scholarly journals Macrophage activation syndrome in MDA5 antibody-positive dermatomyositis and COVID-19 infection

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Marzieh Keshtkarjahromi ◽  
Sumit Chhetri ◽  
Amulya Balagani ◽  
Umm-ul-Banin B. Tayyab ◽  
Christopher J. Haas
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
High Dose ◽  
Potential Contribution ◽  
Clinical Reminder ◽  
High Dose Methylprednisolone ◽  
History Of ◽  
Rheumatological Disease ◽  
System Disorder ◽  
Activation Syndrome

Abstract Background Macrophage activation syndrome (MAS) is a rare multiorgan system disorder that may present as a fatal complication of underlying rheumatological disease, including dermatomyositis. Case presentation Here, we report the case of a 65-year-old Caucasian female with a history of psoriasis and a recent diagnosis of Coronavirus disease 2019 (COVID-19) who presented with progressive generalized weakness, joint pains, an erythematous rash, shortness of breath, and weight loss. She was ultimately diagnosed with biopsy-confirmed melanoma differentiation-associated protein 5 (MDA5)-positive dermatomyositis complicated by MAS, requiring intravenous immunoglobulin and high-dose methylprednisolone. Conclusions This report serves as a clinical reminder of the rare, yet clinically relevant association between MDA5-positive dermatomyositis and MAS, as well as highlights the potential contribution of other immune system activating diseases, such as COVID-19, associated with a cytokine storm and hyperinflammatory state.

scholarly journals High-dose anakinra as treatment for macrophage activation syndrome caused by refractory Kawasaki disease in an infant

2019 ◽  
Vol 12 (8) ◽  
pp. e229708 ◽  
Author(s):  
Marie Lind-Holst ◽  
Ulla Birgitte Hartling ◽  
Anne Estmann Christensen
Keyword(s):  
Pericardial Effusion ◽  
Kawasaki Disease ◽  
Coronary Arteries ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Additional Treatment ◽  
High Dose ◽  
Acute Phase Reactants ◽  
High Dose Methylprednisolone ◽  
Activation Syndrome

We report a 12-week-old boy presenting with incomplete refractory Kawasaki disease (KD) complicated with macrophage activation syndrome (MAS). The infant presented with cerebral irritability, pain, tachypnoea and vomiting for 10 days. He did not fulfil any of the classic diagnostic criteria for KD. Pericardial effusion on echocardiography in addition to severe dilatation of the coronary arteries in combination with leucocytosis and raised acute phase reactants led to the diagnosis of incomplete KD. Treatment with intravenous immunoglobulin and aspirin was initiated but without any response. The condition was subsequently refractory to additional treatment with infliximab and high-dose methylprednisolone. His condition worsened, fulfilling the criteria for MAS. High-dose anakinra was initiated, and remission of the inflammation was achieved.

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 Efficacy of high-dose anakinra in refractory macrophage activation syndrome in adult-onset Still’s disease: when dosage matters in overcoming secondary therapy resistance

2020 ◽  
Vol 12 ◽  
pp. 1759720X2097485
Author(s):  
Sofia Ajeganova ◽  
Ann De Becker ◽  
Rik Schots
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Adult Onset Still’S Disease ◽  
High Dose ◽  
Adult Onset ◽  
Still’S Disease ◽  
Standard Dosage ◽  
Still's Disease ◽  
Adult Onset Still's Disease ◽  
Activation Syndrome

Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of rheumatic diseases. This case demonstrates the significant challenges and therapeutic considerations in adult-onset Still’s disease (AOSD) complicated with MAS at initial presentation, which will be discussed. MAS in our patient was refractory to the first-line therapy with high-dose corticosteroids, early administration of anakinra at a standard dosage and subsequent add-on treatments with cyclosporine A, IVIG, etoposides and tocilizumab. At 2 months after presentation, the patient was still critically ill with clinical, laboratory and histological signs of an active uncontrolled MAS. Notably, adoption of anakinra at a high dosage finally induced remission. This case confirms that adjusted dosage of anakinra is an effective therapeutic strategy in a severe AOSD-related MAS. It is tempting to speculate that anakinra at a high dosage, if used earlier, would have significantly changed the course of the disease in our patient and could have led to earlier remission.

scholarly journals Macrophage activation syndrome complicating early course of adult-onset Still’s disease

2020 ◽  
pp. 83-83
Author(s):  
Ksenija Bozic ◽  
Marija Elez ◽  
Branislava Glisic
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Early Recognition ◽  
Successful Outcome ◽  
Unknown Etiology ◽  
High Dose ◽  
Adult Onset ◽  
Early Course ◽  
Elevated Liver Enzymes ◽  
Activation Syndrome

Introduction. Adult-onset Still?s disease is a rare inflammatory disorder of unknown etiology. It can be complicated by macrophage activation syndrome, potentially life-treatening condition. While macrophage activation syndrome and adult-onset Still?s disease shared similar features, early recognition is very difficult in clinical praxis. Case outline. We report a young woman, which illness was presented suddenly, with spiking fever, sore throat, myalgia, arthralgia and maculopapular rash. In suspicion of sepsis, she received antibiotics, despite no evidence of infection. After two weeks, her condition worsened, which has been followed by cytopenia, elevated liver enzymes and high serum levels of ferritin. She was diagnosed as macrophage activation syndrome in early course of adult-onset Still?s disease. She was treated with high dose corticosteroids and cyclosporine A and recovered completely. Conclusion. Macrophage activation syndrome can occur at the beginning of adult-onset Still?s disease. Early recognition and timely administration of immunosuppressive drugs are important for successful outcome in this condition.

scholarly journals Successful treatment of macrophage activation syndrome complicating adult-onset Still’s disease with tocilizumab: a case report

2017 ◽  
Vol 17 (2) ◽  
pp. 64-68
Author(s):  
Ho So ◽  
Tak-Lung Wong ◽  
Hin Ting Pang ◽  
Weng Nga Lao ◽  
Man-Lung Yip
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Adult Onset Still’S Disease ◽  
High Dose ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Initial Control ◽  
Adult Onset Still's Disease ◽  
Activation Syndrome

Abstract We report a case of 50-year-old female patient with adult-onset Still’s disease (AOSD) complicated by macrophage-activation syndrome (MAS). After initial control of the disease with high-dose parenteral corticosteroids, tocilizumab (TCZ) therapy aided in maintaining the remission with rapid tapering of steroid dose. TCZ may be useful for MAS complicating AOSD.

scholarly journals P27 Life-threatening macrophage activation syndrome with neurological involvement responding to very high dose IV anakinra

Rheumatology ◽  
2018 ◽  
Vol 57 (suppl_8) ◽  
Author(s):  
Akhila Kavirayani ◽  
Robin Basu-Roy ◽  
Seilesh Kadambari ◽  
Shelley Segal ◽  
Amrana Qureshi ◽  
...  
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Neurological Involvement ◽  
High Dose ◽  
Life Threatening ◽  
Very High ◽  
Activation Syndrome

scholarly journals AB0725 MACROPHAGE ACTIVATION SYNDROME IN CHILDREN WITH RHEUMATIC DISEASES: ANALYSIS OF CASE SERIES

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1393.3-1394
Author(s):  
M. Kaleda ◽  
I. Nikishina ◽  
V. Matkava ◽  
A. Shapovalenko ◽  
E. Fedorov ◽  
...  
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Early Stage ◽  
Clinical Manifestations ◽  
Case Series ◽  
Overlap Syndrome ◽  
High Dose ◽  
Female Ratio ◽  
Course Of Disease ◽  
Activation Syndrome

Background:Macrophage activation syndrome (MAS) is a rare, but severe life-threatening complication of chronic rheumatic disease (RD) in children, which associated with high risks of the multiple organ failure and mortality.Objectives:Tо analyze demographic, clinical and laboratory parameters, timing of MAS and disease outcome in patients (pts) with MAS and RD.Methods:The study included all pts of single center with RD, who developed the MAS. The diagnosis was recognized according to Classification criteria for MAS in sJIA [1].Results:We observed 52 pts with RD and MAS: 31 (59.6%) with sJIA, 19 (36.5%) – SLE, 1(1.9%) – juvenile dermatomyositis (JDM), 1 (1.9%) – overlap syndrome (JIA+JDM). Pts with MAS accounted of 26% of all pts with sJIA, 7.6% of all pts with SLE. The mean age of pts at onset of sJIA was 2.6 y [1.5; 5.75], at onset of SLE – 11.8 y [8.6; 13.95]. The patient with JDM was 6.5 years old, pts with overlap syndrome – 3.5 years old. Male to female ratio was 1:1,7. A total of 63 episodes of MAS was recorded (41 – in sJIA, 20 – SLE, 1 – JDM, 1 - overlap syndrome). 23 pts (44.2%) had MAS at onset, 26 pts (50%) – “classic” MAS (during the course of disease), 3 pts (5.8%) – recurrent MAS. The clinical manifestations of MAS included fever (90.4%), hepatomegaly (50%), pericarditis (46.2%), hemorrhagic rash (32.7%), neurologic involvement (42.3%), lung involvement (34.6%). We found hyperferritinemia in 98%, thrombocytopenia in 78.8%, increased transaminases in 88.5%, hypofibrinogenemia in 40.4%, hypertriglyceridemia in 42.3 % of pts. Most severe course of MAS was established in pts with ferritin levels >1500 ng/ml and with hypertriglyceridemia more than 6.0 mmol/l at an early stage. Bone marrow investigation was performed in 21 pts, and the evidence of haemophagocytosis was confirmed in 8 pts (38%). First features of MAS were fever, sleepiness, lower platelet counts, high TA. Lesions of the skin and mucous were mainly represented by point hemorrhages at an early stage in pts with SLE and MAS, the development of a bright rash with itching was typical for pts with sJIA and MAS. There are no principal differences between course of MAS in sJIA and other RD in children, but mild “subclinical” cases of MAS were observed only in pts with sJIA on biologics. For treatment of MAS all pts were administered high dose of glucocorticoids (per os+iv). Pts with SLE received: cyclophosphamide iv - 5 (26.3%), cyclosporine per os 1 (5.2%), IVIGs - 6 (31.6%), rituximab - 2 pts (10.5%). Pts with sJIA received: cyclosporine per os - 20 (64.5%), IVIGs - 25 (80.6%), 1 etoposide - (3.2%). Patient with overlap syndrome received cyclosporine per os. 8 pts (15.4%) died from MAS (3 with sJIA, 5 – with SLE).Conclusion:MAS can develop in various children’s RD, but more often in pts with sJIA. In our observation more pts had MAS during the course of disease, not at onset. Rapid recognition of MAS can improve treatment outcomes and prognosis.References:[1]Ravelli A, Minoia F, Davì S, et al. 2016.Disclosure of Interests:None declared

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