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In the largest cohort published to date, no splenic involvement is described in Wegener’s granulomatosis (WG).1 We report on two patients who required splenectomy for symptomatic spleen infarction in the course of WG.
A 42 year old man was admitted with an eight month history of arthritis and lower limb dysesthesia. Examination showed an acutely ill patient with a 39°C fever, oral ulcers, haemorrhagic gingival hyperplasia, bilateral haemorrhagic nasal discharge with crusts, diffuse necrotic purpura, neuritis, and black discoloration of some fingers and toes. The spleen was not palpable. Silent anterior myocardial infarction was diagnosed because of raised MB-CK levels and ST-segment increase with loss of R waves in leads V1,V2,V3 on electrocardiogram.2Antineutrophil cytoplasmic antibodies (c-ANCA) were disclosed in serum and necrotising vasculitis was shown on skin biopsy specimen.3 No antiphospholipid antibody or coagulation protein abnormality could be disclosed. Treatment consisted of intravenous administration of prednisolone, cyclophosphamide, sodium heparinate, diltiazem, dinitrosorbide and enalapril. His short-term course was uneventful. At day 14, the patient suddenly developed a severe haemorrhagic shock. Echotomography of the abdomen showed a splenic mass. At laparotomy, the spleen was almost disrupted by voluminous haematoma. Histological analysis of the spleen showed widespread necrotising vasculitis with haemorrhagic infarction. After five years of follow up, the patient is in complete remission with oral cotrimoxazole treatment.
A 23 year old young man was admitted in August 1996 because of repeated otitis media, sinusitis, epistaxis, headache, arthralgia with fever and weight loss. Despite a short course of oral corticosteroids and antibiotics, his general condition worsened. Antiproteinase 3 c-ANCA were disclosed in serum. Chest computed tomography showed pulmonary nodules. Intranasal endoscopic biopsies demonstrated necrotising vasculitis with epithelioid and giant cells. Treatment included oral prednisone and intravenous cyclophosphamide pulses. After a few days, serum creatinine concentrations abruptly increased to 198 μmol/l and urine analysis showed microscopic haematuria and proteinuria. High dose methylprednisolone pulses were then given, intravenous cyclophosphamide was changed to a 100 mg oral daily regimen and the patient eventually achieved remission. In October 1996, abdomen computed tomography showed an intrasplenic lesion that was consistent either with a splenic infarct or haematoma (fig 1). The later course was marked by a WG flare in January 1997, which was complicated with massive thrombosis of the left iliofemoral vein and the inferior vena cava. No thrombophilic disorder could be found. Intravenous heparin then oral anticoagulation with acenocoumarol were given. Because of persistent left hypocondrium tenderness, splenectomy was performed in September 1997. Histological examination showed spleen infarction with organised haematoma and sequelae of vasculitis (fig 2).
Because they are vessels without collateral flow, occlusion of distal parenchymal splenic arteries leads invariably to splenic infarction. Of note, two of the three patients described by Wegener in 1936 had spleen involvement.4 The frequency of spleen involvement ranges from 50% to 100% of WG cases at necropsy.5-7Histological data frequently showed massive or multiple areas of splenic necrosis, associated to a variable extent with central arteritis, splenic trabeculitis, follicular arteriolitis, disseminated parenchymatous granulomata and capsulitis (fig 2). Patients with splenic infarction in WG usually remain asymptomatic. Prominent splenomegaly is rare.6 With computed tomography, focal splenic infarction appears as well defined, peripheral wedge shaped areas of low attenuation. In WG, the diffuse vasculitis process often results in massive hypodense lesions involving the spleen parenchyma (fig 1).8-10 A peripheral rim of enhancement may be seen, as for spleen abscess, haematoma and lymphoma.11Splenectomy has been performed successfully in some patients.10 ,12 ,13 Spleen lesions may also appear to heal on repeated computed tomography under medical treatment only, consisting of prednisone and cyclophosphamide.14
Few recent reports of splenic involvement in WG provide histological analysis from live patients.10 ,12 ,13 Inaugural spontaneous rupture of a normal sized spleen with only sub-capsular neutrophil infiltrate has been described in a patient who subsequently developed full blown WG.15 In another case, a spontaneous splenic haemorrhage was ascribed to vasculitis in a patient who had severe WG that required haemodialysis.13 In our two patients, microscopical study of the spleen also showed haemorrhagic infarction caused by specific WG related vasculitis process. A severe splenic haemorrhage occurred in patient 1, which was clearly related to both necrotising vasculitis and hypocoagulable state. Anticoagulation was indicated for inaugural myocardial infarction in case 1 and deep venous thrombosis in case 2, in both cases during active WG flare. Splenectomy was required in both our cases.
Our data suggest that antithrombotic treatment entails a specific risk of bleeding complications in patients with WG vasculitis. When anticoagulation is necessary in WG patients, computed tomography of the abdomen should be systematically performed and, if splenic infarction is disclosed, splenectomy should be considered.
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