Successful Reversal of Complete Heart Block in Granulomatosis With Polyangiitis Without Permanent Pacing: A Case Report With Literature Review Publisher Pubmed



Mirzamohamadi A ; Azizan A ; Mahalleh M ; Shahrzad S ; Moosavi NS ; Sadeghi S ; Motamed S ; Ardestani Rostami V ; Alikhani M
Source: Modern Rheumatology Case Reports Published:2025

Abstract

Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a rare antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis primarily affecting the respiratory tract and kidneys. Cardiac involvement in GPA, though uncommon, can lead to life-threatening arrhythmias like complete heart block (CHB). A 33-year-old male with a 12-year history of GPA presented with syncope, palpitations, and reduced consciousness. He had discontinued his medications 3 months earlier. Clinical findings included sensorineural hearing loss, saddle-nose deformity, chronic dacryocystitis, and sinonasal destruction, with no neurological findings. Electrocardiography revealed CHB, prompting temporary pacemaker placement. Echocardiography and coronary angiography excluded structural or ischaemic heart disease. Laboratory parameters and imaging findings supported the diagnosis of GPA relapse. After excluding infectious, metabolic, and drug-related causes of CHB, intravenous methylprednisolone and rituximab were administered. Within 48 hours of initiating glucocorticoids, CHB improved to a first-degree atrioventricular (AV) block, permitting pacemaker removal. After stabilization, cardiac MRI demonstrated mild biventricular dilation with preserved systolic function, absence of myocardial inflammation or fibrosis, and a minimal pericardial effusion. He was discharged on high-dose prednisolone and scheduled for rituximab, achieving complete symptom resolution and stable cardiac conduction at 1-month follow-up. This case and literature review highlight that early appropriate immunosuppression therapy can successfully reverse CHB without requiring permanent pacing. Patients with unexplained arrhythmias and signs of systemic inflammation should be evaluated early for GPA to guide timely treatment. © 2025 Elsevier B.V., All rights reserved.