Experimental Biology 2010: Coronary Arteritis In Wegener's Granulomatosis

Purpose of Study: Introduction

Wegener's granulomatosis is a form of vasculitis that affects the lungs, kidneys, heart and other organ that requires long-term immunosuppression

Methods Used: Case Presentation
A 33 year old man was admitted with complains of exertional retrosternal chest pain mild to moderate in intensity, nonradiating, aggravated by exercise and relieved at rest.
In 2002, he had presented with complaints of hemoptysis and hematuria. A classic anti –neutrophil antibody test (C-ANCA) performed was positive and a diagnosis of Wegener’s granulomatosis was made. Treatment with cyclophosphamide and corticosteroid was started, with improvement in his symptoms.
On admission the patient was afebrile, his pulse was 98 /min; blood pressure 140/90 mmHg; and his lungs were clear to auscultation and cardiac examination was normal with no murmur or gallop. Initial investigations showed normal urine analysis, chest radiograph, full blood count, plasma chemistry, thyroid function test and cardiac enzymes. The electrocardiogram showed sinus rhythm with normal PR interval and no significant ST-T changes.
The cardiac catheterization revealed non-obstructive major epicardial coronary arteries with intra coronary thrombi and a beaded appearance involving the first and second diagonal vessels (Figures 1 and 2) with mildly reduced LV systolic function.

Figure 1:

Figure 2:

The patient was treated with short term low molecular weight heparin and was discharged on aspirin, ß blockers, ACE inhibitors, nitrates, statins and medical therapy for underlying Wegener’s granulomatosis was continued.

Summary of Results: Discussion
Wegeners granulomatosis is a disease that is characterized by necrotizing granulomatous vasculitis of the upper and the lower respiratory tracts, and the kidneys, was first described in 1936. A limited form of disease involving only the lung was later reported by Carrington and Liebow. Cardiac involvement has been reported in 6-44% of cases.
The case clearly depicts that possibility of coronary vasculitis must be considered in patients with Wegener’s granulomatosis who develops chest pain both in the diagnosis or remission period.

Conclusions: References
Arend WP, The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis.