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CLINICAL OUTCOMES OF MACROPHAGE ACTIVATION SYNDROME IN UNIVERSITY HOSPITAL CENTRE

Background: Macrophage activation syndrome (MAS) is an infl ammatory, potentially life-threatening process that accompanies autoimmune diseases. It is more common in children than in adults. Outcome success depends on early diagnosis and prompt treatment. Objectives: To evaluate causes and clinical features of MAS, and to discuss clinical pitfalls, with respect to available 2016 classifi cation criteria (EULAR/ACR PRINTO for MAS in JIA)1. Methods: Retrospective study (2012–2018) included 6 patients. Th e basic characteristics (age, gender, comorbidities), laboratory fi ndings, biopsy (bone marrow, lymph node, liver) results, treatment, underlying disease, clinical course of illness and outcome were reported. Results: Four female and two male patients, age (26–71), were diagnosed with MAS. MAS was the fi rst manifestation of autoimmune disease in one patient, Adult onset Still disease (AOSD), and appeared in previously known Rheumatoid arthritis (RA) and Undiff erentiated Connective Tissue Disease (UCTD). Triger infections were Cytomegalovirus (CMV) and Clamidophila pneumoniae. In one patient, antibodies to Syogren syndrome (SS) and Primary biliary cirrhosis occured aft er one year of follow up. Laboratory abnormalities included elevated liver enzymes and LDH and trombocytopenia. Th e hallmark was ferritin level above 5000 [ug/L]. Poorer outcomes were observed in cases of delayed diagnosis (≥20 days) and in elderly patients with comorbidites. All patients were treated with glucocorticoids and cyclosporin, and if necessary etoposide. One patient had refractory disease without any evidence of infectious, malignant or autoimmune background despite additional therapy with IVIG, IL-1 and IL-6 inhibitors, and probably had an primary form.Th ree patients had a lethal outcome. More detailed information are shown in Table 1. Conclusions: A combination of symptoms and laboratory fi ndings of high fever, increased ferritin level, elevated liver function tests and trombocytopenia should always arouse suspicion of MAS in patient with known predisposing factors. Recognising trigers and aggressive treatment could signifi cantly improve survival outcomes.

Macrophage activation syndrome

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