Coronary vasculitis in granulomatosis with polyangiitis

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a granulomatous disorder usually associated with vasculitis involving the small and medium-sized blood vessels. It most commonly affects the upper and lower respiratory tracts and the kidneys, but almost any organ can be involved [1,2]. Cardiac involvement in GPA is rare and different manifestations are described such as pericarditis, valvular lesions, coronary arteritis, myocarditis and cardiac rhythm disorders [3,4]. We described a young woman, with a previous diagnosis of GPA, who has been subjected to coronography after a positive cardiac stress test. A 35-year old woman was admitted to our outpatient clinic for a recent history of dyspnea onmoderate exertion, fever and cough. She denied a familiar history of cardiac disease. She was no smoker. The patient was diagnosed a year earlier with GPA with upper and lower respiratory tract involvement, renal failure at second stage, positive for antineutrophil cytoplasmic antibodies, anti-proteinase 3 (cANCA). No renal biopsy was performed. Moreover a previous brain MRI showed a brain involvement with microvascular inflammatory lesions in various stages of development. She also had arterial hypertension for 2 years before admission, for which she was taking 20 mg of olmesartan with normal blood pressure values and she had a hypothyroidism secondary to thyroidectomy for multinodular goiter. She was on therapy with prednisone at 25 mg/day, esomeprazole and in the weeks prior our hospitalization she had a bronchiolitis with resolution after antibiotic therapy. On admission, she complained of constrictive chest pain during moderate effort; blood pressure was 130/70 mm Hg, heart rate was 72/min, clinical examination was unremarkable, with no respiratory or cardiac pathological findings on auscultation. The chest radiograph was normal. The ECG looks almost normal (Fig. 1A). Laboratory showed an elevated sedimentation rate (73 mm/h) and C-reactive protein levels (5.93 mg/dl). Troponin I and myoglobin were normal; c-ANCA was 63 (normal value 0.0–2.0 UI/ml). Creatinine was 1.5 mg/dl, hemoglobin was 12.9 mg/dl, white blood cell was 9700 mm. Echocardiogram showed: mild concentric hypertrophy of the left ventricle, hypokinesia of the distal segment of the anterolateral wall with preserved global pump function, sclerosis and calcification of mitral and aortic valves; small pericardial effusion rear right atrium and systolic pericardial dissection of the free wall of the right ventricle (Fig. 1B and C); I degree diastolic dysfunction. For further diagnostic evaluation the patient undergone to dobutamine stress echocardiography [5,6], early stopped (20 γ/kg/ min) for the onset of constrictive retrosternal pain associated with electrocardiographic changes suggestive of ischemia in the absence of new mechanical alterations (Fig. 2). The following coronary angiography showed a diffuse coronary artery disease without hemodynamically significant stenosis with the exception of a 90% stenosis of the proximal portion of the left anterior descending artery, engaging the emergence of the first diagonal branch (Fig. 3A and B), treatedwith percutaneous transluminal coronary angioplasty (PTCA) with position of stent (Fig. 3C). Cardiac involvement in GPA has been reported to range between 6% and 44% [3,4] with coronary artery disease reported in the 50% of all cases of heart involvement in GPA [7]. Accelerated atherogenesis, one of the most important mechanisms triggering coronary disease, is well documented in GPA and patients affected by Wegener's granulomatosis are at a significantly increased risk of cardiovascular morbidity and mortality [8,9]. Moreover the GPA patients were found to have a 3.6-fold increased risk of acute MI occurring 5.0 years after the date of the GPA diagnosis [10,11].