Makrofaj Aktivasyon Sendromu Ve Difüz Alveolar Hemoraji ile Komplike Olan Erişkin Still Hastalığı Olgusu

Öz

Erişkin Still Hastalığı (ESH) yüksek ateş, deri döküntüsü, artrit, ferritin yüksekliği ve organ tutulumuyla karakterize etyolojisi tam olarak bilinmeyen, nadir görülen inflamatuar bir hastalıktır. ESH’de nadirde olsa, potansiyel olarak ölümcül bir komplikasyon olarak Makrofaj Aktivasyon Sendromu (MAS) görülebilir. Biz burada kronik artiküler form ESH tanısıyla takip edilirken genel durum bozukluğu, ferritin artışı ve sitopeni gelişmesi üzerine MAS tanısı alan ve kısa bir süre sonra da Diffüz alveoler hemoraji (DAH) gelişen bir olguyu sunduk. Kortikosteroid ve tosilizumab tedavisine dramatik cevap veren bu olgu sunumu ile ESH sonrası gelişen nadir komplikasyonlara dikkat çekmeyi amaçladık.

Anahtar Kelimeler

• Erişkin Still Hastalığı
• Diffüz alveoler hemoraji
• Makrofaj Aktivasyon Sendromu
• tosilizumab

A Case of Adult-onset Still’s Disease Complicated with Macrophage Activation Syndrome and Diffuse Alveolar Hemorrhage

Öz

Adult-onset Still’s Disease (AOSD) is a rare inflammatory disease of unknown etiology characterized by high fever, skin rash, arthritis, elevated ferritin and organ involvement. Macrophage Activation Syndrome (MAS) can be seen as a rare, potentially fatal complication in AOSD. Here, we presented a case who was diagnosed with MAS due to general condition disorder, increased ferritin and cytopenia while being followed up with the diagnosis of chronic articular form ESH, and then developed diffuse alveolar hemorrhage (DAH) after a short time. We aimed to draw attention to rare complications after AOSD with this case report that showed a dramatic response to corticosteroid and tocilizumab treatment.

Anahtar Kelimeler

• Adult-onset Still’s Disease
• Diffuse alveolar hemorrhage
• Macrophage Activation Syndrome
• tocilizumab

Kaynakça

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