Department of Rheumatology, Royal Free and University College Medical School, Pond Street, London NW3 2QG, UK
SIR, In 1986 Leavitt and Fauci [1] described a series of patients who had features of systemic vasculitis that could not be classified into one of the well-defined vasculitic syndromes. Here we report the case of a patient in whom clinical and immunological features of a small vessel vasculitis were associated with pulmonary aneurysms and silent myocardial infarction.
A 49-yr-old male smoker with a past history of chronic lower back pain presented to the ENT Department with a 6 week history of nasal discharge, sinusitis, cacosmia, marked swelling of the nose and progressive loss of hearing. Symptoms of weight loss and malaise were also present. Examination revealed muco-pus in the post-nasal space and crusting of the left nasal septum. Audiometry demonstrated mixed sensorineural and conductive deafness. The chest X-ray was normal.
Two weeks later the patient returned with worsening constitutional symptoms and rapidly progressive dyspnoea. On examination he was afebrile, tachypnoeic and tachycardic with a blood pressure of 100/60. Signs of a left pleural effusion were present. Urine dipstick testing revealed 1+ of protein and blood. Laboratory tests showed a normal white cell count (8x109/l), haemoglobin (14.3 g/dl), urea (3.3 mmol/l) and creatinine (78 µmol/l). The C-reactive protein was raised at 92 mg/l, and cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) was positive with high specificity for anti-proteinase-3 (titre 17.3 U; NR<2 U). Antinuclear antibodies, anticardiolipin antibodies and lupus anticoagulant status were negative. Arterial blood gases taken whilst the patient was breathing room air demonstrated hypoxia (oxygen partial pressure of 6.48 kPa) with a carbon dioxide partial pressure of 5 kPa. The chest X-ray confirmed a left pleural effusion with a small right pleural effusion (Fig. 1). Twelve-lead electrocardiography showed right bundle branch block with right axis deviation and no evidence of acute ischaemia. A pleural aspirate was performed revealing a serosanguinous exudative effusion (protein 24 g/dl; serum albumin 28 g/dl). No organisms were observed on Gram-stain or subsequent culture of the pleural fluid. Urine microscopy showed few leukocytes and red cells with no casts. Pulmonary function tests showed a restrictive pattern and no evidence of alveolar haemorrhage with a reduced carbon dioxide transfer factor (kCO=43% of predicted) and a forced expiratory volume to forced vital capacity (FEV1:FVC) ratio of 96%.
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An echocardiogram, performed as part of a multisystemic workup, unexpectedly revealed poor systolic function with an ejection fraction of <25% and an adherent apical left ventricular mural thrombus. The patient denied any past history of angina or hypertension and had no family history of ischaemic heart disease. His cholesterol level was 4.3 mg/dl. Troponin T was positive at 0.14 ng/dl. Left-sided cardiac catheterization revealed anterior hypokinesia consistent with a prior myocardial infarction, but no focal coronary stenosis. The presence of apical thrombus in the left ventricle was confirmed. Right-sided cardiac catheterization showed widespread, diffuse aneurysms in the medium-sized vessels of the pulmonary vascular bed (Fig. 2).
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The second unusual feature of this case is the occurrence of a silent myocardial infarction in a 49-yr-old patient resulting in mural thrombosis and poor left ventricular function. Although atherosclerotic coronary disease is a likely explanation, the absence of any significant coronary stenosis or prior history of chest pain raises the possibility of coronary arteritis leading to myocardial infarction. Although predominantly a disease of small vessels, involvement of medium-sized and even large vessels is well-described in Wegener's granulomatosis [7]. However, coronary arteritis leading to silent myocardial infarction is a rare manifestation of Wegener's granulomatosis [8]. Papo et al. [9] described two cases of silent myocardial infarction in patients with Wegener's granulomatosis who had no prior history of angina. More recently, Lawson and Williams [10] reported the death of a young man with Wegener's granulomatosis from a massive myocardial infarction. A third case report from Italy [11] reported an elderly woman suffering from a clinically unrecognized autoimmune disorder who died of a cardiac arrest. Post mortem results showed coronary arteritis with histological features of Wegener's granulomatosis and polyarteritis nodosa. The case we report above is thus also a further example of medium vessel involvement in the form of coronary arteritis in the context of a cANCA-positive small vessel vasculitis.
In conclusion, we report a unique case of cANCA-positive small-vessel vasculitis presenting with upper and lower respiratory tract involvement associated with pulmonary aneurysms and silent myocardial infarction.
Notes
Correspondence to: R. Farzaneh-Far. E-mail: ramfar{at}hotmail.com
References
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