CO02 | Macrophage activation syndrome in a patient affected by Takayasu’s arteritis and leishmaniasis

F. Albonetti¹, E. Magnani², D. Tortola², M. Bruschi², F.L. Renzullo², M.T. Milite², A. Andreacchio², L. Montaguti², M. Norata³, E.V. Liardo³ | ¹Azienda Ospedaliero-Universitaria di Ferrara, ²Ospedale Bufalini di Cesena, AUSL Romagna, ³Istituto Romagnolo per lo Studio dei Tumori “Dino Amadori”, Meldola (FC), Italy

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.