Macrophage Activation Syndrome in a Patient with Systemic-Onset Juvenile Idiopathic Arthritis Initially Treated for Atypical Kawasaki Disease : A Case-Based Review

2018 ◽  
Vol 66 (1) ◽  
pp. 49-53
Author(s):  
Essa Hariri ◽  
Rawan Demachkieh ◽  
Ahmad Naja
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Kawasaki Disease ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Atypical Kawasaki Disease ◽  
Systemic Onset ◽  
Case Based ◽  
Activation Syndrome

Abstract O.36: Kawasaki Disease and Systemic Juvenile Idiopathic Arthritis - Two Ends of the Same Spectrum

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Rae S Yeung ◽  
Mira van Veenendaal ◽  
Cedric Manlhiot ◽  
Rayfel Schneider ◽  
Brian W McCrindle
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Coronary Artery ◽  
Kawasaki Disease ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Presumptive Diagnosis ◽  
Treatment Regimens ◽  
Hepatic Transaminases ◽  
Activation Syndrome

Background: Clinical similarities between Kawasaki disease (KD) and systemic Juvenile Idiopathic Arthritis (sJIA) are well known. We critically reviewed children with KD and sJIA to identify those with both diagnoses in order to characterize discriminating findings at baseline. Methods: Data from prospectively acquired KD (n=1765) and sJIA (n=112) cohorts were reviewed for common patients (1990-2011). Those with both diagnoses (KD/sJIA n=8) were reviewed for clinical presentation, laboratory investigations, treatment regimens, coronary artery outcome and complications including macrophage activation syndrome, and results were compared to the overall KD cohort. Results: All children with KD/sJIA fulfilled diagnostic criteria for KD and sJIA (ILAR classification). Co-diagnosis was present in 0.45% (8 of 1765) and 7.1% (8 of 112) of those with KD and sJIA, respectively. Time between diagnosis of KD and presumptive diagnosis of sJIA was a median of 24 days (IQR 21-45 days). KD/sJIA patients had bilateral conjunctival injection less frequently, lower hepatic transaminases together with signs of more intense inflammation as expressed a by higher white blood cell count and lower albumin than the KD cohort alone. All KD/sJIA patients had recalcitrant disease consisting of prolonged fever requiring multiple doses of intravenous immunoglobulin and steroids. Coronary artery abnormalities (CAAs) were observed in 5 KD/sJIA patients. Macrophage activation syndrome occurred in one KD/sJIA patient and in 0.9% and 8% of KD patients and sJIA patients respectively. Conclusions: A small portion of our patients with KD developed subsequent sJIA. KD/sJIA patients were characterized by more intense inflammation at initial presentation, a recalcitrant disease course and a high prevalence of CAAs. These patients may provide clues to potentially shared immunopathology. The clinical presentations of MAS, KD and sJIA are quite similar with fever, rash, hepatomegaly, and lymphadenopathy. All 3 entities are syndrome complexes defined by massive immune activation. We propose that the intensity and duration of the immune response may be the key distinguishing features, which dictate which one of these clinical syndromes the affected child presents with.

Sign in / Sign up

Export Citation Format

Share Document