Case Presentation: This is a 79-year-old female with a history of stage 3 chronic kidney disease and Sjogren’s syndrome who presented with a two month history of worsening malaise and arthralgia, and a two day history of abdominal distention. On admission, laboratory testing revealed an alkaline phosphatase of 132U/L, aspartate aminotransferase (AST) of 195U/L, alanine aminotransferase (ALT) of 204U/L, creatinine kinase (CK) of 2426U/L, lactose dehydrogenase (LDH) of 1437U/L and aldolase of 30.1U/L. On physical exam, her abdomen was distended and tympanic to percussion. Computed tomography of the abdomen revealed cirrhosis and a moderate right-sided ascites. Ultrasound of the abdomen showed varices adjacent to the lesser curvature of the stomach. A diagnostic paracentesis performed revealed a serum-ascites albumin gradient of >1.1g/dL, confirming the diagnosis of portal hypertension. An autoimmune work up was initiated; patient tested positive for antinuclear antibodies and anti-SSA secondary to her Sjogren’s syndrome. A core needle biopsy of the gracilis showed myofiber necrosis and regeneration while the myositis antibody panel came back positive for PL-7. In concordance with her clinical presentation, the diagnosis of antisynthetase syndrome was made. Patient was started on Solumedrol with marked improvement of symptoms.

Discussion: Idiopathic inflammatory myopathies, mainly dermatomyositis (DM) and polymyositis (PM) are characterized by proximal skeletal muscle weakness with other frequent organs manifestation. Approximately 30% of patients with DM and PM exhibit myositis-specific autoantibodies, with antibodies to aminoacyl-transfer (t)RNA synthetases (antisynthetase antibodies), including anti PL-7, being the most commonly described.  In literature review, interstitial lung disease (77%), myositis (75%), and arthritis (56%) are the most common clinical manifestation in anti-PL-7 antisynthetase syndrome.  Hence, the pathophysiology of liver involvement is not well understood. It is commonly accepted that CK, aldolase, AST, ALT and LDH are muscle-derived enzymes in myositis that have levels correlating with disease activity.  Actual liver dysfunction is also speculated to occur via an autoimmune mechanism, however the detailed pathogenesis is unknown.  Fatty liver, hepatic congestion, and non-specific reactive hepatitis were reported to be the main histological findings. Cirrhosis and liver failure are rare, and non-specific abnormalities require no treatment. In our patient, transaminases were not attributed to her history of Sjogren’s syndrome given the acuity of her symptoms and negative anti-mitochondrial antibodies to suggest primary biliary cirrhosis.

Conclusions: Although hepatic manifestations leading to complications are rare, clinicians should have a low threshold of clinical suspicion to accurately diagnose this rare anomaly. We believe we have described an unusual case of antisynthetase syndrome presenting as myositis and inflammatory arthritis with liver involvement.