SHM Converge Scientific Abstract ePoster Gallery-An Unusual Case of Vanishing Bile Duct Syndrome and Optic Neuritis in a Patient With an Acute Lupus Flare

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This case describes a 23-year-old man with systemic lupus erythematosus (SLE) who presented to the emergency department with months of progressive blurred vision and blind spots. He had recently been diagnosed with optic nerve edema as an outpatient. On admission, lumbar puncture attempts were unsuccessful in obtaining cerebrospinal fluid, and he was started on IV fluids and high-dose IV methylprednisolone, then admitted in stable condition.<br /><br />His history included multiple prior medical problems, ongoing immunosuppressive therapy, cigarette smoking, and marijuana/synthetic cannabinoid use. During hospitalization, prednisone was continued to reduce inflammation affecting both the optic nerves and liver. By hospital day eight, his symptoms began improving.<br /><br />Workup revealed unusual multisystem involvement. Liver biopsy on day three showed bile duct loss, reticuloendothelial iron overload, and periportal fibrosis with bridging fibrosis (stage 3), supporting a diagnosis of vanishing bile duct syndrome (VBDS). Although VBDS is usually linked to drug injury, autoimmune disease, malignancy, or other systemic disorders, it is rarely reported in SLE. The authors note that synthetic cannabinoid use may have contributed to liver injury, though the overall picture favored lupus-related inflammation. Notably, transaminases were not significantly elevated, making lupus hepatitis less likely.<br /><br />His ocular findings were also atypical for SLE. While common eye manifestations include conjunctivitis and retinal vasculopathy, his vision loss and MRI findings were most consistent with optic neuritis. Both the liver and eye disease responded to corticosteroid therapy, supporting the diagnosis of an acute lupus flare affecting multiple organs.<br /><br />The report emphasizes that clinically significant liver involvement in SLE is uncommon and VBDS may be underrecognized. It highlights the need to consider immune-mediated liver injury and optic neuritis in severe lupus flares.

Asset Subtitle

Luke J. Rasmussen

Meta Tag

Author List Luke J. Rasmussen, Pinky Jha, Thomas A. Kotsonis

Category Clinical Vignettes

Concept Systemic Lupus Erythematosus

Concept Vanishing Bile Duct Syndrome

Concept Optic Neuritis

Concept Severe Flare

Concept Cholestatic Liver Injury

Distinguished Non-Finalist

Presenter Organization Medical College of Wisconsin

Presenting Author Luke J. Rasmussen

Track Adult

Keywords

systemic lupus erythematosus

SLE

vanishing bile duct syndrome

VBDS

optic neuritis

optic nerve edema

liver biopsy

corticosteroid therapy

lupus flare

multisystem involvement

Systemic Lupus Erythematosus

Vanishing Bile Duct Syndrome

Optic Neuritis

Severe Flare

Cholestatic Liver Injury