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Macrophage activation syndrome as a rare complication of primary Sjögren’s syndrome and systemic lupus erythematosus: two case reports

Background. Macrophage activation syndrome (MAS) or secondary hemophagocytic lymphohistiocytosis is a life-threatening hyperinflammatory state that occurs as a complication of autoimmune diseases and requires prompt diagnosis and treatment. It shares similar clinical presentation with primary (p)HLH. Unlike pHLH which is hereditary autoinflammatory disorder, pathophysiology of MAS is unclear, although recent studies have found that MAS and pHLH share significant genetic overlap. Case 1. A 65-year-old male without history of autoimmune disease was admitted due to NSTEMI. During hospitalization patient developed high fever, pancytopenia, liver damage, splenomegaly, lymphadenopathy, rash, irritability, confusion, high ferritin (FER) and triglycerides (TG). After diagnostic workup a diagnosis of MAS secondary to Sjogren’s syndrome was made. HSscore was 204 showing an 88–93% probability of HLH. Treatment with methylprednisolone (MP) 2 mg/kg/day and hydroxychloroquine (HCQ) 200 mg/day have led to a rapid resolution of symptoms and normalization of laboratory findings. Case 2. A 28-year-old SLE patient was hospitalized due to high fever, pancytopenia, liver damage, rash, aphthae, hepatosplenomegaly, lymphadenopathy, high FER, and TG. The diagnosis of MAS with visual loss due to retinal vasculitis (RV) was made. HS score was 219 showing an 93–96% likelihood of HLH. Treatment with MP 2 mg/kg/day, HCQ 400 mg/day and mycophenolate mofetil 2000 mg/day improved his clinical condition and laboratory findings, however, due to further active RV without visual recovery and in agreement with ophthalmologist, treatment with rituximab 1 g on day 0. and 14. and local anti VEGF bevacizumab was started with an excellent response: complete recovery of vision acuity and clinical and laboratory immunological remission of SLE. Conclusion. In patients with fever refractory to antibiotics and high FER, we should suspect a hyperinflammatory state and perform HS score. MAS diagnosis is constellation of clinical features and clinical judgment. Prompt intervention with corticosteroids (GC) to suprress cytokine storm followed by identification and appropriate treatment of the underlying autoimmune disease is needed. Early immunosuppression primary with GC in MAS can be lifesaving and more aggressive treatment as in pHLH in our opinion is usually unnecessarily if MAS is recognized in time.

macrophage activation syndrome, Sjögren’s syndrome, SLE, ferritin

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