Atypical Presentation of Macrophage Activation Syndrome Associated with Primary Sjogren\'s Syndrome

During the 1st week of admission, the cause of cholestatic jaundice was evaluated, but no evidence of obstructive features (like stones, malignancy, intrahepatic biliary radical dilatation) was found, except mild hepatomegaly with liver measuring 16.5 cm.Hence, an autoimmune workup was done.Her anti-mitochondrial M2 antibody, liver kidney microsome type 1 antibody, anti-smooth muscle antibody, and anti-soluble liver antigen were negative, ruling out primary biliary cirrhosis, sclerosing cholangitis, and autoimmune liver disorders, but were strongly positive for anti-Sjögren's syndromerelated antigen A and anti-Sjögren's syndrome-related antigen B. The history was reviewed again; she was having dry eyes and blurring of IntroductIonMacrophage activation syndrome (MAS) is associated with primary Sjogren's syndrome and is a rare entity. 1 The term MAS was first coined by Stephen et al.It emerged in parallel with the development of the Histiocyte Society Classification of Histiocyte Disorders in 1987. 2 When secondary hemophagocytic lymphohistiocytosis (HLH) occurs in the context of autoimmunity, it is termed MAS.The incidence of MAS in rheumatological disorders is still unknown due to its rarity. 2acrophage activation syndrome is precipitated by a broad range of bacterial, fungal, and viral triggers.In the pediatric group, the disease is well described in systemic juvenile idiopathic arthritis (sJIA), while in adults, it is prevalent in adult-onset Still's disease (AOSD) (10-15%) and systemic lupus erythematosus (SLE) (0.9-9%).The incidence of secondary HLH (sHLH) at the onset of the rheumatological disease has also been reported (5%) and has a high mortality rate. 2