C1 COMPLETE HEART BLOCK AS A RARE MANIFESTATION OF GRANULOMATOSIS WITH POLYANGITIS: A CASE REPORT

Abstract

Abstract A 42–year–old male with prior smoking habit presented at our emergency department after atraumatic syncope preceded by severe pain in the left lower limb. One month prior to presentation, due to persistence of nonproductive cough he underwent a chest computed tomography (CT), which showed 2 pulmonary nodules with hypercaptation at PET performed later (figure 1). The 12–lead electrocardiogram showed sinus rhythm with complete atrioventricular block and ventricular escape rhythm at 40 bpm (figura 1). Ecofast revealed a mildly impaired left ventricular systolic function (left ventricular ejection fraction [LVEF] 45%) with basal septal hypokinesis. Laboratory assessment showed leukocytosis (marked neutrophilia and eosinophilia), thrombocytosis anemia, elevated C–reactive protein (193 mg/dl), troponin I (18483 ng/L), NTproBNP (14627 ng/l) and creatinine (2.27 mg/dL) levels. The patient was transported to the catheterization laboratory for temporary pacemaker insertion and coronary angiography, which depicted distal occlusion of the first septal branch (Figure 2A), the first diagonal branch (Figure 2B) and the obtuse marginal branch (Figure 2C). After the procedure due to persistent ischemia of the left lower limb, several unsuccessful procedures to restore limb vascularization by means of locoregional thrombolysis and percutaneous angioplasty were attempted, but a leg amputation above the knee was eventually performed after few days. Neoplastic markers, blood cultures and urine cultures resulted negative, urine sediment examination showed cylinders, leucocytes and microhematuria. A brain/facial bones CT scan was performed, showing left maxillary sinusitis (figure 1). A rheumatologic screening was also performed, showing high titer of cANCA(125 UI/ml) hinting at a systemic vasculitis. The histopathological results of the amputated limb depicted marked necrotizing vasculitis with extensive involvement of small–medium caliber and larger caliber arterial vessels (figure 2). Based on these clinical and laboratory findings, a diagnosis of granulomatosis with polyangiitis (GPA) was made. The patients was treated with ev methylprednisolone and rituximab. After the induction therapy, chest CT scan showed a marked reduction of the size of the pulmonary nodule (Figure 3); renal function progressively improved and physiological atrioventricular conduction being restored