Clinical and laboratory observation
Macrophage activation syndrome as the presenting manifestation of rheumatic diseases in childhood

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We describe 3 patients who presented with features of macrophage activation syndrome (MAS) at the time of presentation of systemic lupus erythematosus (SLE), systemic juvenile idiopathic arthritis, and Kawasaki disease. Immunohistochemical studies in the patient with SLE demonstrated extensive expression of CD163 on hemophagocytic macrophages, suggesting a possible role as a marker of MAS.

Section snippets

Case 1

A previously healthy 15-year-old girl presented with fever, difficulty breathing, abdominal pain, vomiting, and watery diarrhea. On admission, she had a temperature of 40.0°C, heart rate of 100 beats/minute, and blood pressure of 100/60 mm Hg. She exhibited no rash, mucous membrane abnormalities, hepatosplenomegaly, lymphadenopathy, or arthritis (Table I).

The serial values of her laboratory tests are given in Table II. Her infectious disease workup demonstrated positive urine (Escerichia coli >

Discussion

MAS is a serious complication of childhood rheumatic diseases caused by excessive activation and proliferation of T lymphocytes and macrophages. The etiology of this syndrome is not fully understood; however, it appears that MAS develops due to an abnormality in immune regulation that contributes to the lack of control of an exaggerated immune response. MAS may be triggered by infections or changes in medications during the course of primary rheumatic disease.4, 8 We have described 3 patients

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    Most studies focus on MAS in the context of systemic juvenile idiopathic arthritis (SJIA), the pediatric equivalent of adult-onset Still’s disease (AOSD).7 Case reports abound of MAS occurring in other rheumatic diseases,8 particularly systemic lupus erythematosus,9 in which it is likely the most common cause of adult MAS.10 Criteria have been developed to distinguish MAS from primary HLH11 and from SJIA disease flare,12 but are less widely used than the 2016 criteria classifying SJIA-associated MAS.13

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    Thus, high index of suspicion and proper investigations are crucial to diagnose MAS in cSLE. Several studies showed that sIL-2r is considered a good marker to detect MAS in children with systemic juvenile idiopathic arthritis (sJIA) and SLE [22,23]. Ferritin is an intracellular protein that functions as an acute phase reactant, it has been positively correlated with disease activity scores of adult SLE and rheumatoid arthritis as well [9,24,25].

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    Upon appropriate activation of the cells, the extracellular portion of CD163 is shed from the cell surface in the form of soluble CD163 (sCD163). Previously, extensive expansion of CD163+ macrophages has been reported in the bone marrow of a patient with MAS [6]. Reportedly, the serum sCD163 levels are a valuable diagnostic marker in hemophagocytic syndromes and MAS [10,11].

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