Granulomatosis with Polyangiitis Presenting with Hepatic Involvement

肉芽肿性多血管炎伴肝脏受累

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Abstract

Granulomatosis with polyangiitis (GPA) is an uncommon autoimmune anti-neutrophilic cytoplasmic antibody-associated vasculitis. Although it was first recognized nearly a century ago, its etiology is still under investigation. Numerous previous studies have widely suggested that neutrophils play a central role in its pathogenesis. This granulomatous necrotizing inflammation has historically been recognized to commonly affect the ear, nose, throat, lungs, and kidneys; however, other organ involvement, including hepatic involvement, has also been occasionally observed. Some older retrospective case studies have reported fatal outcomes associated with liver involvement due to GPA, highlighting the diagnostic challenges it presents. Hence, we present a case report describing a middle-aged gentleman who presented with persistent constitutional symptoms and continuously deranged liver function tests (LFTs) and was ultimately diagnosed with GPA with hepatic involvement.

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