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Abstract Number: 079

Traditional Laboratory Parameters and New Biomarkers in Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis

Arianna De Matteis 1, Denise Pires Marafon 2, Ivan Caiello 2, Manuela Pardeo 2, Giulia Marucci 2, Emanuela Sacco 2, Giusi Prencipe 2, Fabrizio De Benedetti 2 and Claudia Bracaglia2, 1Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Lazio, Italy, 2Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy

Meeting: 2020 Pediatric Rheumatology Symposium

Keywords: Biomarkers, chemokines, interferons, macrophage activation syndrome, Systemic JIA

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Session Information

The 2020 Pediatric Rheumatology Symposium, originally scheduled for April 29 – May 2, was postponed due to COVID-19; therefore, abstracts were not presented as scheduled.

Date: Friday, May 1, 2020

Session Title: Poster Session 2

Session Type: ACR Abstract Session

Session Time: 5:00PM-6:00PM

Background/Purpose: Macrophage Activation Syndrome (MAS) and Secondary Hemophagocytic Lymphohistiocytosis (sHLH) are hyperinflammatory conditions caused by a cytokine storm. Prompt recognition and early treatment are essential to reduce the high disease mortality. Data in human and animals point to a pivotal role of IFN-γ in sHLH and in MAS in the context of systemic Juvenile Idiopathic Arthritis (sJIA).

Methods: Routine laboratory parameters of disease activity and severity were collected from a cohort of patients with sHLH (n=35), MAS in the context of sJIA (n=21), and sJIA (n=25) at 3 different time points: active disease (T0), 7-10 days from starting therapy (T1) and in clinical inactive disease (1 to 3 months from onset) (T2). Serum levels of the IFN-γ related biomarkers (CXCL9, CXCL10 and Neopterin) were measured at each time point by ELISA.

Results: A total of 265 samples were collected (data are detailed in Table 1). 33 (94%) patients with sHLH met the HLH-2004 diagnostic criteria, while 20 (95%) patients with MAS met the 2016 classification criteria for MAS. Fever was present in the majority of patients (93%), while hepatomegaly and splenomegaly were observed more frequently in MAS and sHLH patients (98% and 94% respectively) compared to sJIA (29.4% for both characteristics). Laboratory parameters at T0 are shown in Table 1. Using the 2016 classification criteria for MAS, we found that platelet count is a specific parameter, no patients with sJIA without MAS presented a value 684 mg/ml. Lactate dehydrogenase (LDH) levels are statistically higher in MAS and sHLH groups compared to sJIA. The ROC curve for LDH in MAS group shows an area under the curve (AUC= 0.89, p-value < 0.0001) with high sensitivity and specificity for a cut-off of 712 UI (79.3% and 79.4% respectively). CXCL9, CXCL10 and neopterin levels in T0 were significantly higher in MAS and sHLH patients compared to sJIA (Table 2). The ROC curves performed for each biomarker showed a statistically significant AUCs (p< 0.0001) and identified a cut off value for each biomarker in MAS and sHLH groups (Figure 1). CXCL9, CXCL10 and neopterin levels decreased progressively at T1 with a normalization in T2. CXCL9 decreased faster compared to neopterin, similarly to the decrease of the routine laboratory parameters.

Conclusion: Our results confirmed that platelet counts and ferritin are two relevant laboratory parameters, with high specificity and sensitivity respectively, to diagnose MAS in the context of sJIA. Even if LDH is not included in 2016 classification criteria for MAS in sJIA, we found that, in addition to the others, this parameter could help to discriminate MAS in sJIA. Moreover, our results confirmed that the IFN-γ related biomarkers are significantly increased in patients with MAS and sHLH and could be useful for diagnosis in addition to traditional laboratory parameters. Moreover, these biomarkers seem to be also useful to monitor clinical evolution and treatment response.

Table 1. Number of samples at T0, T1, T2 and laboratory parameters at T0.

Table 2. IFN_γ related biomarkers during active disease at T0.

Figure 1. ROC curve for CXCL9 and neopterin at T0 in MAS and sHLH.


Disclosure: A. De Matteis, None; D. Pires Marafon, None; I. Caiello, None; M. Pardeo, None; G. Marucci, None; E. Sacco, None; G. Prencipe, None; F. De Benedetti, Novartis, 1, 2, Novimmune, 1, Sobi, 1, 2, Roche, 1, 2, Pfizer, 1, Sanofi, 1, AbbVie, 1, Novimmune/Sobi, 1; C. Bracaglia, None.

To cite this abstract in AMA style:

De Matteis A, Pires Marafon D, Caiello I, Pardeo M, Marucci G, Sacco E, Prencipe G, De Benedetti F, Bracaglia C. Traditional Laboratory Parameters and New Biomarkers in Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 4). https://acrabstracts.org/abstract/traditional-laboratory-parameters-and-new-biomarkers-in-macrophage-activation-syndrome-and-secondary-hemophagocytic-lymphohistiocytosis/. Accessed May 26, 2022.
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