scholarly journals Macrophage Activation Syndrome Presented in a Case of Neonatal Lupus

2021 ◽  
Vol 28 (3) ◽  
pp. 139-142
Author(s):  
Chang Min Kang ◽  
Jinwha Choi ◽  
JungHwa Lee
Keyword(s):  
Autoimmune Disease ◽  
Autoimmune Diseases ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Early Recognition ◽  
Transplacental Passage ◽  
Neonatal Lupus ◽  
Life Threatening ◽  
Life Threatening Complication ◽  
Activation Syndrome

Macrophage activation syndrome (MAS) is a potentially life-threatening complication in many autoimmune diseases. Early recognition and intervention are essential for a favorable outcome. Neonatal lupus, an acquired autoimmune disease in neonates caused by the transplacental passage of maternal autoantibodies, is rare and usually self-limited. Herein, we report a case of MAS in a patient with neonatal lupus, which improved with intravenous immunoglobulin.

scholarly journals Macrophage Activation Syndrome in Adults: A Retrospective Case Series

2021 ◽  
Vol 9 ◽  
pp. 232470962110264
Author(s):  
Taylor Warmoth ◽  
Malvika Ramesh ◽  
Kenneth Iwuji ◽  
John S. Pixley
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Pediatric Patients ◽  
Inflammatory Diseases ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Case Series ◽  
Adult Patients ◽  
Retrospective Case ◽  
Life Threatening ◽  
Activation Syndrome

Macrophage activation syndrome (MAS) is a form of hemophagocytic lymphohistocytosis that occurs in patients with a variety of inflammatory rheumatologic conditions. Traditionally, it is noted in pediatric patients with systemic juvenile idiopathic arthritis and systemic lupus erythematous. It is a rapidly progressive and life-threatening syndrome of excess immune activation with an estimated mortality rate of 40% in children. It has become clear recently that MAS occurs in adult patients with underlying rheumatic inflammatory diseases. In this article, we describe 6 adult patients with likely underlying MAS. This case series will outline factors related to diagnosis, pathophysiology, and review present therapeutic strategies.

scholarly journals Etoposide as an Effective Drug for Adult Macrophage Activation Syndrome: A Retrospective Study

2021 ◽  
Author(s):  
Lingbo He ◽  
Zhili Jin ◽  
Menghan Liu ◽  
Tingting Cui ◽  
Lin Wu ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
Initial Treatment ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Hemophagocytic Syndrome ◽  
Response Rate ◽  
Initial Therapy ◽  
Group 2 ◽  
Activation Syndrome ◽  
Group 1

Abstract BackgroundAutoimmune disease related hemophagocytic syndrome, in other words, macrophage activation syndrome(MAS), is a rare, but lethal complication of autoimmune disease. At present, specific treatment guidelines for adult MAS have not been formulated, most experience are derived from children, researches about etoposide are scarce. As the importance of etoposide in the initial treatment had been proved in other subtypes of hemophagocytic syndrome, the objective of this study is to investigate the effectiveness of etoposide in the treatment of the adult macrophage activation syndrome.Result74 patients with autoimmune disease related hemophagocytic syndrome were involved in this study, they were divided into two groups based on initial treatment, group 1(n=53): initial therapy did not contain etoposide, group 2(n=21): initial therapy contained etoposide. The overall response rate and complete response rate of group 2 were significantly higher than group 1(ORR 90.5% vs 24.5%, CRR 33.3% vs 3.8%, P<0.05). Patients with different HLH remission states have significantly different prognosis(P<0.001).ConclusionAdopting VP-16 in initial treatment can significantly increase the OR rate and CR rate of adult MAS patients, and the HLH states influenced the prognosis significantly.

scholarly journals 10. Drug-induced lupus as a trigger for macrophage activation syndrome

2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Mark Leith ◽  
Eimear Savage
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Inflammatory Arthritis ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
High Dose ◽  
Systemic Lupus ◽  
Drug Induced ◽  
Life Threatening ◽  
Activation Syndrome

Abstract Introduction Macrophage activation syndrome (MAS) or haemophagocytic lympohistiocytosis (HLH) is a rare, life threatening cause of fever. It can be due to a primary haematological condition, but can also be triggered by several rheumatological conditions such as Stills disease or systemic lupus erythematosus. It can often be misdiagnosed as infection, leading to a delayed or even missed diagnosis. Given its life threatening course, we need not only recognise the syndrome, but also identify the underlying trigger so that appropriate treatment of the underlying cause can be initiated early. This case is the first reported case of drug-induced lupus causing MAS. Case description This is a 56-year-old female of Indian origin who initially presented to rheumatology in January 2018 with a seronegative inflammatory arthritis. ANA was negative at this time and she had no other clinical features of a connective tissue disease. She was intolerant of methotrexate, so switched to sulphasalazine in October 2018. Unfortunately, sulphasalazine failed to control her disease, and she was assessed for biologic therapy in March 2019. It was noted she had travelled to India at the start of 2019, but IGRA screening in March returned negative prior to being considered for biologics. She was admitted to Daisy Hill Hospital in Newry, Northern Ireland on 22/3/19 with pyrexia, right sided abdominal pain and leucopenia. She was treated with several courses of broad spectrum antibiotics, but multiple blood and urine cultures came back negative. CT chest, abdomen and pelvis found duodenitis, but failed to identify a source of sepsis or evidence of tuberculosis. Echocardiogram was normal. Investigations from infectious diseases ruled out HIV, Hepatitis B&C, EBV, CMV, stongyloides, leishmaniasis, syphilis and malaria. Daily pyrexia persisted, and she developed a progressive pancytopenia, rash, mucositis and a rising ferritin up to 30000. Skin biopsy was non-specific but showed weak staining for IgM and C3 raising the possibility of vasculitis but was not definitive. Triglycerides were elevated at 3.6 and fibrinogen 1.2. ANA, which had initially been normal before sulphasalazine, was now positive at 1in40 with an anti-chromatin of 3.5 and ds-DNA 18. Complement was normal. CD25 soluble receptor later returned at 5370. Anti histone antibody was negative. Bone marrow biopsy confirmed MAS. She was treated with intravenous immunoglobulins, intravenous methylprednisolone for 3 days followed by prednisolone, and anakinra. Her fevers subsequently settled, ferritin normalised and her blood counts gradually improved. She was commenced on hydroxychloroquine and prednisolone dose weaned. Discussion Our working diagnosis in this case was that of a drug-induced lupus secondary to sulphasalazine therapy which then was complicated by MAS. This is the first reported case in the literature of a drug-induced lupus-driven MAS. We had considered if this could have represented a systemic lupus erythematosus picture from the onset of the inflammatory arthritis, however, the initial ANA was normal and only became positive after treatment with sulphasalazine. Interestingly, this patient’s ANA profile became negative following treatment with steroid/anakinra and following withdrawal of the drug. It is unusual that complement would be normal if this was a presentation of systemic lupus, and whilst anti histone antibody negativity perhaps points away from drug induced lupus, it can be negative in 5% cases of drug induced lupus. Key learning points Early recognition of MAS is imperative if we are to improve morbidity and mortality from this condition. It is important to be aware of potential triggers of the syndrome, and this case has highlighted a previously unrecorded cause of MAS in drug induced lupus. In this case, treatment with high dose steroid, intravenous immunoglobulin and anakinra, as well as withdrawing the causative drug, proved to be very effective in resolving her MAS. Conflict of interest The authors declare no conflicts of interest.

scholarly journals Case Report: VEXAS Syndrome: From Mild Symptoms to Life-Threatening Macrophage Activation Syndrome

2021 ◽  
Vol 12 ◽  
Author(s):  
Frederik Staels ◽  
Albrecht Betrains ◽  
Sherida Woei-A-Jin ◽  
Nancy Boeckx ◽  
Marielle Beckers ◽  
...  
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Somatic Mutations ◽  
Castleman Disease ◽  
Mild Phenotype ◽  
Multicentric Castleman Disease ◽  
Transfusion Requirements ◽  
Life Threatening ◽  
Systemic Symptoms ◽  
Activation Syndrome

Recently, a novel disorder coined VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome was identified in patients with adult-onset inflammatory syndromes, often accompanied by myelodysplastic syndrome1. All patients had myeloid lineage-restricted somatic mutations in UBA1 affecting the Met41 residue of the protein and resulting in decreased cellular ubiquitylation activity and hyperinflammation. We here describe the clinical disease course of two VEXAS syndrome patients with somatic UBA1 mutations of which one with a mild phenotype characterized by recurrent rash and symmetric polyarthritis, and another who was initially diagnosed with idiopathic multicentric Castleman disease and developed macrophage activation syndrome as a complication of the VEXAS syndrome. The latter patients was treated with anti-IL6 therapy (siltuximab) leading to a resolution of systemic symptoms and reduction of transfusion requirements.

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