Hypocomplementemia Associated with Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis and Adult Onset Still’s Disease: 3 Cases: Table 1.
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To the Editor: Macrophage activation syndrome (MAS) is a life-threatening complication seen in association with childhood inflammatory diseases, most commonly in systemic juvenile idiopathic arthritis (sJIA)1,2,3. MAS has also been described in the setting of adult-onset Still’s disease (AOSD), systemic lupus erythematosus, drug reactions, and viral infections4,5. Decrease in C3 has been documented in patients with lupus and MAS4; however, to our knowledge, hypocomplementemia has not been previously described as associated with MAS in sJIA or AOSD. We describe 2 patients with sJIA, and a third with AOSD, who developed MAS with hypocomplementemia during the acute phase of illness. Patient 1, a 9-year-old boy, presented with high quotidian fever, evanescent rash, and arthritis. He had leukocytosis (24 × 109/l) with elevated markers of inflammation: erythrocyte sedimentation rate (ESR) 52 mm/h and C-reactive protein (CRP) 17.3 mg/dl. Liver enzymes and renal function were normal. Ferritin was 5623 ng/ml. Serologic tests for Epstein-Barr virus, parvovirus, cytomegalovirus, Lyme disease, mycoplasma, and group A streptococcus were negative. Complement levels, obtained by the primary intensive care team, showed slightly increased C3 (166 mg/dl) and normal C4 (32 mg/dl). He was discharged with a diagnosis of new-onset sJIA and prescribed indomethacin. Several days later, he returned to clinic with worsening symptoms and petechial … Address correspondence to Dr. Gorelik; E-mail: mark.gorelik{at}chp.edu
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Hypocomplementemia resulting from increased consumption by immune complexes can be observed in cytokine storm.
“Hypocomplementemia, resulting from increased consumption by immune complexes, can be observed in cytokine storm.49”
From Cytokine storm (2020) · cites this paper as [49]
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(2011) · Current Opinion in Pediatrics · doi:10.1097/mop.0b013e32834dda34
- Authors
- GORELIK, M., TOROK, K. S., KIETZ, D. A., & HIRSCH, R.
- Affiliations
- University of Pittsburgh (US)
- Venue
- The Journal of Rheumatology
- Publisher
- The Journal of Rheumatology
- Published
- 2011-02
- Volume
- 38
- Issue
- 2
- Pages
- 396-397
- ISSN
- 0315-162X (ISSN-L)
- 1499-2752
- PMC
- PMC3672253
- OpenAlex
- W2144950711
- Cited by
- 21
- References
- 12
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 4 · 2023: 2 · 2022: 1 · 2021: 1 · 2020: 2 · 2019: 1 · 2016: 1 · 2015: 3 · 2013: 1 · 2012: 1
- FWCI
- 0.66
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- The Journal of Rheumatology (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Institute of Arthritis and Musculoskeletal and Skin Diseases (T32AR052282, K23AR059722)
- Topics
- Health Sciences › Medicine › Hematology › Autoimmune and Inflammatory Disorders Research
- Health Sciences › Medicine › Surgery › Kawasaki Disease and Coronary Complications
- Life Sciences › Immunology and Microbiology › Immunology › Complement system in diseases
- Keywords
- macrophage activation syndrome, hypocomplementemia, systemic juvenile idiopathic arthritis, adult-onset Still's disease, complement C3, C4, hyperferritinemia, acute phase proteins
- MeSH terms
- Adolescent, Arthritis, Juvenile, Child, Complement C3, Female, Humans, Male, Still's Disease, Adult-Onset, Macrophage Activation Syndrome
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