scholarly journals SAT0222 CLINICAL SPECTRUM AND LONG TERM FOLLOWUP OF SYSTEMIC LUPUS ERYTHEMATOSUS-ASSOCIATED MACROPHAGE ACTIVATION SYNDROME

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1054.2-1054
Author(s):  
T. Okano ◽  
J. Saegusa ◽  
K. Yoneda ◽  
I. Shirasugi ◽  
Y. Ueda ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Classification Criteria ◽  
Single Center ◽  
Retrospective Design ◽  
One Year ◽  
Center Site ◽  
Activation Syndrome

Background:Systemic lupus erythematosus (SLE) patients present with variable clinical features ranging from mild joint and skin involvement to life-threatening organ involvement such as nephritis, neuropsychiatric involvement, diffuse alveolar hemorrhage, and hemophagocytic lymphohistiocytosis (HLH). HLH is rare, but fatal complication of SLE. Recently, European League Against Rheumatism, the American College of Rheumatology, and the Pediatric Rheumatology International Trials Organization was to develop a set of classification criteria for MAS complicating systemic juvenile idiopathic arthritis (PRINTO criteria) [1]. Sung Soo Ahn and his colleagues reported PRINTO criteria predicted mortality of adult SLE patient, but they followed only one year [2].Objectives:To reveal association PRINTO criteria with long term mortalities in SLE patient in our Hospital.Methods:We performed a retrospective analysis of SLE patients who received moderate dose glucocorticoid therapy (>0.4mg/kg/d) in our hospital between April 2008 and April 2019. Patients were evaluated for HLH using the 2016 PRINTO classification criteria for MAS. Clinical features and laboratory findings were compared and overall survival rate was analyzed.Results:Among 164 episode (144 patients) with SLE, 31 episode (31 patients) 5.2% were considered to have MAS on admission.The overall survival rate was significantly lower in patients with MAS than without MAS (86.2% vs. 95.3%, p = 0.048). Interestingly, SLEDAI had no association with mortality, relapse rate, and MAS complication. SLEDAI more focused on renal and neuropsychiatric symptoms than hematologic features. So SLEDAI might not be associated with MAS secondary to SLE. Furthermore, we observed no death patient with MAS after one year, and only 1 case relapse in MAS patient. So MAS might have fatal but less relapsing property compared with other lupus cases.Conclusion:PRINTO criteria may be useful to differentiated fatal MAS patients from others. Further investigations are required to confirm our findings.Limitation The main limitations of our study include its retrospective design, single center site, and that the number of admitted patients with SLE was small.Limitation:The main limitations of our study include its retrospective design, single center site, and that the number of admitted patients with SLE was small.Limitation:Limitation The main limitations of our study include its retrospective design, single center site, and that the number of admitted patients with SLE was small.References:[1]Wulffraat N, Schneider R, Filipovic L, et al. 2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis. Ann Rheum Dis 2016;75:481–9.[2]Ahn SS, Yoo BW, Jung SM, et al. In-hospital mortality in febrile lupus patients based on 2016 EULAR/ACR/PRINTO classification criteria for macrophage activation syndrome. Semin Arthritis Rheum 2017;47:216–21.Disclosure of Interests:None declared

scholarly journals Tocilizumab modifies clinical and laboratory features of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

2020 ◽  
Vol 18 (1) ◽  
Author(s):  
Masaki Shimizu ◽  
Mao Mizuta ◽  
Nami Okamoto ◽  
Takahiro Yasumi ◽  
Naomi Iwata ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Clinical Features ◽  
Early Treatment ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Classification Criteria ◽  
Real Patient ◽  
Laboratory Features ◽  
Activation Syndrome

Abstract Background This study aimed to determine the influence of tocilizumab (TCZ) in modifying the clinical and laboratory features of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (s-JIA). Furthermore, we assessed the performance of the 2016 MAS classification criteria for patients with s-JIA-associated MAS while treated with TCZ. Methods A panel of 15 pediatric rheumatologists conducted a combination of expert consensus and analysis of real patient data. Clinical and laboratory features of s-JIA-associated MAS in 12 TCZ-treated patients and 18 untreated patients were evaluated. Possible MAS was defined as having characteristic laboratory features but lack of clinical features of MAS, or atypical MAS, or early treatment that prevented full-blown MAS. Results Clinically, the TCZ-treated patients with s-JIA-associated MAS were less likely febrile and had significantly lower ferritin, triglyceride, and CRP levels than the untreated patients with s-JIA-associated MAS. Other laboratory features of MAS including lower platelet counts and lower fibrinogen were more pronounced in TCZ-treated patients. The TCZ-treated patients with s-JIA-associated MAS were less likely to be classified as MAS based on the MAS classification criteria (25% vs 83.3%, p < 0.01). This is ascribed to the absence of fever or insufficient ferritin elevation, compared with the untreated patients. Conclusion TCZ could modify the clinical and laboratory features of s-JIA-associated MAS. When evaluating the s-JIA patients while treated with TCZ, it is not applicable to use MAS classification criteria. Care must be taken to not underdiagnose MAS based on the MAS classification criteria.

2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

2016 ◽  
Vol 75 (3) ◽  
pp. 481-489 ◽  
Author(s):  
Angelo Ravelli ◽  
Francesca Minoia ◽  
Sergio Davì ◽  
AnnaCarin Horne ◽  
Francesca Bovis ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Consensus Conference ◽  
Classification Criteria ◽  
Systemic Jia ◽  
Patient Profiles ◽  
Activation Syndrome

To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of 28 experts was first asked to classify 428 patient profiles as having or not having MAS, based on clinical and laboratory features at the time of disease onset. The 428 profiles comprised 161 patients with systemic JIA—associated MAS and 267 patients with a condition that could potentially be confused with MAS (active systemic JIA without evidence of MAS, or systemic infection). Next, the ability of candidate criteria to classify individual patients as having MAS or not having MAS was assessed by evaluating the agreement between the classification yielded using the criteria and the consensus classification of the experts. The final criteria were selected in a consensus conference. Experts achieved consensus on the classification of 391 of the 428 patient profiles (91.4%). A total of 982 candidate criteria were tested statistically. The 37 best-performing criteria and 8 criteria obtained from the literature were evaluated at the consensus conference. During the conference, 82% consensus among experts was reached on the final MAS classification criteria. In validation analyses, these criteria had a sensitivity of 0.73 and a specificity of 0.99. Agreement between the classification (MAS or not MAS) obtained using the criteria and the original diagnosis made by the treating physician was high (κ=0.76). We have developed a set of classification criteria for MAS complicating systemic JIA and provided preliminary evidence of its validity. Use of these criteria will potentially improve understanding of MAS in systemic JIA and enhance efforts to discover effective therapies, by ensuring appropriate patient enrollment in studies.

scholarly journals PRECORDIAL PAIN, LEUKOCYTOSIS AND BICYTOPENIA IN A TEENAGER WITH SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS UNDER IMMUNOSUPPRESSIVE THERAPY

2019 ◽  
Vol 37 (2) ◽  
pp. 252-256 ◽  
Author(s):  
Marina de Sousa Vieira ◽  
Flávia Regina de Andrade ◽  
Letícia Maria Kolachinski Raposo Brandão ◽  
Virgínia Tafas da Nóbrega ◽  
Vanessa Ramos Guissa ◽  
...  
Keyword(s):  
Heart Failure ◽  
Juvenile Idiopathic Arthritis ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Systemic Disease ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Classification Criteria ◽  
Morbidity And Mortality ◽  
Disease Case ◽  
Activation Syndrome

ABSTRACT Objective: To highlight the importance of the new classification criteria for the macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis in order to reduce morbidity and mortality outcome related to this disease. Case description: A 12-year-old female patient with diagnosis of systemic juvenile idiopathic arthritis under immunosuppression therapy for two years developed cough, acute precordial chest pain, tachypnea, tachycardia and hypoxemia for two days. Chest tomography showed bilateral laminar pleural effusion with bibasilar consolidation. The electrocardiogram was consistent with acute pericarditis and the echocardiogram showed no abnormalities. Laboratory exams revealed anemia, leukocytosis and increased erythrocyte sedimentation rate, as well as C-reactive protein rate and serum biomarkers indicative of myocardial injury. Systemic infection and/or active systemic juvenile idiopathic arthritis were considered. She was treated with antibiotics and glucocorticoids. However, 10 days later she developed active systemic disease (fever, evanescent rash and myopericarditis with signs of heart failure) associated with macrophage activation syndrome, according to the 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis. She was treated for five days with pulse therapy, using glucocorticoids, immunoglobulin and cyclosporine A, with improvement of all clinical signs and laboratory tests. Comments: Myopericarditis with signs of heart failure associated with MAS is a rare clinical presentation of systemic juvenile idiopathic arthritis. Macrophage activation syndrome occurs mainly during periods of active systemic juvenile idiopathic arthritis and may be triggered by infection. Knowledge about this syndrome is crucial to reduce morbidity and mortality.

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