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

Abstract

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.

Keywords

• 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

Abstract

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.

Keywords

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

References

1. 1. Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Sève P. Adult-onset Still's disease. Autoimmunity reviews. 2014;13(7):708-22.
2. 2. Hot A, Toh M-L, Coppéré B, Perard L, Madoux MHG, Mausservey C, et al. Reactive hemophagocytic syndrome in adult-onset Still disease: clinical features and long-term outcome: a case-control study of 8 patients. Medicine. 2010;89(1):37-46.
3. 3. Arlet J-B, Huong DLT, Marinho A, Amoura Z, Wechsler B, Papo T, et al. Reactive haemophagocytic syndrome in adult-onset Still’s disease: a report of six patients and a review of the literature. Annals of the Rheumatic Diseases. 2006;65(12):1596-601.
4. 4. Travis WD, Colby TV, Lombard C, Carpenter HA. A clinicopathologic study of 34 cases of diffuse pulmonary hemorrhage with lung biopsy confirmation. The American journal of surgical pathology. 1990;14(12):1112-25.
5. 5. Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, et al. Preliminary criteria for classification of adult Still's disease. The Journal of rheumatology. 1992;19(3):424-30.
6. 6. Gavand P-E, Serio I, Arnaud L, Costedoat-Chalumeau N, Carvelli J, Dossier A, et al. Clinical spectrum and therapeutic management of systemic lupus erythematosus-associated macrophage activation syndrome: a study of 103 episodes in 89 adult patients. Autoimmunity reviews. 2017;16(7):743-9.
7. 7. Sari I, Birlik M, Binicier O, Akar S, Yilmaz E, Onen F, et al. A case of adult-onset Still's disease complicated with diffuse alveolar hemorrhage. Journal of Korean medical science. 2009;24(1):155-7.
8. 8. Arndt PG, Fantuzzi G, Abraham E. Expression of interleukin-18 in the lung after endotoxemia or hemorrhage-induced acute lung injury. American journal of respiratory cell and molecular biology. 2000;22(6):708-13.

9. 9. Jennings CA, King Jr TE, Tuder R, Cherniack RM, Schwarz M. Diffuse alveolar hemorrhage with underlying isolated, pauciimmune pulmonary capillaritis. American journal of respiratory and critical care medicine. 1997;155(3):1101-9.
10. 10. Fujii T, Akizuki M, Kameda H, Matsumura M, Hirakata M, Yoshida T, et al. Methotrexate treatment in patients with adult onset Still’s disease—retrospective study of 13 Japanese cases. Annals of the rheumatic diseases. 1997;56(2):144-8.
11. 11. Franchini S, Dagna L, Salvo F, Aiello P, Baldissera E, Sabbadini MG. Efficacy of traditional and biologic agents in different clinical phenotypes of adult‐onset Still's disease. Arthritis & Rheumatism. 2010;62(8):2530-5.
12. 12. Kaneko Y, Kameda H, Ikeda K, Ishii T, Murakami K, Takamatsu H, et al. Tocilizumab in patients with adult-onset still’s disease refractory to glucocorticoid treatment: a randomised, double-blind, placebo-controlled phase III trial. Annals of the rheumatic diseases. 2018;77(12):1720-9.