PMC7848179_01
Correlation between clinical aspects, laboratory tests and treatment of sJIA-related MAS. WBC, white blood cells n./mm3; PMNs, polymorphonuclear cells n./mm3; Hb, hemoglobin gr/dl; PLT, platelets x 103/mm3; CRP, C-reactive protein mg/l (n.v. <5); FDP, fibrinogen degradation products, mug/l (n.v. <250); FBG, serum fibrinogen g/l, (n.v.1.5 - 4.5); FER, ferritin mug/l (n.v. 20 - 250); IV HD-MPDN, intravenous methylprednisolone 30 mg/Kg/die iv; CSA, cyclosporine-A 2 mg/Kg/day; IVIG, intravenous immunoglobulin 400 mg/Kg/day; scANA, subcutaneous Anakinra 2 mg/Kg/day; HDIV-ANA, high dose intravenous Anakinra 2 mg/Kg/6 hours; CMV, Conventional mechanical ventilation; VA-ECMO, veno-arterial extracorporeal membrane oxygenation); SCLS, systemic capillary leak syndrome; MAS: macrophage activation syndrome.
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F1
Suspecting an incipient MAS, high doses IV MDPN and oral Cyclosporin A (CSA, 2 mg/Kg/day) were started (Figure 1). 24 h later (day 2) he presented a systemic capillary leak syndrome (SCLS) with rapidly increasing weight (+ 5 Kg, ~30% BW), persistent hypotension (85/45 mmHg), tachycardia (160/min) and oliguria. Laboratory test showed improvement of MAS markers (Figure 1) but also the presence of a severe neutropenia (PMNs 0-100/mm3).
Chest X-Ray during PICU admission. (A) Severe bilateral interstitial thickening and right perihilar consolidation soon after VA-ECMO placement (day 4); (B) Complete resolution of the pattern after HDIV-ANA treatment at PICU discharge (day 16).
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F2
Unfortunately, 48 h later (day 4) an episode of acute hypotension, bradycardia and oxygen desaturation led to cardiac arrest needing cardiopulmonary resuscitation and then VA-ECMO because cardiac function did not improve (Figure 2). The patient was discharged from the PICU 16 days after admission (Figure 2).