A Rare Case of Granulomatosis With Polyangiitis Complicated by Splenic Rupture

TL;DR

A rare case of a spleen rupture caused by a rare autoimmune disease called Granulomatosis with Polyangiitis is presented, highlighting the need for prompt diagnosis.

Contribution

This paper reports a rare clinical presentation of GPA with splenic rupture as the initial symptom.

Findings

Splenic rupture can be a life-threatening complication of GPA.

CT scan, ANCA test, and biopsy confirmed AAV-related splenic rupture in the patient.

Spontaneous splenic rupture without a clear cause should prompt evaluation for connective-tissue diseases.

Abstract

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), is a condition marked by necrotizing vasculitis of the small-medium vessels that results in necrotizing granulomatous inflammation. Splenic involvement in GPA is a potentially life-threatening consequence of connective tissue disease and is rarely described as the main presenting feature. We present a case of a patient with perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) who experienced spontaneous splenic rupture. A CT scan of the abdomen, an ANCA test, and a splenic biopsy were employed to identify ANCA-associated vasculitis (AAV) splenic rupture. Our patient's splenic rupture could be attributed to GPA. Nonetheless, since it may alter patient follow-up and therapy, a patient with spontaneous splenic rupture without an obvious explanation should be promptly evaluated for connective-tissue disease. This report highlights the intricacy and unpredictability of the clinical symptoms linked to AAV, as well as the possibility of misinterpreting them.

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