Multisystem Inflammatory Syndrome in Adults (MIS-A)
Özet
MIS-A has been defined as a hyperinflammatory state with multiorgan dysfunction in individuals with current or antecedent evidence of an asymptomatic and symptomatic infection. Although the precise pathophysiology of MIS-A remains unclear, it is believed to be caused by a delayed and dysregulated immune response involving both innate and adaptive immune cells. This uncontrolled immune response results in systemic inflammation, endothelial dysfunction, and a procoagulant state. The cardiovascular system was the most frequently involved (81%), followed by the gastrointestinal (73.4%) and mucocutaneous (51.9%) systems. Cardiac involvement in MIS-A may result in a range of clinical scenarios, including arrhythmias, myocarditis, pericarditis, pericardial effusion, and coronary aneurysm. In the initial stages of assessment, clinicians should consider the possibility of a MIS-A diagnosis in patients presenting with elevated inflammatory markers and severe extrapulmonary multiorgan dysfunction. The optimal treatment for MIS-A remains uncertain. However, anti-inflammatory drugs, including intravenous immunoglobulin (IVIG) and pulse glucocorticoids, are frequently employed. In select cases, immune modulators (e.g., tocilizumab or anakinra) may be employed. Patients who are severely ill and experiencing shock or hypotension may require supportive management, including the use of vasoactive medications such as inotropes, intra-aortic balloon pump or extracorporeal membrane oxygenation.
MIS-A, semptomatik veya asemptomatik enfeksiyonlar sonrasında multiorgan disfonksiyonu ile karakterize hiperinflamatuvar durum olarak tanımlanır. Patofizyolojisi net olmasa da, altta yatan nedenin hem doğal hem kazanılmış immün sistem hücrelerinin uzamış ve disregüle immün yanıtının olduğuna inanılmaktadır. Bu kontrolsüz immün yanıt sistemik inflamasyon, endotelyal disfonksiyon ve prokoagülan duruma yol açmaktadır. Kardiyovasküler sistem en sık tutulan sistemdir (81%), ve gastrointestinal sistem tutulumu (73.4%) ve mukokütanöz tutulum (51.9%) bunu takip eder. Kardiyak tutulumda aritmi, myokardit, perikardit, perikardiyal efüzyon ve koroner anevrizmalar görülebilir. İlk değerlendirmede, extrapulmoner organ tutulumu olan ve inflamasyon belirteçleri yüksek seyreden hastalarda klinisyenlerin MIS-A şüphesini akılda tutmaları tanı açısından çok önemlidir. İntravenöz immünglobulin ve yüksek doz pulse steroidler sıklıkla kullanılır. Seçilmiş vakalarda Tosilizumab veya Anakinra gibi immünmodulatör ilaçlar verilebilir. Şok ve hipotansiyon ile gelen kliniği ağır hastalarda destek tedavisi önerilir. Bu hastalarda inotroplar, intraaortik balon pompası (İABP), ekstrakorpereal oksijen (ECMO) ve/veya mekanik ventilasyon gerekebilmektedir.
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