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Severe cholestatic hepatitis due to large vessel vasculitis: report of two cases
Jason Xu1, Einar S Björnsson2, Vinay Sundaram3
1Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
Insights
Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) can rarely cause liver enzyme abnormalities. Early diagnosis is crucial to prevent severe complications like stroke and blindness.
Area of Science:
- Rheumatology
- Hepatology
- Internal Medicine
Background:
- Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are systemic inflammatory conditions affecting medium and large vessels, primarily in individuals over 50.
- While typically presenting with constitutional symptoms, headache, and jaw claudication (GCA) or musculoskeletal pain (PMR), liver involvement is uncommon.
Observation:
- Liver enzyme abnormalities, specifically elevated aminotransferases, are rare in GCA and PMR.
- In elderly patients on polypharmacy, drug-induced liver injury is a common consideration for abnormal liver function tests.
Findings:
- This report details two cases where severe cholestatic liver enzyme elevations were initially misattributed to drug toxicity.
- The underlying cause was ultimately identified as large vessel vasculitis, specifically GCA and PMR.
Implications:
- Misdiagnosis of liver enzyme abnormalities in GCA/PMR can delay critical treatment, increasing risks of ischemic stroke and blindness.
- Clinicians should consider GCA/PMR in the differential diagnosis of unexplained liver enzyme elevations, especially in older adults.
Abstract:
Giant cell arteritis (GCA) is a vasculitis of medium and large sized vessels that occurs most often in people >50 years of age with associated symptoms of fever, weight loss, headache and jaw claudication. Polymyalgia rheumatica (PMR), which is characterized by aching and stiffness in the shoulders, hip girdle, neck and torso, is intimately associated with GCA, and evidence suggests that GCA and PMR are two phases of the same disease. The occurrence of liver enzyme abnormalities in either of these conditions is rare. Furthermore, as these conditions occur most commonly in the elderly population who may be subject to polypharmacy, patients with elevated aminotransferases due to underlying GCA/PMR may mistakenly have their abnormal liver function tests attributed to drug-induced liver injury. Given the potential complications of these diseases if left untreated, including ischemic stroke and blindness, early recognition and treatment are critical. We report two patients who developed severe cholestatic liver enzyme elevation, which had been initially attributed to drug toxicity, but was ultimately caused by large vessel vasculitis, specifically GCA and PMR.
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