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Wegener's granulomatosis (WG) is a necrotising, granulomatous vasculitis that classically involves the clinicopathological triad of upper and lower respiratory tracts and the kidney.1 Less frequently, the disease may affect other organs as well. Serious and occasionally fatal complications within the spleen occur in many autoimmune rheumatic diseases,2 and prompt recognition of these complications is important. In a necropsy series of patients with WG, the spleen was commonly affected: 78–100% of patients had splenic lesions with a combination of necrosis, vasculitis, and granuloma formation.3,4 Clinically apparent splenic disease is rare, however.1 We wish to report briefly the case of a 47 year old woman who presented with manifestations of classical WG and radiological evidence of splenic infarcts.
A 47 year old woman during the past month developed fevers to 38.6°C associated with weight loss, diffuse arthralgias, anaemia, and erythrocyte sedimentation rate of more than 100 mm/1st h. During the past three months she complained of nasal congestion and occasional episodes of epistaxis. A chest x ray examination showed a left upper lobe density, and her family doctor prescribed oral amoxicillin in combination with clarithromycin, without improvement. Ten days before admission gross haematuria was noted and a freshly collected urinary sediment showed the presence of red blood cell casts. Renal function declined rapidly with a serum creatinine level of 600 μmol/l and the patient was referred to the hospital for further investigation. A positive cANCA titre (1/160) was found using the indirect fluorescence technique and a positive antiproteinase 3 result on enzyme linked immunosorbent assay (ELISA; 66 U/ml). WG was considered on the basis of ANCA analysis results and the multisystemic nature of the disease and a percutaneous renal biopsy was performed under computed tomography (CT) guidance. Evaluation of renal biopsy specimens with light microscopy and immunofluorescence showed an acute necrotising segmental pauci-immune glomerulonephritis with crescent formation in more than 50% of the glomeruli. An impressive and rather unexpected CT finding was the presence of well defined areas of low attenuation within the spleen, consistent with infarction, with small areas of enhancement within the larger hypodense lesions (fig 1). A search for lupus anticoagulant and anticardiolipin antibodies was negative and no abnormalities of blood clotting could be detected. A trans-oesophageal echocardiogram failed to detect cardiac sources of emboli.
Treatment was started with 500 mg methylprednisolone intravenously per day for three consecutive days together with cyclophosphamide at a dose of 2 mg/kg body weight per day orally. After three days methylprednisolone was continued at 48 mg/day orally. Pulmonary and renal function progressively recovered and serum creatinine was 150 μmol/l on discharge.
Today, three years after the initial presentation, the patient is in stable remission and serum creatinine is 125 μmol/l. A recently performed CT scan showed a considerable volume reduction and scarring of the spleen (fig 2).
Splenic involvement in WG has included such abnormalities as splenomegaly, capsular adhesion, impaired splenic function, and infarcts.2 Infarction may occur as a result of a distal occlusion of the splenic artery or its branches, because splenic parenchymal arteries are end vessels that do not communicate with one another.5 There are few reports on splenic infarction on post mortem in patients with WG.3,4 Histological examination frequently shows massive or multiple areas of splenic necrosis, usually associated with extensive central arteritis, splenic trabeculitis, follicular arteriolitis and necrosis, disseminated visceral granulomata, and capsulitis. On CT, splenic infarcts classically and more commonly appear as peripheral, well defined, wedge shaped areas of low attenuation.5 However, other patterns of infarction have been recognised. These include multiple heterogeneous low attenuation lesions; regions of normal enhancement centrally with peripheral low attenuation; and large, low attenuation hypodense lesions that may have a rim of enhancing tissue peripherally.5,6 Examination with ultrasound in combination with duplex sonography of splenic blood supply permits non-invasive diagnosis of splenic infarction.7 The diagnosis can be confirmed by magnetic resonance imaging or CT scan, which permits assessment of the extent of splenic infarction.
Splenic involvement in WG may be more prevalent than previously believed.6–10 Pain in the left upper quadrant and left shoulder and fever may be present after splenic infarction, but many patients remain asymptomatic.5,7 Consequently, cross sectional imaging is not often carried out and the lesion may frequently go unrecognised. Unless there are signs of imminent rupture of the spleen or bleeding,11 a conservative approach is justified. In the long term these patients may be more susceptible to pneumococcal infection because of the functionally asplenic condition.7 This possibility provides further help in the diagnosis of this rare condition in vivo.
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