Division of Nephrology, China Medical University Hsinchu Hospital, Hsinchu, Taiwan.
Division of Rheumatology, Allergy and Immunology, Chang Gung Memorial Hospital, Linkou branch, Taoyuan, Taiwan.
Medicine (Baltimore). 2022 Feb 4;101(5):e28612. doi: 10.1097/MD.0000000000028612.
Previous treatment for macrophage activation syndrome (MAS) includes high-dose intravenous methylprednisolone along with intravenous immunoglobulin G. If MAS worsened, second-line therapy consisted of anakinra; if the disease remained refractory, third-line therapy with etoposide was considered. In addition, cyclosporine A plays a role in early MAS and in preventing recurrence. Some studies have reported the use of cytokine-targeting agents other than anakinra, such as canakinumab, tocilizumab, abatacept, and tofacitinib.
The patient with systemic lupus erythematosus (SLE) had an uncommon combination of intermittent fever, hyperferritinemia, hypertriglyceridemia, jaundice, and significantly abnormal liver function test results. The patient reported a history of daily fever of 38 to 39°C, painful oral ulcer, anorexia, abdominal bloating, diarrhea, and malar rash progression for 2 weeks, and jaundice, tea-colored urine, and clay-colored stool for 1 week preceding hospital admission.
SLE flareups in the patient were initially suspected. However, the final diagnosis was acute respiratory distress syndrome (ARDS) associated with MAS.
The treatment included disease-modifying antirheumatic drugs (DMARDs), such as azathioprine, and titrated steroid doses of methylprednisolone (40 mg q8 h) and dexamethasone (15 mg q8 h), after the patient had ARDS and was intubated.Dose-adjusted monotherapy with dexamethasone was found to be effective; this may be attributed to some DMARDs being unsuitable for cytokine storms, that is, some DMARDs may cause complications in cytokine storms.
After dexamethasone 15 mg q8 h treatment, the patient's fever subsided within 2 days, and liver function became normal within 3 weeks. The patient regularly attended scheduled outpatient follow-up visits after discharge. After 2 years, the patient reported no symptoms or signs of SLE with 2 mg/d oral dexamethasone.
Early diagnosis of MAS and dexamethasone treatment for MAS with ARDS appear to be crucial for these patients.
巨噬细胞活化综合征（MAS）的既往治疗包括大剂量静脉注射甲基强的松龙和静脉注射免疫球蛋白 G。如果 MAS 恶化，二线治疗包括阿那白滞素；如果疾病仍然难治，则考虑三线治疗依托泊苷。此外，环孢素 A 在早期 MAS 和预防复发中发挥作用。一些研究报告了使用阿那白滞素以外的细胞因子靶向药物，如康纳单抗、托珠单抗、阿巴西普和托法替尼。
系统性红斑狼疮（SLE）患者出现间歇性发热、高铁蛋白血症、高三酰甘油血症、黄疸和肝功能检查结果显著异常等不常见的组合症状。该患者报告称，其有 2 周每日发热 38 至 39°C、疼痛性口腔溃疡、厌食、腹胀、腹泻和蝶形皮疹进展，以及入院前 1 周出现黄疸、茶色尿和泥色大便。
最初怀疑患者出现 SLE 发作。然而，最终诊断为伴有 MAS 的急性呼吸窘迫综合征（ARDS）。
在患者发生 ARDS 并插管后，治疗包括疾病修饰抗风湿药物（DMARDs），如硫唑嘌呤和甲泼尼龙（40mg q8h）和地塞米松（15mg q8h）的剂量调整类固醇治疗。发现地塞米松剂量调整单药治疗有效；这可能归因于一些 DMARDs 不适合细胞因子风暴，即一些 DMARDs 可能在细胞因子风暴中引起并发症。
在接受地塞米松 15mg q8h 治疗后，患者的发热在 2 天内消退，肝功能在 3 周内恢复正常。患者出院后定期定期进行门诊随访。2 年后，患者报告在 2mg/d 口服地塞米松治疗下无 SLE 症状或体征。
对于这些患者，早期诊断 MAS 和 ARDS 时使用地塞米松治疗 MAS 似乎至关重要。
