362 COMPLETE HEART BLOCK AS A RARE MANIFESTATION OF GRANULOMATOSIS WITH POLYANGIITIS: A CASE REPORT
Authors: Manai Rossella , Cingolani Marco , Raineri Claudia , Bocchino Pier Paolo , Frea Simone , De Ferrari Gaetano Maria 
Published: December 2022

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. He had no past relevant medical history until one month prior to presentation, when the onset and persistence of nonproductive cough deemed the performance of a chest computed tomography (CT), which showed a 28 mm pulmonary nodule. A positron emission tomography-CT scan performed thereafter showed hypercaptation of the apical pulmonary nodule (SUV 6.87), and hypercaptation of the left nasal cavities. He was not on daily medications. On presentation the patient complained of dyspnoea at rest with no chest pain. On physical examination the left foot was cold and numb with no pedidial pulses. Laboratory assessment showed leukocytosis, thrombocytosis, anemia and elevated C-reactive protein, troponin I, NTproBNP and creatinine levels. The 12-lead electrocardiogram showed sinus rhythm with complete atrioventricular block and ventricular escape rhythm at 40 bpm; the transthoracic echocardiography revealed a mildly impaired left ventricular systolic function with basal septal hypokinesis; a chest-CT scan confirmed the stability of the aforementioned pulmonary lesions. The patient was transported to the catheterization laboratory for temporary pacemaker insertion and coronary angiography, which depicted distal occlusion of the first septal branch, the first diagonal branch and the obtuse marginal branch; no percutaneous coronary intervention was performed. The patient was then transferred to our Coronary Intensive Care Unit: due to persistent ischemia of the left lower limb, and several unsuccessful procedures to restore limb vascularization, a leg amputation above the knee was performed on day 2 of hospitalization. Meanwhile, the patient also developed fever, epididymitis, digital infarcts with bilateral hand purpura, upper and lower extremities paresthesia, worsening renal failure, persistent anemia and leukocytosis with eosinophilia. Neoplastic markers, blood cultures and urine cultures resulted negative; urine sediment examination showed cylinders, leucocytes and severe dysmorphic microhematuria suggestive of glomerulonephritis; bronchoalveolar lavage showed inflammatory cells. 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 depicting marked necrotizing vasculitis with extensive involvement of small-medium caliber and larger caliber arterial vessels. Based on these clinical and laboratory findings, a diagnosis of granulomatosis with polyangiitis (GPA) with myocardial infarction due to coronary involvement and subsequent atrioventricular block was made on day 4 of hospitalization. Daily 500 mg boluses of intravenous methylprednisolone were administered for three days since day 4 of hospitalization, followed by high dose oral glucocorticoids (prednisone 1 mg/kg/day) and subsequent remission induction therapy with rituximab 375 mg/m2 weekly. Renal function progressively improved; heart conduction abnormalities gradually recovered and there was a marked reduction of the size of the pulmonary nodule. GPA presents remains one of the most challenging diagnostic dilemmas in clinical medicine. Despite we performed a prompt diagnosis, a leg amputation had to be done due to the common rapid progression of the disease. Clinicians should be aware of the various manifestations of GPA and bear in mind the possibility of coronary vasculitis.