CO02 | Macrophage activation syndrome in a patient affected by Takayasu’s arteritis and leishmaniasis
Year: 2025
Type: article
Study design: Case Report
Abstract: Introduction: Macrophage activation syndrome (MAS) is a rare life-threatening syndrome which may complicate rheumatologic diseases. It is a type of secondary hemophagocytic lymphohistiocytosis (HLH), characterized by hyperactivation of the immune system with non-malignant histiocytes multisystemic infiltration and uncontrolled hemophagocytosis. Secondary HLH can also be caused by infectious agents. We present a rare case of MAS in Takayasu’s arteritis and leishmaniasis.Case report: A 67 years-old woman affected by Takayasu’s arteritis was in remission phase with low dose of steroid and metotrexate. She developed asthenia, joint pains, fever, hyperferritinemia, hepatitis and pancytopenia. MAS was suspected;all major possible infectious trigger agents and ongoing neoplastic diseases were excluded. Hemophagocytosis was showed in bone marrow sample. The suspect of MAS related to a relapsed vasculitis was treated with high-dose steroids and IL-1R, but only with etoposide the arrest of systemic deterioration was achieved. Later Leishmania infection was detected. We associated amphotericin B to steroid therapy and best supportive care. Conclusions: Leishmaniasis is increasing in our country and could lead to MAS, especially in immunodepressed patients as rheumatologic ones. In our case coexistence of Takayasu’s arteritis and leishmaniasis infection provoked this fearsome complication. The case underlines the importance of differential diagnosis of sepsis like conditions; also, it’s a first example of a Takayasu-MAS correlation, which has not been described in the literature.
Source: Italian Journal of Medicine
Authors: Vo Van Kha, Tran Thi Huong Ly, Pham Thi Thanh Hoa, Vo Huynh Nhu, Damas Hendriansyah, Nusratuddin Abdullah, Rudy B. Leonardy, Mardiah Tahir, Samrichard Rambulangi, Firdaus Hamid, Filomena Pietrantonio, Vera Kopsaj, Francesco Rosiello, Aurora Tomassoni, Luca Moriconi, Donatella Padua, Kiki Rizki Amelia, Efendi Lukas, Nurbani Bangsawan, Isharyah Sunarno, Retno Budiarti Farid, Sriwijaya Qadar, Rabab H. Elshaikh, Ashok Kumar Sah, Abdelrahman Mohamed Abukanna, Abdulrahman Algarni, Ammar Abdelmola, Hussam Ali Osman, Tagwa Yousif Elsayed Yousif, Elryah I. Ali, Hiba Ali, Ayman Hussein Alfeel, Thanh Long Nguyen, Victor Storm, Lam Toi Phung, Cong Thien Le, Thi Thu Ha Le, Thi Hoa Nguyen, Son Tung Vu, Thien Thang Tran, Minh Hanh Vu, Van Tuan Nguyen, Francesco Ventrella, Anna Belfiore, Antonio Greco, Franco Mastroianni, Salvatore Lenti, Domenico Ruggiero, Michele Cannone, Vincenzo Ostilio Palmieri, Massimo Errico, Giuseppe Mancia, Guido Grassi, Sverre E. Kjeldsen
Language: English
Subfield: Hematology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4413795385