Granulomatosis with Polyangiitis Presenting with Hepatic Involvement

TL;DR

This paper reports a rare case of Granulomatosis with Polyangiitis (GPA) affecting the liver, highlighting the diagnostic difficulties and potential severity of hepatic involvement.

Contribution

The novelty lies in presenting a case of GPA with hepatic involvement, emphasizing its rarity and diagnostic challenges.

Findings

GPA can present with hepatic involvement, which is rare and challenging to diagnose.

A middle-aged patient with persistent constitutional symptoms and abnormal liver function tests was diagnosed with GPA.

Liver involvement in GPA can lead to severe outcomes if not recognized early.

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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