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Patients with adult-onset Still disease with macrophage activation syndrome show poorer prognosis
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Patients with adult-onset Still disease who developed macrophage activation syndrome had poorer outcomes than patients who did not, according to a study from the Ajou University School of Medicine in Suwon, Korea.
Researchers retrospectively identified 109 patients with adult-onset Still disease (AOSD) from hospital records between 1996 and 2013. All patients underwent laboratory tests that included C-reactive protein (CRP), complete blood count, erythrocyte sedimentation rate (ESR), ferritin, fibrinogen, lactate dehydrogenase (LDH), liver function tests, triglyceride levels and urinalysis. Systemic disease score was evaluated, and patients with cytopenia or unknown fever underwent bone marrow, liver, or lymph node biopsies.
Twenty-one (19.3%) of the patients with AOSD were diagnosed with macrophage activation syndrome (MAS), also known as reactive hemophagocytic syndrome. An additional 17 patients with primary hemophagocytic lymphohistiocytosis (HLH) and no autoimmune diseases were also studied.
MAS was confirmed in seven patients (6.4%) by bone marrow, liver or lymph node biopsy, and 14 patients (12.8%) without biopsy confirmation were considered to have probable MAS, which was characterized by high fever; unexplained, progressive cytopenia in at least two cell lineages; occurrence of MAS since diagnosis of AOSD despite treatment and the exclusion of MAS associated with malignancy.
Statistical analysis revealed active disease flare, infection and possibly antibiotics as potential triggers. No significant differences were seen between patients who developed MAS and those who did not in sex, age at diagnosis, disease score or follow-up period, according to the researchers.
White blood cell and platelet counts, hemoglobin, albumin, and fibrinogen levels were lower in patients who developed MAS compared with those who did not, but ferritin, LDH and triglyceride levels were significantly higher in patients with MAS. Patients with MAS without biopsy confirmation had similar laboratory results, according to the researchers.
Overall survival was not different between patients who developed MAS and patients who did not; however, patients with MAS had higher frequencies of ICU admission, splenomegaly, hepatomegaly and lymphadenopathy, but lower incidences of sore throat.
Patients with HLH were more likely to have hepatomegaly, lymphadenopathy and pneumonitis, along with higher frequencies of ICU admissions and mortality rates than patients with AOSD who developed MAS, but treatment methods were different between the groups, according to the researchers. – by Shirley Pulawski
Disclosure: The researchers report no relative financial disclosures.
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