Severe infections following leflunomide therapy for Rheumatoid Arthritis

started on antibiotics and transferred to a renal unit where he was treated with plasma exchange followed by prednisolone and cyclophosphamide. He made a good recovery and returned to work 3 months later. Cardiac involvement in WG was first described by Wegener in 1936 [1]. Abnormalities reported in this and subsequent case studies include myocarditis, pericarditis, valvular defects, vegetations, conduction defects and coronary arteritis, caused by either granulomatous infiltration or vasculitis [1–3]. The incidence of cardiac disease in WG is unclear. In one of the largest studies, where 158 patients with WG were followed up for an average of 8 years, 6% had disease affecting the heart, with only a third of these having involvement of the coronary arteries [3]. However, there are several reports of patients developing ‘silent’ MIs, often only identified following post mortem or during routine investigations [2, 4–6]. This calculation of the incidence of coronary arteritis could, therefore, be an underestimate. Surprisingly, there are only three reports of patients with WG developing MIs with symptoms of chest pain [7–9]. In all three cases, the MI occurred early in the course of the disease and approximately a week after the diagnosis of WG being made. One patient died from the MI before immunosuppressive treatment could be started [7]. Post mortem examination revealed all three arteries to be thrombosed at the distal segments. With the other two cases, the MI occurred after commencing immunosuppressive treatment [8, 9]. Both patients underwent coronary angiography and were both found to have significant stenotic lesions in the LAD artery requiring stenting. They both made a good recovery. There has also been a case report of a patient with WG developing pulmonary oedema with ST changes consistent with an MI, but who did not experience chest pain [10]. The MI also occurred early in the course of the disease, soon after diagnosis and once steroid therapy had been started. He died shortly after. Post mortem examination reported diffuse coronary arteritis with arterial occlusion, although did not specify which artery. Our case is unique in that this is the first reported case of a patient presenting with a symptomatic MI as the initial presenting symptom of their WG. Wegener’s granulomatosis classically presents with granulomatous disease of the respiratory tract (localized WG) followed by inflammation of the smallto medium-sized blood vessels (generalized WG) [11]. Interestingly, in our case, the coronary angiogram showed that our patient had developed a coronary vasculitis, whilst having only mild upper respiratory tract symptoms and few of the constitutional symptoms typical of generalized WG. It is possible that the pre-existing atherosclerosis in our patient led to symptoms of myocardial ischaemia in the early stages of the vasculitis. However, it is noted that of the eight previous reports of MIs in patients with WG, several also appear to have occurred during an early stage of the disease, suggesting that this is the period in which patients with WG may be most susceptible to coronary arteritis [4–10]. This is only the third coronary angiogram reported in a patient with active WG. Like the other two angiograms [8, 9], this showed no abnormalities diagnostic of vasculitis, as is often seen in other systemic vasculitides such as polyarteritis nodosa, but did show an unusual distribution of occluded smalland medium-sized vessels, particularly at the cardiac apex. Involvement of the distal segments was also seen in one of the other previously reported angiograms [9] and has also been seen during post-mortem examination [7]. These observations would seem to reflect the fact that WG is a vasculitis that predominantly affects the smaller blood vessels first [11], with the distal branches possibly being more likely to be affected in WG because of their smaller diameter. It is also noted that all three angiograms identified stenoses in the LAD artery rather than the other main branches but we cannot provide an explanation for this. In conclusion, we report a unique case of a young man with multiple cardiac risk factors, who presented with an apparently straightforward acute inferior MI who subsequently developed symptoms, signs and serological evidence of WG. We have observed that coronary arteritis may occur early in WG and suggest careful cardiac screening of all patients with newly diagnosed WG.