Small vessel vasculitis with pulmonary aneurysms and silent myocardial infarction

R. Farzaneh-Far, J. Watkins, H. Tahir, F. Wykes and H. Beynon

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. 1Go). 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%.



View larger version (152K):
[in this window]
[in a new window]
 
FIG. 1. Chest X-ray showing left pleural effusion.

 
Based on the clinical presentation and immunological markers, a provisional diagnosis of limited Wegener's granulomatosis was made and treatment was commenced with pulsed intravenous methylprednisolone and cyclophosphamide followed by high-dose oral prednisolone. A dramatic clinical improvement occurred over the next 24–48 h, mirrored by a reduction of the C-reactive protein to 18 mg/l. The patient's dyspnoea and constitutional symptoms resolved and his hearing began to return.

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. 2Go).



View larger version (163K):
[in this window]
[in a new window]
 
FIG. 2. Pulmonary angiogram showing widespread, diffuse aneurysms in the medium-sized vessels of the pulmonary vascular bed.

 
The case described above is noteworthy in two important respects. First, the presence of multiple pulmonary aneurysms has not hitherto been described in association with a cANCA+ small vessel vasculitis. However, there are several case reports of multiple aneurysms occurring in other organs in the context of a cANCA-positive small vessel vasculitis [2, 3]. Grosser et al. [4] reported the case of a patient with Wegener's granulomatosis who was found to have multiple aneurysms in the peripheral branches of the renal artery. At post mortem these angiographic abnormalities corresponded with necrosis of the arterial wall and destruction of the elastic lamina. Pumpe et al. [5] also reported two cases of Wegener's granulomatosis associated with multiple arterial aneurysms in the renovascular bed. Aneurysm formation in the renal vasculature is a well-recognized feature of classical polyarteritis nodosa, an ANCA-negative non-granulomatous, focal necrotizing vasculitis of medium-sized muscular arteries. Therefore, these cases probably constitute overlap syndromes of Wegener's granulomatosis and polyarteritis nodosa. However, classical polyarteritis nodosa invariably spares the pulmonary vasculature [6]. Our patient thus exhibited the clinical and immunological features of Wegener's granulomatosis with angiographic features of polyarteritis nodosa in an atypical vascular territory.

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 Back

References

  1. Leavitt RY, Fauci AS. Polyangiitis overlap syndrome. Classification and prospective clinical experience. Am J Med 1986;81:79–85.[ISI][Medline]
  2. Shitrit D, Shitrit AB, Starobin D et al. Large vessel aneurysms in Wegener's granulomatosis. J Vasc Surg 2002;36:856–8.[ISI][Medline]
  3. Sieber SC, Cuello B, Gelfman NA, Garfinkel HB. Pulmonary capillaritis and glomerulonephritis in an antineutrophil cytoplasmic antibody-positive patient with prior granulomatous aortitis. Arch PAthol Lab Med 1990;114:1223–6.[ISI][Medline]
  4. Grosser G, Mundinger A, Pumpe C, Herbst EW, Kuhlmann M, Kropelin T. Renal artery aneurysms in Wegener's granulomatosis. Rofo Forthschr Geb Rontgenstr Neuen Bildgeb Verfahr 1989;151:167–70.
  5. Pumpe K, Mundinger A, Herbst EW, Peter P. Wegener's granulomatosis of the prostate with the development of arterial microaneurysms of the kidney. Pathologe 1989;10:318–21.[ISI][Medline]
  6. Cohen RD, Conn DL, Ilstrup DM. Clinical features, prognosis and response to treatment in polyarteritis. Mayo Clin Proc 1980;55:146–55.[ISI][Medline]
  7. Parums DV. The arteritides. Histopathology 1994;25:1–20.[ISI][Medline]
  8. Parry SD, Clark DM, Campbell J. Coronary arteritis in Wegener's granulomatosis causing fatal myocardial infarction. Hosp Med 2000;61:284–5.[ISI][Medline]
  9. Papo T, Piette JC, Larkaki R, Bletry O, Huong DL, Godeau P. Silent myocardial infarction in Wegener's granulomatosis. Rheum Dis 1995;54:233–4.
  10. Lawson TM, Williams BD. Silent myocardial infarction in Wegener's granulomatosis. Br J Rheumatol 1996;35:188–91.[ISI][Medline]
  11. Morbini P, Dal Bello B, Arbustini E. Coronary artery inflammation and thrombosis in Wegener's granulomatosis-polyarteritis nodosa overlap syndrome. Giornalo Italiano di Cardiologia 1998;28:377–82.
Accepted 3 January 2003





This Article
Full Text (PDF)
Alert me when this article is cited
Alert me if a correction is posted
Services
Email this article to a friend
Similar articles in this journal
Similar articles in ISI Web of Science
Similar articles in PubMed
Alert me to new issues of the journal
Add to My Personal Archive
Download to citation manager
Search for citing articles in:
ISI Web of Science (1)
Disclaimer
Request Permissions
Google Scholar
Articles by Farzaneh-Far, R.
Articles by Beynon, H.
PubMed
PubMed Citation
Articles by Farzaneh-Far, R.
Articles by Beynon, H.
Related Collections
Other Rheumatology