A rare case of underlying pulmonary sequestration in a patient with recently diagnosed medium and large vessel vasculitis
A 25-year-old female presented with complaints of malaise, weight loss, and diffuse abdominal pain, more inthe umbilical region since 1-2 months. Pain was aggravated with meals and relieved with fasting. On examination, her blood pressure was 160/92 mm Hg in right arm and 158/90 in left arm; lower limb blood pressure was 126/80 in right lower limb and 128/82 in left lower limb. Pulse rate was 90 per minute, regular, good volume with no radiofemoral delay. A renal bruit was heard on the left side. Rest of the systemic examination was within normal limits. Complete blood count, liver function tests, kidney function tests, and urine routine microscopy were within normal limits. Erythrocyte sedimentation rate (ESR) was raised to 35 mm/hour and C-reactive protein (CRP) was more than 5 mg/L. Her chest X-ray and electrocardiogram (ECG) were normal. Her fundus examination was normal. Serology for HIV 1 and 2, hepatitis B surface antigen (HBsAg), anti hepatitis C virus (anti-HCV), venereal disease research laboratory (VDRL) test, anti-nuclear antibodies (ANA), cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) and perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) were negative. Complement levels were normal. Ultrasonography (USG) of the abdomen was normal. Contrast enhanced computed tomography (CT) scan of the thorax and abdomen was done, which revealed thickening of the descending aorta upto bilateral renal artery origin with thickening of the superior mesenteric artery. CT angiography was done, and it revealed wall thickening of the descending aorta with narrowing of lumen. There was also thickening of the superior mesenteric artery with narrowing of lumen. Pulmonary trunk and bilateral pulmonary arteries were also normal. There was evidence of a heterogeneous, hyperdense region in the left medial basal segment deriving its arterial supply separately from the aorta. There was no direct communication with the tracheobronchial tree, and the heterogeneous lung segment had no separate pleural covering. The above-mentioned findings were suggestive of an intralobar pulmonary sequestration []. Bilateral renal artery showed stenosis with involvement of the ostia and proximal segment with post-stenotic dilatation []. The other branches of the abdominal aorta were normal. The thoracic aorta and its branches were also normal in course and caliber. The findings were consistent with large vessel vasculitis.