukemia (ALL) is a hematological malignancy characterized by the proliferation and accumulation of B lymphoid progenitors, blocked at an early stage of differentiation, leading to suppression of polyclonal hematopoiesis and subsequent development of signs associated with bone marrow failure. In this context, we report the observation of a macrophage activation syndrome (MAS) associated with ALL, diagnosed at Hôpital Principal de Dakar/Senegal, in a 69-year-old patient with a well-controlled type 2 diabetes under oral antidiabetic therapy (OAD) and good general condition.

Macrophage activation syndrome as a complication of dermatomyositis: A case report

BACKGROUND Macrophage activation syndrome(MAS)can be a fatal complication of rheumatic disorders,which occurs most commonly in patients with systemic juvenile idiopathic arthritis or systemic lupus erythematosus.It has rarely been reported in patients with dermatomyositis.Here,we describe a fatal case of MAS that developed in an adult patient with dermatomyositis.CASE SUMMARY A 44-year-old woman was admitted to our hospital with fever,generalized rash and muscle weakness.Fifteen days later,the fever persisted after the use of antibiotics,and repeat blood culture was negative.The patient then exhibited a typical Gottron sign and diffuse erythema on the face and neck,which were consistent with a diagnosis of dermatomyositis.The patient exhibited limb muscle strength of 2,and electromyography was suggestive of muscle-derived damage,which also supported a diagnosis of dermatomyositis.In addition,the patient exhibited high serum ferritin level,cytopenia,liver dysfunction,coagulopathy,enlarged spleen and hypertriglyceridemia,all of which are typical manifestations of MAS.The patient was diagnosed with dermatomyositis complicated by MAS.Although a high dose of methylprednisolone was administered for 15 d,the patient’s condition continued to deteriorate and central nervous system symptoms developed.Eventually,treatment was discontinued,and the patient died.CONCLUSION MAS is an important,potentially fatal,complication of dermatomyositis.Although MAS is rare in dermatomyositis,it should be considered in the differential diagnosis of an unexplained change of hemoglobin,platelet,fibrinogen,ferritin and triglyceride,which may complicate dermatomyositis.

Macrophage activation syndrome as an initial presentation of systemic lupus erythematosus

In a recent article on World J Clin Cases 2019;7:3859-3865,Sun et al reported a case of 36-year-old female with macrophage activity syndrome as an onset of systemic lupus erythematosus.Although this is a very interesting case,some concerns still need to be addressed.First,the patient had an extremely elevated serum ferritin but a normal C-reactive protein level,which was unparallel with the inflammatory condition before she received any treatments.Second,the diagnosis of systemic lupus erythematosus seemed to be insufficient according to the patient’s medical information presented,most of which were not specific to lupus but could be explained by macrophage activity syndrome.Hence,more medical information on the patient should be provided,and a profound discussion needs to be addressed.

Clinical and laboratory features,treatment,and outcomes of macrophage activation syndrome in 80 children:a multi-center study in China

Background Macrophage activation syndrome(MAS)is a major cause of morbidity and mortality in pediatric rheumatology.We aimed to further understand the clinical features,treatment,and outcome of MAS in China.Methods A multi-center cohort study was performed in seven hospitals in China from 2012 to 2018.Eighty patients with MAS were enrolled,including 53 cases with systemic juvenile idiopathic arthritis(SJIA-MAS),10 cases of Kawasaki disease(KD-MAS),and 17 cases of connective tissue disease(CTD-MAS).The clinical and laboratory data were collected before(pre-),at onset,and during full-blown stages of MAS.We compared the data among the SJIA-MAS,KD-MAS,and CTD-MAS subjects.Results 51.2%of patients developed MAS when the underlying disease was first diagnosed.In patients with SJIA,22.6%(12/53)were found to have hypotension before the onset of SJIA-MAS.These patients were also found to have significantly increased aspartate aminotransferase(AST)and lactate dehydrogenase(LDH),as well as decreased albumin(P<0.05),but no difference in alanine aminotransferase,ferdtin,and ratio of ferritin/erythrocyte sedimentation rate(ESR)at onset of MAS when compared to pre-MAS stages of the disease.In addition,ferritin and ratio of ferritin/ESR were significantly elevated in patients at full-blown stages of SJIA-MAS compared to pre-MAS stage.Significantly increased ferritin and ratio of ferritin/ESR were also observed in patients with SJIA compared to in KD and CTD.Receiver-operating characteristic analysis showed that 12,217.5μg/L of ferritin and 267.5 of ferritin/ESR ratio had sensitivity(80.0%and 90.5%)and specificity(88.2%and 86.7%),respectively,for predicting full-blown SJIA-MAS.The majority of the patients received corticosteroids(79/80),while biologic agents were used in 12.5%(10/80)of cases.Tocilizumab was the most commonly selected biologic agent.The overall mortality rate was 7.5%.Conclusions About half of MAS occurred when the underlying autoimmune diseases(SJIA,KD,and CTD)were first diagnosed.Hypotension could be an important manifestation before MAS diagnosis.Decreased albumin and increased AST,LDH,ferritin,and ratio of ferritin/ESR could predict the onset or full blown of MAS in patient with SJIA.

Macrophage activation syndrome in children with Kawasaki disease: diagnostic and therapeutic approaches

Background Macrophage activation syndrome(MAS)is a rare,life-threatening complication of Kawasaki disease(KD).Early recognition and treatment of MAS are very important,but sometimes it is difficult to distinguish MAS from a severe form of KD.Data sources A PubMed search was performed in Clinical Queries using the key terms“macrophage activation syndrome or secondary hemophagocytic lymphohistiocytosis(HLH)”and“Kawasaki disease”.Results KD patients with MAS show high intravenous immunoglobulin(IVIG)resistance and coronary complications.Mortality is also as high as MAS in other diseases.Persistent fever greater than 10 days is highly associated with development of MAS in KD.Splenomegaly is observed in more than two-thirds of KD patients with MAS.Thrombocytopenia is often the earliest laboratory finding of MAS.Hyperferritinemia is highly specific and sensitive for detecting MAS in KD;so,ferritin levels should be checked if there are unexplained clinical exacerbations in KD patients.Given the under-recognition of MAS in KD,it is prudent to consider resistant KD as occult/subclinical MAS.Many KD patients with MAS have good outcomes on immune modulators.However,if KD patients fulfill the HLH-2004 diagnostic criteria,they may undergo longer and more intensive treatment than needed.Conclusions The possible existence of MAS should be taken into account when a KD patient shows persistent fever,splenomegaly,thrombocytopenia,hyperferritinemia,or IVIG resistance.The under-diagnosis of MAS in patients with KD is an important issue to be addressed.Therapeutically,however,there is a possibility of over-treatment of MAS in patients with KD.

Clinical characteristics of macrophage activation syndrome secondary to systemic lupus erythematosus

Objective To investigate the clinical features of macrophage activation syndrome(MAS)associated with systemic lupus erythematosus(SLE).Methods The clinical data of 15 patients with SLE-induced MAS diagnosed in Peking Union Medical College Hospital from July

Adult-onset Still's disease evolving with multiple organ failure and death:A case report and review of the literature

BACKGROUND Adult-onset Still’s disease(AOSD)is a rare systemic inflammatory disease,which is characterized by daily fever and arthritis,with an evanescent rash and neutrophilic leukocytosis.To date,there has been no definite laboratory or imaging test available for diagnosing AOSD;the diagnosis is one of exclusion,which can be very challenging.In particular,AOSD patients may experience different complications affecting their clinical picture,management,and prognosis.The treatment of AOSD remains largely empirical and involves therapeutic agents.CASE SUMMARY We report the case of a 36-year-old woman who presented with fever,red rash,arthralgia,and sore throat.Her serum ferritin level and white blood cell count were markedly elevated,and the first diagnosis 22 years prior was"juvenile rheumatoid arthritis of systemic type".The patient was treated with prednisone,sulfasalazine,methotrexate,and leflunomide.After remission of her symptoms,the patient stopped taking the medications,and the disease recurred.Ultimately,the patient was diagnosed with adult-onset Still's disease.Relapse occurred several times due to self-medication withdrawal,and an interleukin-6 antagonist(tocilizumab/Actemra)was administered to control the disease.Recently,she was hospitalized because an incision did not heal,and the patient suddenly developed high fever and diarrhea during hospitalization.The patient's disease progressed violently and quickly developed into macrophage activation syndrome,disseminated intravascular coagulation,shock,and multiple organ failure.The patient had sudden cardiac arrest,and she died despite emergency rescue efforts.CONCLUSION AOSD patients need regular follow-up in the long-term treatment process,and must press formulary standard medication,and do not voluntarily withdraw or reduce the dose.Otherwise it may cause disease back-and-forth or serious lifethreatening complications.Meanwhile,strict management of trauma,infections,tumors,and other diseases may contribute to improved outcomes in patients with complications.

Single-cell RNA sequencing in juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is one of the most common chronic inflammatory rheumatic diseases in children,with onset before age 16 and lasting for more than 6 weeks.JIA is a highly heterogeneous condition with various consequences for health and quality of life.For some JIA patients,early detection and intervention remain challenging.As a result,further investigation of the complex and unknown mechanisms underlying JIA is required.Advances in technology now allow us to describe the biological heterogeneity and function of individual cell populations in JIA.Through this review,we hope to provide novel ideas and potential targets for the diagnosis and treatment of JIA by summarizing the current findings of single-cell RNA sequencing studies and understanding how the major cell subsets drive JIA pathogenesis.