Treatment of Macrophage Activation Syndrome (MAS) With Anakinra (MAS)
Macrophage Activation Syndrome
About this trial
This is an interventional treatment trial for Macrophage Activation Syndrome
Eligibility Criteria
Inclusion Criteria:1] Previous diagnosis of systemic juvenile idiopathic arthritis (sJIA) and fulfills the Ravelli criteria (4) for macrophage activation syndrome with either:
two or more Laboratory criteria: 1. Decreased platelet count (≤262 ×10 9/L) 2. Elevated levels of aspartate aminotransferase (>59 U/L) 3. Decreased white blood cell count (≤4.0 × 109/L) 4. Hypofibrinogenemia (≤2.5 g/L) or, three or more combined clinical/laboratory criteria:
- Decreased platelet count (≤262 × 109/L)
- Elevated levels of aspartate aminotransferase (>59 U/L)
- Decreased white blood cell count (≤4.0 × 109/L)
- Hypofibrinogenemia (≤2.5 g/L)
- Central nervous system dysfunction (irritability, disorientation, lethargy, seizures, coma)
- Hemorrhages (purpura, easy bruising, mucosal bleeding)
- Hepatomegaly (≥3 cm below the costal margin or confirmed by imaging)
OR
2] No previous diagnosis of sJIA and serum ferritin > 2,000 ng/ml and 3 out of the following:
Bicytopenia with two of the following:
- Absolute Neutrophil Count < 1,000,
- Platelets < 100, 000/mm3,
- Hemoglobin < 9 mg/dl
- Fasting triglyceride >265 mg/dL
- Splenomegaly
- ALT OR AST > 120 IU/L (or > 2x upper limit of normal)
- Fever with temp ≥ 101° F
Fibrinogen < 1.5 g/L (150 mg/dl) or INR > 1.5 or d-dimer > 500 ng/ml
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Exclusion Criteria:
- Evidence of malignancy
- Culture evidence of systemic bacterial infection at the time of screening
- Known EBV viremia by PCR at time of screening (positive serologies are not an exclusion; results of EBV testing will not be necessary for enrollment, but may be ordered as part of the standard of care assessment to guide future management as results become available)
- Previous treatment for the current MAS episode with corticosteroids, anakinra, tocilizumab, anti-TNF therapy or cyclosporine
- <1 year of age
- Family history of familial HLH
Evidence of any of the following
- Creatinine at the time of screening > 2X ULN or > twofold increase from patient's baseline creatinine within past 3 months (if known)
- Albumin < 1.5 at the time of screening
- Mechanical ventilation at the time of screening
- Hypotension requiring use of pressors at the time of screening
Sites / Locations
- University of Alabama at Birmingham
Arms of the Study
Arm 1
Arm 2
Placebo Comparator
Experimental
placebo
anakinra (Kineret)
methylprednisolone intravenously, placebo shots every 6 hours
methylprednisolone intravenously, anakinra shots every 6 hours