SHM Converge Scientific Abstract ePoster Gallery-Significance of Early Treatment in Granulomatosis With Polyangiitis Vasculitis

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This case report highlights the importance of early treatment in granulomatosis with polyangiitis (GPA), an ANCA-associated vasculitis that can rapidly progress and become fatal if untreated. Although tissue biopsy is important to confirm diagnosis, the authors emphasize that treatment should not be delayed when clinical findings strongly suggest vasculitis.<br /><br />The patient was a 72-year-old man who presented with dyspnea, headache, hearing loss, and right eye pain/redness with photophobia and vision loss. Examination showed palpable purpura on the extremities. Laboratory testing revealed acute kidney injury and elevated inflammatory markers. Chest CT demonstrated bilateral airspace disease. Based on concern for ANCA-associated vasculitis, steroids were started before biopsy confirmation.<br /><br />During hospitalization, the patient deteriorated and developed acute hypoxic respiratory failure requiring intubation. Bronchoscopy showed diffuse alveolar hemorrhage. He also developed a left lower extremity deep vein thrombosis and required an inferior vena cava filter. Infectious workup was negative. He was treated with high-dose steroids, plasma exchange, and rituximab. Due to worsening renal function, he required hemodialysis. He eventually improved and was extubated.<br /><br />Further rheumatologic testing confirmed C-ANCA positivity and PR3 autoantibodies, supporting the diagnosis of GPA. He was discharged with follow-up arranged with rheumatology, nephrology, and ophthalmology.<br /><br />The case underscores that early recognition and prompt initiation of therapy can reduce morbidity and mortality in vasculitis. While biopsy remains valuable, clinicians should not wait for tissue confirmation if the presentation is highly suggestive of ANCA-associated vasculitis.

Asset Subtitle

Jenifer Centeno Gavica

Meta Tag

Author List Jenifer Centeno Gavica, Leslie Raymond

Category Clinical Vignettes

Concept Granulomatosis with Polyangiitis

Concept ANCA-Associated Vasculitis

Concept Renal involvement

Concept Pulmonary Involvement

Concept Diffuse Alveolar Hemorrhage

Distinguished Non-Finalist

Presenter Organization NCH HealthCare System

Presenting Author Jenifer Centeno Gavica

Track Adult

Keywords

granulomatosis with polyangiitis

ANCA-associated vasculitis

early treatment

diffuse alveolar hemorrhage

acute kidney injury

C-ANCA positivity

PR3 autoantibodies

plasma exchange

rituximab

hemodialysis

Granulomatosis with Polyangiitis

ANCA-Associated Vasculitis

Renal involvement

Pulmonary Involvement

Diffuse Alveolar Hemorrhage