scholarly journals Macrophage activation syndrome at the onset of glucocorticoid-resistant systemic lupus erythematosus: a case report

2018 ◽  
Vol 56 (1) ◽  
pp. 67-70
Author(s):  
Delia Tulbă ◽  
Marius Balea ◽  
Cristian Băicuş
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Side Effects ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Glucocorticoid Resistance ◽  
High Dose ◽  
Systemic Lupus ◽  
Activation Syndrome

Abstract Introduction. Macrophage activation syndrome (MAS) is a life-threatening hyperinflammatory state mediated by uncontrolled cytokine storm and haemophagocytosis. Although rarely reported, MAS might occur in systemic lupus erythematosus (SLE), notably as an inaugural manifestation. Glucocorticoids (GCs) are the cornerstone of SLE therapy. However, in some cases high doses of GCs are required to achieve remission (i.e. glucocorticoid-resistance), leading to significant side effects. Case report. A 28-year-old Romani male was admitted to our hospital for polyarthralgia, polyserositis and fatigability. The patient had high-grade fever, jaundice and generalized lymphadenopathy. Laboratory tests revealed severe mixed hemolytic autoimmune anemia, leukopenia, hepatocytolysis, coagulation abnormalities, hypertriglyceridemia, biological inflammatory syndrome, hyperferritinemia and persistent proteinuria of nephritic pattern. Imaging studies showed pleuropericardial effusion, hepatosplenomegaly and polysynovitis. Additional blood tests revealed hypocomplementemia and positive ANA, anti-dsDNA and anti-Sm antibodies. Haemophagocytosis was not identified either on bone marrow or axillary lymph node biopsy specimens. However, SLE-associated MAS seemed to fit this set-up. High-dose corticotherapy (6.5 g methylprednisolone followed by prednisone, 1.5 mg/kg/day after discharge) and intravenous cyclophosphamide were necessary to induce and sustain remission. Conclusion. MAS is a potentially severe manifestation that should be considered at SLE onset whenever high fever and elevated serum levels of aspartate aminotransferase, lactate dehydrogenase, C-reactive protein, ferritin and procalcitonin are noted. Early diagnosis and prompt treatment lead to remission in two thirds of cases. Glucocorticoid-resistance leads to the use of high-dose corticotherapy or immunosuppressive agents that could elicit serious side effects. New insights into the molecular mechanisms of glucocorticoid-resistance are needed in order to conceive more adequate GC-therapies.

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 MACROPHAGE ACTIVATION SYNDROME PRESENTING AS THE FIRST MANIFESTATION OF SYSTEMIC LUPUS ERYTHEMATOSUS: CASE REPORT AND LITERATURE REVIEW

2021 ◽  
Author(s):  
Renata Maria Monteiro Pinto ◽  
Adriana Cristiane Machado ◽  
Paula de Medeiros Nacácio e Silva ◽  
Barbara Luzia Berredo Reis Alexandre ◽  
Marina de Azevedo Martins ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Literature Review ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Systemic Lupus ◽  
Systemic Lupus Erythematosus Case ◽  
Activation Syndrome

scholarly journals Macrophage Activation Syndrome as Onset of Systemic Lupus Erythematosus: A Case Report and a Review of the Literature

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Guido Granata ◽  
Dario Didona ◽  
Giuseppina Stifano ◽  
Aldo Feola ◽  
Massimo Granata
Keyword(s):  
Rheumatoid Arthritis ◽  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Treatment Options ◽  
Review Of The Literature ◽  
Systemic Lupus ◽  
Activation Syndrome

Macrophage activation syndrome (MAS) is a potentially fatal condition. It belongs to the hemophagocytic lymphohistiocytosis group of diseases. In adults, MAS is rarely associated with systemic lupus erythematosus, but it also arises as complication of several systemic autoimmune disorders, like ankylosing spondylitis, rheumatoid arthritis, and adult-onset Still’s disease. Several treatment options for MAS have been reported in the literature, including a therapeutic regimen of etoposide, dexamethasone, and cyclosporine. Here we report a case of 42-year-old woman in whom MAS occurred as onset of systemic lupus erythematosus.

scholarly journals Arthritis and use of hydroxychloroquine associated with a decreased risk of macrophage activation syndrome among adult patients hospitalized with systemic lupus erythematosus

Lupus ◽  
2018 ◽  
Vol 27 (7) ◽  
pp. 1065-1071 ◽  
Author(s):  
E M Cohen ◽  
K D’Silva ◽  
D Kreps ◽  
M B Son ◽  
K H Costenbader
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Hospital Admission ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Activity Index ◽  
Conditional Logistic Regression ◽  
Systemic Lupus ◽  
Factors Associated ◽  
Activation Syndrome

Background Macrophage activation syndrome (MAS) is an uncommon but serious complication of systemic lupus erythematosus (SLE). We aimed to identify factors associated with MAS among adult hospitalized SLE patients. Methods Within the Brigham and Women’s Hospital (BWH) Lupus Center Registry, we identified adult SLE patients > age 17 who had been hospitalized from 1970 to 2016, with either ferritin > 5000 ng/ml during admission or “macrophage activation syndrome” or “MAS” in discharge summary. We confirmed MAS by physician diagnosis in medical record review. We matched each hospitalized SLE patient with MAS to four SLE patients hospitalized without MAS (by SLE diagnosis date ±1 year). We employed conditional logistic regression models to identify clinical factors associated with MAS among hospitalized SLE patients. Results Among 2094 patients with confirmed SLE, we identified 23 who had a hospitalization with MAS and compared them to 92 hospitalized without MAS. Cases and controls had similar age at SLE diagnosis (29.0 vs. 30.5, p = 0.60), and hospital admission (43.0 vs. 38.3, p = 0.80), proportion female (78% vs. 84%, p = 0.55), and time between SLE diagnosis and hospitalization (1971 vs. 1732 days, p = 0.84). Arthritis (OR 0.04 (95% CI 0.004–0.35)) and hydroxychloroquine use (OR 0.18 (95% CI 0.04–0.72)) on admission were associated with decreased MAS risk. Admission Systemic Lupus Erythematosus Disease Activity Index scores (30 vs. 19, p = 0.002) and lengths of stay (16 days vs. 3 days, p < 0.0001) were much higher among cases. Death during hospitalization was 19% among cases and 3% among controls ( p = 0.03). Conclusions In this case-control study of hospitalized adult SLE patients, arthritis and hydroxychloroquine use at hospital admission were associated with decreased MAS risk. Further studies are needed to validate these factors associated with lowered MAS risk.

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