Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is the most common cause of chronic arthritis in children. It is a group of heterogeneous disorders that have chronic arthritis as a common feature. It has a worldwide distribution and many studies have shown that subtype frequencies in JIA seem to be showing geographical distribution. The aim of this study was to define subtype frequencies, demographic features, and the rates of macrophage activation syndrome, uveitis and remission in Turkish JIA patients. The files of all JIA patients (378 cases) that were being followed in Pediatric Rheumatology Clinic of our institution, between May 2010 and February 2016 were reviewed. Two hundred and sixty-five patients were included into the study. Gender, JIA subtype, age at diagnosis, age at the initial symptoms, JIA medications, uveitis presence, JIA status at the time of enrollment were recorded from the files. There were 87 enthesitis related arthritis, 87 oligoarthritis (81 persistent, 6 extended), 36 rheumatoid factor (RF) negative polyarthritis, 35 systemic arthritis, 10 RF-positive polyarthritis, 5 psoriatic arthritis and 5 undifferentiated arthritis cases. Mean age at diagnosis was 9.9 ± 4.9 years and male/female ratio was 1.05. Uveitis was found in 4.5% of the cases. Biologics were used in 26% of the patients. At the time of enrollment, 69% of the patients were under remission while 31% of them were active. Systemic arthritis and persistent oligoarthritis cases were the groups that most commonly achieved remission, while patients with polyarticular involvement, namely RF positive polyarthritis, RF negative polyarthritis and extended oligoarthritis patients were the groups with high number of active patients. In conclusion, JIA is a heterogeneous group of disorder, and differences in subtype frequencies from country to country make it even more heterogeneous disease. Patients with polyarticular involvement may need early and aggressive treatment to control the disease activity.

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“…13 In 34% of systemic arthritis cases we have observed MAS. MAS frequency in systemic arthritis patients is reported to be between 7%-12.6%.…”

Section: Discussionmentioning

confidence: 70%

Subtype frequencies, demographic features, and remission rates in juvenile idiopathic arthritis - 265 cases from a Turkish center

Çakan¹,

Aktay-Ayaz²,

Keskindemirci³

et al. 2017

Turk J Pediatr

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“…In general, however, HLH is under-recognised and the true prevalence, particularly of secondary HLH, is likely to be higher 10. In the context of sJIA, MAS occurs in 7–13% of patients 11. Evidence of subclinical MAS based on bone marrow findings was present in 53% of patients with sJIA at diagnosis 12…”

Section: Epidemiologymentioning

confidence: 99%

Diagnosing haemophagocytic syndrome

2016

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Haemophagocytic syndrome, or haemophagocytic lymphohistiocytosis (HLH), is a hyperinflammatory disorder characterised by uncontrolled activation of the immune system. It can result from mutations in multiple genes involved in cytotoxicity or occur secondary to a range of infections, malignancies or autoimmune rheumatic diseases. In the latter case, it is also known as macrophage activation syndrome (MAS). Characteristic features are persistent fever, hepatosplenomegaly, petechial/purpuric rash, progressive cytopenias, coagulopathy, transaminitis, raised C reactive protein, falling erythrocyte sedimentation rate, hypertriglyceridaemia, hypofibrinogenaemia and extreme hyperferritinaemia often associated with multi-organ impairment. Distinguishing HLH from systemic sepsis can present a major challenge. Criteria for diagnosis and classification of HLH and MAS are available and a serum ferritin >10 000 µg/L is strongly supportive of HLH. Without early recognition and appropriate treatment, HLH is almost universally fatal. However, with prompt referral and advancements in treatment over the past two decades, outcomes have greatly improved.

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“…Se recomienda IgIV conjuntamente con inmunosupresores e inmunomoduladores en los siguientes casos: 119 • Síndrome de activación de macrófagos en artritis idiopática juvenil.…”

Section: Síndrome De Activación De Macrófagosunclassified

Guía colombiana de práctica clínica para el uso de inmonuglobulinas en el tratamiento de reemplazo e inmunomodulación

Olivares

Olmos²,

Álvarez

et al. 2017

RAM

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Immunoglobulins are heterodimeric proteins composed of 2 heavy chains and 2 light chains. Human immunoglobulin G (IgG) is a plasma derivative and contains more than 95% of IgG. The composition of IgG subclasses is similar to that of normal human plasma. Immunoglobulin therapy was first introduced more than 50 years ago, and its use has been described in numerous diseases. In Colombia, the importance of this immunomodulatory resource prompted the need for clinical practice guidelines to be available for its use. For this reason, a multidisciplinary group of experts was brought together and distributed in working groups, by specialties, in order to develop an initial manuscript. Systematic literature searches were undertaken; identified evidences were evaluated and classified to support a preliminary draft that was discussed, analyzed and amended. Recommendations were issued on the use of intravenous immunoglobulin in pathologies that include primary and secondary immunodeficiencies, autoimmune diseases, neurological disorders, infections, transplants and miscellaneous conditions; grades were assigned to each one of them according to the GRADE system. The final result translated into recommendations that are put forth with the purpose to inform, guide and support on optimal use of this immunomodulatory resource.

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Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis

Cited by 85 publications

References 55 publications

Subtype frequencies, demographic features, and remission rates in juvenile idiopathic arthritis - 265 cases from a Turkish center

Subtype frequencies, demographic features, and remission rates in juvenile idiopathic arthritis - 265 cases from a Turkish center

Diagnosing haemophagocytic syndrome

Guía colombiana de práctica clínica para el uso de inmonuglobulinas en el tratamiento de reemplazo e inmunomodulación

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