Granulomatosis with polyangiitis (GPA) is characterised by systemic necrotising vasculitis of small arteries and veins with multitude of organ involvement, with the most common being the upper and lower respiratory tract and renal system. Gastrointestinal involvement is a rare late manifestation with a high mortality rate and usually results in intestinal perforation. Our patient presented with gastrointestinal bleeding secondary to jejunal artery vasculitis. Gastrointestinal bleeding as initial presentation of GPA is very rarely documented. CT mesenteric angiogram is helpful for the localisation of bleed in these cases. In case of refractory bleeding, surgical excision is required.

Patients with history of mitochondrial disorders are at increased risk of having conduction disorders and cardiomyopathy and should have low threshold for pacemaker and implantable cardioverter defibrillator placement. Kearns Sayre syndrome is the result of deletions in mitochondrial DNA which causes bilateral pigmentary retinopathy and conduction abnormalities. Judicious use of implantable cardioverter defibrillator in this subset population with cardiomyopathy or prolonged QT interval is required in addition to pacing to prevent risk of sudden cardiac death. A subset of these patients might continue to experience life threatening arrhythmias including torsade de pointes and ventricular fibrillation despite a functional pacemaker.

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