Article Text

AB0921 Secondary Macrophage Activation Syndrome: Report of 13 Clinical Cases and the Difference between the 4 Groups of Diseases
  1. C.A. Egües Dubuc,
  2. C. Meneses Villalba,
  3. N. Errazquin Aguirre,
  4. M. Uriarte Ecenarro,
  5. I. Hernando Rubio,
  6. O. Maiz Alonso,
  7. I. Bañegil Espinoza,
  8. J. Cancio Fanlo,
  9. M. Yague Asensio,
  10. E. Uriarte Isacelaya,
  11. J. Belzunegui Otano
  1. Rheumatology, Donostia University Hospital, San Sebastian, Spain


Background The secondary macrophage activation syndrome (SMAS) is a group of diseases that include the: Autoimmune (AI), Hemato-Oncological (HO), Infectious (Inf) and Oncological (Onco). We make a review of the the cases presented in a our hospital.

Objectives Describe the characteristics demographic, clinical, laboratory, treatment, underlying disorders and mortality of patients diagnosed with SMAS.

Methods A cohort was studied retrospectively of patients diagnosed with SMAS between the period December/2008-January/2014 by reviewing medical records from the diagnosis until January/2014. The nominal variables were: diagnosis, treatments during hospitalization and after discharge. Dichotomous variables were: sex, fever, organomegaly, mortality, admission to the Intensive Care Unit (ICU) and recurrence of SAMS. Quantitative variables were: age, laboratory findings, hospital stay, days from admission to the bone marrow biopsy (BMB). Patients were divided into 4 groups (AI, HO, Inf and Onc). The variables were analyzed between the 4 groups and between AI and HO. Quantitative variables showed asymetric distribution so it showed with the median and interquartile range. For the bivariate analysis the Kruskal-Wallis, Pearson Chi Square and Fisher's Exact tests were used.

Results 13 patients (6 female) with median age of 54 (32-63) were found: 5 with AI diseases (2 Systemic Lupus Erythematosus, 2 Adult Still's Disease and 1 IgG4 associated disease), 5 HO diseases (3 Non Hogkin Lymphoma, 1 acute myeloid leukemia and 1 Lymphoma of Natural Killers cells), 2 Inf. diseases (Campylobacter jejuni and human immunodeficiency virus) and 1 Onc. disease (Chemotherapy in Glioblastoma multiforme). Table shows the descriptive and bivariate analysis of the most important variables. The treatments used were: glucocorticoids in all patients, immunoglobulins in 8 (3 AI, 2 HO, 2 Onc. and 1 Inf.), Cyclosporine in 8 (5 AI and 3 HO), anakinra in 4 (2 HO and 2 AI), tocilizumab and chemotherapy in 2 HO.

Table 1

Conclusions We found that leukopenia was lower in AI disease than HO diseases with statistically significant p. AI diseases had worse prognosis, increased mortality and hospital days than other diseases.

Disclosure of Interest None declared

DOI 10.1136/annrheumdis-2014-eular.5055

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