Macrophage activation syndrome associated with systemic juvenile idiopathic arthritis

Silva, Clóvis A.; Silva, Carlos Henrique M.; Robazzi, Teresa Cristina Martins Vicente; Lotito, Ana Paola N.; Mendroni, Alfredo; Jacob, Cristina Miuki Abe; Kiss, Mária

doi:10.2223/jped.1267

Cited by 11 publications

(3 citation statements)

References 21 publications

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“…8 Acquired MAS is typically triggered by infections, neoplasms, immunodeficiencies, and rheumatic diseases. 9 The clinical presentation of MAS is generally acute and can be dramatic. Patients can become acutely ill with unremitting high fever, hepatosplenomegaly, lymphadenopathy, profound pancytopenia, and elevated serum liver enzymes.…”

Section: Discussionmentioning

confidence: 99%

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An 18-Year-Old with Recurrent Fever, Night Sweats, and Lymphadenopathy

John¹,

Bingi²,

Brant³

et al. 2015

Pediatr Ann

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Section: Discussionmentioning

confidence: 99%

“…9 The described treatment strategy for MAS historically has consisted of high doses of corticosteroids with transition to cyclosporine A in severe or steroidresistant cases. Conflicting results have been published regarding the use of highdose intravenous immunoglobulins, cyclophosphamide, plasma exchange, and etoposide.…”

mentioning

confidence: 99%

An 18-Year-Old with Recurrent Fever, Night Sweats, and Lymphadenopathy

John¹,

Bingi²,

Brant³

et al. 2015

Pediatr Ann

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“…However, patients with rheumatic diseases present higher risk for severe varicella 19 and herpes zoster, 20 in addition to additional risks related to chronic use of anti-inflammatory 21,22 and possible induction of macrophage activation syndrome by this virus. 23 Despite concerns about the use of live agent vaccines, recent studies demonstrate the safety of its use in immunosuppressed patients. In a study conducted in Brazil, 25 pediatric patients with rheumatic diseases were selected and vaccinated against chickenpox.…”

Section: Discussionmentioning

confidence: 99%

Prática de vacinação em crianças com doenças reumáticas

Silva¹,

Terreri²,

Aikawa³

et al. 2010

Rev. Bras. Reumatol.

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Introduction/Objectives: Evaluate clinical practice through assessment of vaccination card and recommendation of specific vaccines in pediatric patients with rheumatic diseases in use of different drugs and reveal the possible association between vaccination frequency and time of the clinical practice of pediatric rheumatologists in the state of São Paulo. Material and Methods: A questionnaire was sent to pediatric rheumatologists of the Departamento de Reumatologia da Sociedade de Pediatria de São Paulo. This instrument included questions about practice time on Pediatric Rheumatology, vaccination of patients with juvenile systemic lupus erythematosus (JSLE), juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM), and immunization according to the treatments used. Results: Vaccination card was seen by 100% of the professionals at the first visit and by 36% annually. Vaccines of live agents were not recommended for patients with JSLE, JIA, and JDM in 44%, 64%, and 48%, respectively. The professionals were divided into two groups: Group A (≤ 15 years of practice, n = 12) and B (≥ 16 years, n = 13). No statistical difference was observed in the use of live agent vaccine and vaccines with inactivated agents or protein components in the two treatment groups (P > 0.05). Moreover, the groups had similar opinion regarding severity of immunosuppression in patients with JSLE, JIA, and JDM (with or without activity) and treatment used (P > 0.05). Conclusions: The frequency of immunization by pediatric rheumatologists in São Paulo is low, especially after the first visit, and not influenced by time of professional practice.

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Macrophage activation syndrome in children with systemic onset juvenile idiopathic arthritis: clinical experience from northwest India

et al. 2011

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The objective of this study is to describe the clinical and laboratory features of macrophage activation syndrome (MAS) in systemic onset juvenile idiopathic arthritis (SOJIA) at a tertiary care center in northwest India. Review of medical records of all children with SOJIA admitted during the period January 1995-December 2008 in Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, was done. Six patients (5 boys and 1 girl) with SOJIA and MAS were identified. Mean age at time of disease onset was 6.5 years. MAS was the presenting manifestation of SOJIA in 4 patients. Clinical manifestations included fever (6/6), clinical shock (6/6), encephalopathy (5/6), generalized lymphadenopathy (4/6), hepatosplenomegaly (3/6), jaundice and abdominal tenderness (3/6), cardiac involvement (3/6), and meningeal irritation (2/6). Laboratory findings at onset of MAS included decreasing total leukocyte and platelet counts, coagulopathy, elevated transaminases, hyponatremia, and lipid abnormalities. Hemophagocytosis was demonstrable in the bone marrow in 4 patients and in the lymph node in 1. For treatment, we used intravenous methylprednisolone (4/6), oral prednisolone (2/6), and intravenous immunoglobulin (2/6). Outcome was favorable in all patients except one who died of rapidly progressive disease. This paper describes the experience of JIA-related macrophage activation syndrome in a tertiary Indian center. We have shown that MAS can be the early presenting manifestation of evolving SOJIA. Early diagnosis and aggressive management can have a significant impact on the mortality associated with this syndrome. We stress on the role of glucocorticoids in the management of this condition and believe that glucocorticoids have a far more important role in the management of this condition than what has been previously reported.

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Macrophage activation syndrome associated with systemic juvenile idiopathic arthritis

Cited by 11 publications

References 21 publications

An 18-Year-Old with Recurrent Fever, Night Sweats, and Lymphadenopathy

An 18-Year-Old with Recurrent Fever, Night Sweats, and Lymphadenopathy

Prática de vacinação em crianças com doenças reumáticas

Macrophage activation syndrome in children with systemic onset juvenile idiopathic arthritis: clinical experience from northwest India

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