Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus: A Systematic Review of the Diagnostic Aspects.

Macrophage Activation Syndrome (MAS) is a very severe complication of different rheumatic diseases, including pediatric Systemic Lupus Erythematosus (pSLE). MAS is not considered as a frequent complication of pSLE; however, its occurrence could be under-estimated and the diagnosis can be challenging. In order to address this issue, we performed a systematic review of the available medical literature, aiming to retrieve all those papers providing diagnostic (clinical/laboratory) data on patients with pSLE-related MAS, in individual or aggregated form. The selected case reports and series provided a pool of 46 patients, accounting for 48 episodes of MAS in total. We re-analyzed these patients in light of the diagnostic criteria for MAS validated in systemic Juvenile Idiopathic Arthritis (sJIA) patients and the preliminary diagnostic criteria for MAS in pSLE, respectively. Five clinical studies were also selected and used to support this analysis. This systematic review confirms that MAS diagnosis in pSLE patients is characterized by several diagnostic challenges, which could lead to delayed diagnosis and/or under-estimation of this complication. Specific criteria should be considered to diagnose MAS in different rheumatic diseases; as regards pSLE, the aforementioned preliminary criteria for MAS in pSLE seem to perform better than the sJIA-related MAS criteria, because of a lower ferritin cut-off.

HLA-B*51 allelic and carrier frequency in Kazakhstan: insights into Behçet's disease prevalence in Central Asia.

Behçet's disease (BD) is a systemic vasculitis whose prevalence varies according to the ethnicity. HLA-B*51 is a well-known genetic factor predisposing to BD: populations with higher HLA-B*51 allelic frequencies are also those with a greater disease prevalence, as occurs in the Silk Road countries. However, the BD prevalence is unknown in many countries, including Kazakhstan and, in general, Central Asia, despite the location on or near the Silk Road. Due to the lack of clinical studies from this area, we analyzed the bone marrow donors registry in Kazakhstan, in order to assess the HLA-B*51 allelic and carrier frequencies, which resulted to be 8.14 and 15.28%, respectively. Through a comparison with the same parameters in other countries (by performing a data search in the medical literature and in the Allele Frequency Net Database), we might speculate a BD prevalence in Kazakhstan of 10-15/100.000 at least. This result should prompt a greater clinical consideration of this disease in Central Asia, as well as the publication of clinical data from this geographic area.