Isolated large vessel pulmonary vasculitis leading to pulmonary artery aneurysm formation: a case report and literature review

Abstract

Pulmonary artery (PA) vasculitis occurs in association with primary vasculitides—in particular, giant cell arteritis, Takayasu’s arteritis, or Behçet’s disease—or secondary vasculitis as a result of infections or malignancy. However, PA vasculitis in isolation and with concomitant aneurysmal dilation is an unusual finding. We present a rare case of PA aneurysm secondary to isolated PA vasculitis in an asymptomatic patient with no features of systemic vasculitis. This case highlights one of the first cases of PA vasculitis managed with surgical resection alone.

Two giants: Giant cell arteritis causing a giant pulmonary artery aneurysm

“… 4 More recent cases demonstrated isolated distal and proximal PAAs associated with vasculitis, with both cases progressing in size, ultimately needing surgical repair. 1 , 5 In a review of 46 cases of PAA secondary to autoimmune disease, the underlying cause was most commonly attributed to Behçet syndrome (n = 31) and only once to GCA. 2 Our patient presented in a similar manner to the previously described cases.…”

Pulmonary vasculitis mimicking chronic thromboembolic disease

“…In the first of their cases, despite surgery and medical treatment for the vasculitis, some of the pulmonary arteries remained occluded and narrowed. Isolated pulmonary vasculitis has also been described as rare causes of pulmonary hypertension and pulmonary artery aneurysms 7 8…”