Ruptured arterial aneurysm of the kidney in a patient with Wegener’s granulomatosis

Raimund Senf1, Jan S. Jürgensen1, Ulf Teichgräber2, Dieter Kampf1 and Ralf Schindler1

1Department of Medicine, Division of Nephrology and Medical Intensive Care and 2Department of Radiology, Charité, Campus Virchow-Klinikum, Humboldt-University, Berlin, Germany

Correspondence and offprint requests to: Raimund Senf, MD, Department of Medicine, Division of Nephrology and Medical Intensive Care, Charité, Campus Virchow-Klinikum, Augustenburger Platz 1, D-13353 Berlin, Germany. Email: raimund.senf{at}charite.de

Keywords: ANCA-associated vasculitis; arterial aneurysm; bleeding complication; Wegener’s granulomatosis



   Introduction
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 Introduction
 Case
 Discussion
 References
 
Wegener’s granulomatosis (WG) is a necrotizing granulomatous vasculitis that has a clinical predilection for the upper airways, lungs and kidneys. It was first reported by Klinger in 1931 and later described in more detail by Wegener [1]. Like the other forms of ANCA-associated small-vessel vasculitides it preferentially involves venules, capillaries and arterioles, but it may also affect arteries and veins. Diagnosis is based on histological findings of granulomatous inflammation in clinically compatible settings. It is strongly associated with antineutrophil cytoplasm antibodies (ANCA), especially the PR3-ANCA, which has a cytoplasmatic immunoflourescent staining pattern (cANCA) [1,2]. Several efforts have been made to distinguish between different forms of systemic vasculitides. Two widely adopted systems for the classification of vasculitis are the ACR criteria from 1990 and the nomenclature system from the 1994 Chapel Hill Consensus Conference [1].

We report on the first manifestation of WG in a young male patient with respiratory and renal failure. Although he clearly met the criteria for WG according to the mentioned classifications, he also showed features and complications typical for polyangiitis nodosa.



   Case
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 Introduction
 Case
 Discussion
 References
 
A 35-year-old man, previously healthy, was admitted to a regional hospital with a history of arthritis and weakness for ~6 weeks. He had elevated body temperature for 2 weeks and had been treated with doxycycline without any improvement. Two days before admission epistaxis, conjunctivitis and haemoptysis occurred.

Pneumonia was considered as a likely differential and broad-spectrum antibiotics were applied. Nevertheless, the patient’s condition deteriorated rapidly and he was transferred to our tertiary care ICU in respiratory and acute renal failure. The patient developed septic shock necessitating catecholamines, mechanical ventilation and continuous renal-replacement therapy.

A chest radiograph on admission showed bilateral nodular infiltrates and pleural effusion. The urine analysis revealed ++ proteinuria and haematuria as well as red-cell casts. cANCAs were detected by immunofluorescence confirmed to be directed against PR3 by ELISA. pANCA, anti-glomerular basement membrane, double-strain DNS and small nuclear antibodies were negative. A bronchoscopic biopsy of the lower respiratory tract demonstrated granulomatous inflammation with several giant-cells and necrosis. Thus, the suspected diagnosis of WG was confirmed in view of these data. Immunosuppressive therapy was initiated including cyclophophamide-pulse, 750 mg i.v., corticosteroid-pulse with tapering off to 2 mg/kg daily and plasmapheresis. The patients condition improved slowly over the next 3 weeks. After 10 days he was successfully weaned off the respirator. Oliguric renal failure persisted necessitating intermittent haemodialysis.

On day 24, he complained of a sharp pain with sudden onset in his left side. Tachycardia, hypotension and a drop in haemoglobin from 10.1 to 7.0 g/dl developed in the subsequent hours, all signs of haemorrhage.

Abdominal ultrasonography and CT scan (Figure 1) revealed a large left-sided perinephric haematoma. A selective arterial angiography (Figure 2) showed multiple impressive aneurysms in splanchnic, hepatic and renal arteries. No active bleeding was present. The patient recovered with transfusion, supportive therapy and monitoring alone.



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Fig. 1. Abdominal CT scan displaying a large left-sided perinephric and intracapsular haematoma.

 


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Fig. 2. Arterial angiography shows disseminated arterial microaneurysms as typically found in polyangiitis nodosa. Similar formations exist in splanchnic and liver arteries.

 
Since oliguric renal failure persisted for more than 6 weeks, an arterio-venous fistula was created and chronic haemodialysis was initiated.



   Discussion
 Top
 Introduction
 Case
 Discussion
 References
 
Systemic vasculitides were classified by the ACR criteria and the nomenclature system from the Chapel Hill Consensus Conference [1]. WG therefore is restricted by definition to patients with necrotizing granulomatous inflammation. The occurrence of multiple arterial aneurysms, as seen in the arterial angiography, is a classical feature of polyarteritis nodosa (PA) and can be found in 50–60% of cases. Most commonly (80–90%) the kidney is involved, followed by the gastrointestinal tract, liver, spleen and pancreas [3]. Almost 20 years before the Chapel Hill Consensus Conference, Lundstrom et al. [4] described nephroangiography as a diagnostic tool to differentiate between WG and a disease they called panarteriitis nodosa, which combined clinical features of PA and microscopic polyangiitis (MPA). As with Churg-Strauss syndrome and MPA, the other two major forms of small-vessel vasculitis, WG is strongly associated with ANCAs detected by immunofluorescence. Most patients with WG have ANCAs directed against proteinase 3 (anti-PR3), whereas ANCAs against myeloperoxidase (anti-MPO) prevail in most patients with Churg-Strauss syndrome or MPA [1]. The described patient presented with clinical features typical for WG: pulmonary manifestation and epistaxis, which occur in up to 90% of WG patients but only in 35–50% of MPA patients [1]. We diagnosed WG regarding the clinical symptoms, the PR3-ANCAs and biopsy-proven necrotizing granulomatous inflammation with giant-cells. A kidney biopsy was not performed because of low platelets and a coagulation disorder early in the course and for the risk of another bleeding complication later in the course.

To the best of our knowledge, only four cases of WG complicated by rupture of an arterial aneurysm have been reported previously. The first report by Baker et al. [5] in 1978 described a 24-year-old male with multiple bilateral renal arterial aneurysms. He survived a massive bleed with interventional occluding of the ruptured aneurysm. In 1987, Hartmann [6] reported the first case with lethal bleeding from a ruptured renal arterial aneurysm in WG. A 56-year-old man died of intraperitoneal haemorrhage from a ruptured aneurysm of his left gastric artery as described by Aoki et al. [7] in 1995. Finally, den Bakker et al. [8] reported a 55-year-old male with WG who died in hypovolaemic shock resulting from a rupture of a hepatic artery aneurysm. Moutsopoulos et al. [9] in 1983 and Pumpe et al. [10] in 1989 described three more cases of WG with renal arterial aneurysms accidentally found by renal angiography, but those showed no bleeding complication. Taken together, these reports illustrate that a clear-cut differentiation of particular vasculitides according to the Chapel Hill Consensus Conference and also the ACR classification may be impossible because of significant overlap of clinical presentations. It is debatable, if a new category of vasculitis like ‘polyangiitis overlap syndrome’ as proposed by some authors in the past, is helpful rather than confusing.

However, due to the very rare occurrence of fatal bleeding in WG it does not seem to be efficient to perform angiography in every diagnosed individual. Finally, it is necessary to monitor patients with acute onset of disease carefully for bleeding complications, especially after an organ biopsy has been undertaken.

Conflict of interest statement. None declared.



   References
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 Introduction
 Case
 Discussion
 References
 

  1. Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med 1997; 337: 1512–1523[Free Full Text]
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Received for publication: 4. 3.03
Accepted in revised form: 27. 5.03





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