A Rare Presentation of Granulomatosis With Polyangiitis with Multiple Cardiac Valvular Insufficiencies

Abstract

Granulomatosis with polyangiitis (GPA), earlier known as Wegener's granulomatosis, is an autoimmune inflammatory disorder that causes necrotizing vasculitis of small- and medium-sized blood vessels. It primarily affects the upper respiratory tract, lungs, and kidneys. Most of the cardiac involvement tends to be subclinical and is often not clinically apparent with involvement of the conduction pathway; myocarditis, pericarditis, or coronary artery involvement are associated with increased morbidity and mortality. These present with the symptoms of shortness of breath, cough, bilateral pedal edema, orthopnea, syncope, and features of heart failure such as elevated jugular venous pressure. We report a rare case of heart involvement with profound valvular deformity involving all four cardiac valves along with renal impairment in a 76-year-old female with recently diagnosed granulomatosis with polyangiitis.