Granulomatosis with polyangiitis is a rare systemic inflammatory disorder
characterized by vasculitis of the small arteries, the arterioles and the
capillaries together with necrotizing granulomatous lesions. This case reports
on a young female patient, previously diagnosed with granulomatosis with
polyangiitis, who was admitted to the intensive care unit with seizures and
hemodynamic instability due to a complete atrioventricular heart block. The
event was associated with multiple episodes of sustained ventricular tachycardia
without any structural heart changes or electrolyte disturbances. In the
intensive care unit, the patient was fitted with a provisory pacemaker, followed
by immunosuppression with corticosteroids and immunobiological therapy,
resulting in a total hemodynamic improvement. Severe conduction disorders in
patients presenting granulomatosis with polyangiitis are rare but can contribute
to increased morbidity. Early detection and specific intervention can prevent
unfavorable outcomes, specifically in the intensive care unit.