Abstract

Granulomatosis with polyangiitis vasculitis (GPA), or formerly known as Wegener’s Granulomatosis (WG) and sarcoidosis are two distinct granulomatous diseases characterized by multisystem involvement. The sequential development of these two diseases is very rare. We report a patient who initially exhibited the symptoms of sarcoidosis and then limited GPA, predominantly affecting the nose and paranasal sinus, which was histologically proven. Imaging, pathological, and laboratory findings were described. After treatment, controlled clinical symptoms and changes in laboratory findings and imaging were demonstrated.

Highlights

  • Granulomatosis with polyangiitis vasculitis (GPA), formerly known as Wegener’s Granulomatosis (WG), and sarcoidosis are two distinct granulomatous diseases characterized by multisystem involvement

  • Granulomatosis with polyangiitis (GPA) is an autoimmune small vessel vasculitis that is highly associated with anti-neutrophil cytoplasmic antibodies (ANCA)

  • We report an unusual case of a patient presenting with sarcoidosis that remitted with therapy who developed granulomatosis with polyangiitis vasculitis three years later

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Summary

Introduction

Granulomatosis with polyangiitis (GPA) is an autoimmune small vessel vasculitis that is highly associated with anti-neutrophil cytoplasmic antibodies (ANCA). Granulomatosis with polyangiitis vasculitis (GPA), formerly known as Wegener’s Granulomatosis (WG), and sarcoidosis are two distinct granulomatous diseases characterized by multisystem involvement. We report a patient who initially exhibited symptoms of sarcoidosis and limited GPA, predominantly affecting the nose and paranasal sinus, which was histologically proven. Sarcoidosis and GPA are granulomatous diseases with major expressions in common sites, such as the respiratory tract, kidneys, skin, nervous system, eye and orbit, musculoskeletal system, and heart [3].

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