Wegener's granulomatosis presenting as an abdominal aortic aneurysm: a case report
© Durai et al; licensee BioMed Central Ltd. 2009
Received: 30 November 2009
Accepted: 18 December 2009
Published: 18 December 2009
Aortic aneurysm is not common in young patient. When a young patient presents with abdominal aortic aneurysm, there may be an underlying cause.
Here, we describe a case of a 33-year-old gentleman who presented with flu like illness, chest and abdominal pains following a tooth extraction. A chest X-ray and subsequent computerised tomogram of the chest and abdomen demonstrated lung nodules and an abdominal aortic aneurysm. The aneurysm was repaired and his serology was positive for Wegener's granulomatosis. A nasal mucosal biopsy confirmed WG. He was treated with oral steroids and cyclophosphamide. His graft leaked and had to be replaced with a synthetic graft. Two months after his re-operation, he remains well.
Whenever a young patient presents with an abdominal aortic aneurysm, an underlying connective disease should be excluded because early steroid/immunosuppressive treatment may prevent the development of further aneurysms.
A young patient presenting with an abdominal aortic aneurysm is extremely rare. Here, we describe a case of a young male who presented with flu like illness, chest and abdominal pains following a tooth extraction. Investigations revealed an abdominal aortic aneurysm associated with Wegener's granulomatosis (WG). There are only two previous case reports in the literature on abdominal aortic aneurysm due to WG . Hence our case is the third of its kind. This case is peculiar because only nasal biopsy confirmed the disease.
Initially it was thought that the aneurysm was mycotic from his left lung abscess. The patient underwent bronchoscopy which was not successful. The aneurysm was excised partially and repaired with an internal jugular vein (IJV) graft. The aneurysm wall was sent for histology but due to a portering error the sample never reached the laboratory. The patient was discharged home on the 6th post-operative day but he returned with severe abdominal pain on the seventh post operative day. A CT scan showed free fluid in the abdomen. The patient underwent re-laparotomy which revealed a hole in the IJV graft. The IJV graft was removed and replaced with an aorto-iliac silver impregnated synthetic trouser graft. During this time bloods were sent off for connective tissue screening which was positive for anti-proteinase PR3 (>1/10). Histology of a CT guided lung biopsy showed only necrotic tissue. His CRP and WCC remained high, but his blood cultures and aortic tissue never grew any bacteria. Therefore a nasal mucosal biopsy was organised which confirmed the presence of Wegener's granulomatosis.
The patient was referred to a Rheumatologist and was started on prednisolone and cyclophosphamide. A few days after initiation of medical treatment, the patient felt a lot better and was discharged home for follow up.
Studies showing involvement of major arteries in WG.
63 year old male an inflammatory aortic aneurysm and polyneuropathy.
Methyl prednisolone and trimethoprim-sulfamethoxazole
34-year old Japanese man
Pneumonia, paranasal sinusitis and clipping of a cerebral aneurysm
Anterior choroidal artery.
Prednisolone + cyclophosphamide
67-year old man
presented with abdominal pain and shock
Superior pancreatico-duodenal artery
Open repair of ruptured artery
Died from multi-organ failure
50-year old woman presented with abdominal pain and shock
Entire aorta(first intercostal artery to iliac bifurcation)
Was on steroids and cyclosphosphamide but could not prevent aortic dissection
Died from aortic dissection
58 year-old woman, pain in the upper limb
Insertion of stent-graft and steroids
Previous AAA patient
Aorta and subclavian
Details not known
56-year-old Japanese man presented with shock
Ruptured left gastric
Aneurysm was diagnosed post mortem
Died of hemorrhagic shock
A hospitalized developed sudden hypovolemic shock
Ruptured hepatic artery aneurysm
Aneurysm was diagnosed post mortem
Died of hemorrhagic shock
Presented with respiratory and renal problems
Renal artery aneurysm
Aneurysm was diagnosed post mortem
24 year old with massive perinephric haematoma
Bilateral renal artery aneurysm
Steroid and angioembolisation
Successfully recovered from the episode
29 year old with know WG presented with abdominal pain and vomiting
Renal and hepatic artery aneurysms
Steroid & angioembolisation
WG may affect the respiratory  and renal tracts . It can also affect the eyes, skin, and peripheral nerves. Non-specific systemic symptoms are common. WG causes upper respiratory tract disease in > 90% of cases and causes sinusitis, nasal crusting, bleeding, obstruction and collapse of the nasal bridge. It can also cause otitis media and tracheal stenosis. When the lungs are affected, it may present with cough, haemoptysis and dyspnoea. Renal involvement may manifest as haematuria and proteinuria and can lead to renal failure. Ophthalmological manifestation includes sub conjunctival haemorrhages, scleritis, uveitis, keratitis, proptosis, or ocular muscle paralysis due to retro-orbital inflammation.
Differential diagnosis for WG
Usually associated with asthma
Necrotising vasculitis affecting small to medium sized vessels
Eosinophilia and p-ANCA will be elevated
In WG c- ANCA will be high
Necrotising glomerulonephritis common
Mainly small vessel vasculitis
Negative for PR 3
Antiglomerular antibody will ne negative
Head ache, jaw claudication
aorta and its major branches, especially extra cranial branches of carotid artery
Often affects temporal artery
Temporal artery biopsy is negative in 50%
Absent upper limb pulses, systemic features such as fever, weight loss and joint pains
Granulomatous inflammation of aorta and its major branches
Periaortitis, aortic dissection
aneurysm and thrombosis of subclavian
left gastric, hepatic and renal artery aneurysms
Diagnosis based upon American college of rheumatology criteria
Mainly affects Asian women
Chronic back pain in young
5% can get aortitis
Patchy destruction of both muscle and elastic tissue of media
Fibrosis of intima
Radiologically there will sacroilitis
The main treatment is immunosuppression and steroid therapy. A combination of cyclophosphamide and prednisone is effective  in > 90% with severe disease. Methotrexate and prednisone are useful when the disease spares the kidneys. Prophylactic antibiotics such as Septrin (Trimethoprim and Sulfamethoxazole) may prevent respiratory infections causing flare ups of WG. Untreated WG is fatal in 5 years . Prednisone may slow progression of the disease but by itself is insufficient to arrest the disease.
Whenever a young patient presents with an isolated aneurysm, dissection or aortitis screening for connective tissue disease should be performed. More than one vessel involvement in a young patient should alert the clinician to exclude a systemic disease. Early steroid therapy and immunosuppression may alter the course of the disease and hence its prognosis.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Appendix 1 - Criteria for diagnosing WG
Painful or painless oral ulcers or purulent or bloody nasal discharge
Chest radiograph showing the presence of nodules, fixed infiltrates, or cavities
Haematuria (>5 red blood cells per high power field) or red cell casts in urine sediment
Histological changes showing granulomatous inflammation within the wall of an artery or in the perivascular or extra vascular area (artery or arteriole)
internal jugular vein
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