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Therapeutic choices for patients with macrophage activation syndrome vary geographically
The disease spectrum was found to be similar in patients around the world with macrophage activation syndrome associated with juvenile idiopathic arthritis, but treatment choices varied by region, according to the results of a study of 362 patients by 95 researchers in 33 countries.
Patient data, including information about demographics, triggering factors, symptoms, laboratory examinations, histopathologic characteristics, cardiovascular and pulmonary symptoms, interventions and outcomes were collected from patients with juvenile idiopathic arthritis (JIA) who developed macrophage activation syndrome (MAS).
Laboratory samples were collected during patients’ visits, including during the last visit prior to onset of MAS, at the time MAS was suspected and during full MAS activity.
Patient data were analyzed in stratified samples by geographic location, the subspecialty of the attending physician (pediatric rheumatology or hemato-oncologist), the presence of hemophagocytosis in bone marrow aspirate or reticuloendothelial organ biopsy and level of disease activity.
Of 362 patients, 179 were located in Europe (49.4%), 72 in North America (19.9%) and 111 in other locations (30.7%). Pediatric rheumatologists treated 88.2% of the patients, and 79% were treated by pediatric hemato-oncologists, the latter more common in North America. Bone marrow aspirate or reticuloendothelial organ biopsy data were available for 252 of the patients.
Macrophage hemophagocytosis was observed in 159 patients (45.7%), and in 96 patients, histopathologic confirmation by biopsy was not performed.
Severe course of disease was observed in 92 of 347 patients (26.5%), who were likely to be older, have longer JIA disease duration at MAS onset, hepatomegaly and other organ involvement, according to the researchers. Patients in Europe were less likely to have central nervous system involvement but more likely to have higher levels of aspartate aminotransferase (AST) than other patients.
Hemoglobin levels were lower in patients treated in other locations compared with patients in North America or Europe, but overall outcomes were worse. In North America, patients were more likely to be treated with intravenous immunoglobin and biologic medications than in Europe or other locations, the researchers reported.
Patients treated by a pediatric hemato-oncologist were more likely to have heart, lung or kidney failure and were more likely to receive a biologic or etoposide treatment. Cyclosporine was more commonly prescribed by pediatric rheumatologists. Other patient data were comparable between the groups, according to the researchers.
The researchers concluded that a need exists to conduct clinical trials or develop consensus initiatives to define uniform treatment protocols of MAS in patients with JIA. - by Shirley Pulawski
Disclosure: The researchers report no relevant financial disclosures.