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BRIEF REPORT

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Wegener Granulomatosis with Spleen Infarction:Case Report and Review of the Literature

Alessandra Ghinoi, MD,* Nicolò Pipitone, MD, PhD,*Alberto Cavazza, MD,† Luigi Boiardi, MD, PhD,* and

Carlo Salvarani, MD*

Objective: To report a case of biopsy-verified Wegener granulomatosis (WG) with positivec-antineutrophil cytoplasmic antibodies who had evidence of spleen infarction and to review therelevant literature on spleen involvement in WG.Methods: Descriptive case report of 1 patient with WG complicated by spleen infarction andreview of the relevant literature (PubMed search 1966 through February 2007).Results: In our patient spleen involvement (infarction) was clinically silent and was incidentallydetected on the caudal slides of a chest computed tomography (CT) performed to investigatepulmonary involvement. Similar cases have been reported: in the published literature reviewed,overall 18 patients have been described and other cases have been described after autopsy. Of these,8 patients were symptomatic: generally, they complained of diffuse or left upper quadrant abdom-inal pain. In 15 patients, spleen involvement was detected by ultrasound, scintigraphy, CT scan,or magnetic resonance imaging. In 1 case only was the diagnosis confirmed by biopsy (aftersplenectomy).Conclusions: The frequency of spleen infarction is probably underestimated in WG since it is oftenclinically silent as in our patient. In addition to spleen infarction, WG has also been implicated ininducing spleen hemorrhage, capsular adhesions, dysfunction, and splenomegaly. Since some ofthe spleen lesions may lead to complications, a high index of suspicion should be maintained forspleen involvement in patients with WG, particularly in the presence of abdominal symptoms.© 2008 Elsevier Inc. All rights reserved. Semin Arthritis Rheum 37:328-333Keywords: Wegener granulomatosis, spleen

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egener granulomatosis (WG) is a systemic ne-crotizing vasculitis affecting small- and medi-um-sized vessels with associated granuloma

ormation. Interaction of varying trigger factors on a het-rogeneous genetic background may be responsible for aorth–South gradient in the incidence of WG. In this

egard, latitudinal trends in the incidence of WG mayxplain the higher incidence observed in North Norwayompared with that observed in Northwest Spain (1).ypically, the upper and lower respiratory tract and often

he kidneys are involved. However, other organs includ-

Department of Rheumatology, Arcispedale S Maria Nuova, Reggio Emilia, Italy.†Department of Pathology, Arcispedale S Maria Nuova, Reggio Emilia, Italy.Address reprint requests to Carlo Salvarani, Servizio di Reumatologia, Department

ff Rheumatology, Arcispedale S Maria Nuova, V.le Risorgimento n 80, 42100,eggio Emilia, Italy. E-mail: salvarani.carlo@asmn.re.

28 0049-0172/08/$-see front matter © 2008 Elsevier Inc. All rights reserved.doi:10.1016/j.semarthrit.2007.07.009

ng the skin, joints, heart, central nervous system, and eyesan also, albeit less frequently, be affected (2). In contrast,pleen involvement secondary to WG has only rarely beenescribed. We report herein a patient with biopsy-verifiedG and positive cytoplasmic antineutrophil cytoplasmic

ntibodies (c-ANCA) who had evidence of diffuse spleennfarction and review the literature concerning spleen in-olvement in WG.

ETHODS

n addition to the case presented, a literature searchPubMed) for articles published between 1966 and 2007as performed using the Medline subheadings keywords

Wegener granulomatosis” and “spleen.” The relevanteferences were retrieved. Additional references were

ound by checking those quoted in the papers retrieved.

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he available clinical, histological, and imaging data of allases were reviewed.

ASE REPORT

51-year-old woman was referred to the internal medi-ine ward because of persistent fever and dry cough thatad not responded to ceftibuten treatment. A chest radio-raph showed shadowing of the lung bases and of theight medium lobe as well as a pleural effusion. Past med-cal history was unremarkable. The patient was treatedmpirically with various antibiotics (azithromycin, ce-hotaxime, levofloxacin, ceftriaxone, and teichoplanin),ut she did not improve clinically. A repeat chest radio-raph demonstrated worsening of the lung lesions. Aomputerized tomography (CT) scan of the lung was thuserformed, which showed widespread parenchymal shad-wing with some bronchograms within areas of consoli-ation and multiple cavities of variable dimensions rang-

ng from a few millimeters to a few centimeters. A bilateralleural effusion was also observed.Incidentally, diffuse low-signal intensity of the spleen

arenchyma with a normal appearance of the capsule wasoted on the caudal thoracic CT slides. Splenic infarctionas suspected and a CT scan of the abdomen was ar-

anged, which confirmed a diffuse low-signal intensity ofhe spleen parenchyma judged to be consistent withplenic infarction (Fig. 1). Septic embolism was ruled outy echocardiography.The patient was referred to the Department of Chestedicine where pleural fluid aspiration yielded 350 mL

f straw-colored yellow fluid; cytology and microbiologyests were all negative. Because the patient also had evi-ence of rhinitis, a second consultation with an ear, nose,nd throat specialist was sought. Examination revealedrusting rhinitis, mucosal hyperemia with nearly com-lete obstruction of the paranasal sinus ostia, while histol-gy of the nasal cavities demonstrated chronic necrotizing

igure 1 Postcontrast computed tomography (CT) axial im-ge of the upper abdomen shows diffuse and homogeneousypodensity in the spleen (white asterisk).

nflammation consistent with WG. t

Similarly, tissue samples obtained at bronchoscopyhowed evidence of necrotizing bronchitis with some gi-nt cells and large clusters of neutrophilic granulocytes inhe absence of granuloma or of neoplastic cells, againonsistent with a diagnosis of WG (Fig. 2A and B).

The patient was referred to our Rheumatology Depart-ent for further management. On admission, she was

ebrile with temperature spikes up to 39.5°C and short ofreath. Oxygen saturation was 94% despite oxygen ad-inistration at 4 L/min. Physical examination revealed

ecreased sounds at the lung bases particularly on theight and dependent edema, but normal heart sounds andssentially normal abdominal findings.

Laboratory tests showed raised inflammatory markerserythrocyte sedimentation rate, 90 mm/1st hour; C-re-ctive protein, 18.6 mg/dL) and chronic inflammatory-ype anemia (hemoglobin, 9.9 g/dL, with a mean corpus-ular volume of 85 fl). c-ANCA (antiproteinase-3ntibody) tested positive with a titer of 33 EU/mL (nor-al values �5.9) on enzyme-linked immunosorbent as-

ay. Serum creatinine was elevated and progressively ris-ng (initially 3.2 mg/dL, subsequently 4.5 mg/dL), whilerinalysis was positive for blood (hemoglobin �1.00 mg/L) and protein (50 mg/dL).WG-associated rapidly progressive glomerulonephritis

as diagnosed on the basis of the following: progressivelyncreasing serum creatinine levels with an estimated cre-tinine clearance of 22 mL/min (calculated using Cock-roft and Gault’s formula) (3); a reduction in urine out-ut with development of dependent edema and weightain of 8 kg within a week; 24-hour urinary protein ele-ated at 1122 mg; and evidence on contrast phase micros-opy of urinary sediment of predominantly eumorphicie, of renal origin) red blood cells.

The patient was treated aggressively with 3 pulses ofntravenous methylprednisolone (1 g each) followed byrednisone 75 mg daily in combination with cyclophos-hamide with a loading dose of 4 mg/kg/d for 3 days,ubsequently tapered to 2 mg/kg/d and intravenous furo-emide (250 mg daily). On this regimen, urinary outputncreased to 2300 mL daily with a net weight loss of 6 kgithin a week, while creatinine values decreased to 3.4g/dL and her shortness of breath improved. However, 8

ays following the onset of the above treatment, the pa-ient had an episode of bleeding through the mouth,hich we interpreted as suspicious for nonmassive he-optysis. Her hemoglobin dropped to 7.5 g/dL; a chest

adiograph showed a reduction in the pleural effusion butore widespread bilateral medio-basal shadowing, whilepulmonary CT scan revealed diffuse parenchymal shad-wing with a “ground glass” appearance and circum-cribed areas of parenchymal consolidation with air bron-hograms.

Pulmonary hemorrhage was diagnosed and the pa-ient was referred to the Department of Chest Medi-ine where she received blood transfusions and paren-

eral nutrition. She was also started on noninvasive

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330 Wegener granulomatosis with spleen infarction

entilation (initially continuous positive airway pressureentilation with a helmet, subsequently positive end ex-iratory pressure ventilation). However, her conditioneteriorated further, with rising creatinine levels (4.9 mg/L) and decreasing urinary output (1000 mL/d), whichequired dialysis therapy. She eventually developed severeypotension with markedly decreased oxygen saturationnd died despite aggressive treatment with adrenalin, at-opin, intubation, and ventilatory support.

ISCUSSION

o date, spleen involvement has only been reported in aew patients of WG, with a range of abnormalities thatnclude splenomegaly, capsular adhesions, dysfunction,nd infarction (4). However, the limited number of au-opsy studies available have shown a high percentage (78o 100%) of spleen lesions, such as vasculitis, granulomaormation, necrosis, and capsulitis (5-7), suggesting thatsymptomatic spleen involvement may be quite common.n fact, 2 of the 3 patients described by Wegener in 1936ad evidence of splenic lesions (8). Spleen involvement

ncluding infarction in WG is often clinically silent, as inur patient, being usually detected as an incidental find-ng on investigations like ultrasound, scintigraphy, CTcan, or magnetic resonance imaging (MRI) (9-17). Inome cases, however, patients with spleen infarction mayresent with diffuse or left upper quadrant abdominalain referred to the left shoulder, fever, and blood test

igure 2 Necrotizing bronchitis with large clusters of neutroB, magnification, �200) in the absence of well-formed gran

bnormalities such as anemia, leukocytosis, and thrombo- b

ytosis. Table 1 summarizes the published reports onpleen infarction, while Table 2 summarizes the pub-ished reports on spleen involvement other than infarc-ion in WG.

Spleen infarction is not thought to be due to occlusionf the main splenic artery, because the distal part of thisrtery can be reconstituted by collateral vessels from othereliac branches. It may result from occlusion of the distalart of the splenic artery or of its branches because splenicarenchymal arteries are end vessels (17). Histologically,t is usually characterized by widespread or multiple areasf necrosis variably associated with central arteritis, trabe-ulitis, follicular arteriolitis, disseminated parenchymalranulomata, and capsulitis.

CT scan is very sensitive for depicting lesions in thepleen. Spleen infarctions classically appear as peripheral,edge-shaped areas of low attenuation. However, otheratterns have also been described, including multiple het-rogeneous low-attenuation regions, areas with normalnhancement centrally but with low peripheral attenua-ion and, as in our case, low-attenuation lesions with a rimf enhancing tissue. The latter lesion has been ascribed toesidual blood supply to the capsula and subcapsular tis-ue, whereas the appearance of small areas of enhance-ent within a hypodense area of infarction has been at-

ributed to the presence of patent vessels that may allowmall areas of parenchyma to be spared (9,10). While thelassical wedge-shaped infarctions are usually due to em-

granulocytes (A, magnification, �100) and some giant cellsas (H&E stain). (Color version of figure is available online.)

philic

olic causes, the arteritis of WG is more likely to result in

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iffuse involvement, as in our patient. Splenic infarctionue to polyarteritis nodosa has an appearance similar tohat observed in our case and in a previously published (9)ase of WG.

MRI with gadolinium enhancement has also been suc-essfully used to document diffuse splenic infarction in a

Table 1 Published Reports on Splenic Infarction in Wegene

Reference, YearNo. ofCases Abdominal Symptoms or Sign

(5), 1954 17 Not reported(27), 1986 1 Absent(13), 1986 1 Not reported(28), 1989 1 Not reported(29), 1990 2 Abdominal pain

(30), 1991 1 Absent

(11), 1994 1 Sharp pain in the left hypochond

(9), 1995 1 Absent

(31), 1996 2 1: Absent2: Vomiting and abdominal pain

(32), 1996 1 Absent

(12), 1998 1 Deep left upper quadrant pain

(33), 1999 1 Absent(22), 1999 2 1: Absent

2: Left hypocondral tenderness(34), 1999 1 Epigastric abdominal pain radiatin

to the left hypocondrium(16), 2000 1 Absent

(26), 2000 1 Abdominal painMild right upper quadrant tender

(35), 2002 1 Absent

atient with WG (12). On MRI, there was diffuse low- a

ignal intensity on T1-weighted sequences with a rim ofnhancement, while T2-weighted sequences demon-trated an area of very high-signal intensity surrounded byrim of intermediate intensity.Ultrasound can show a number of splenic lesions, in-

luding wedge-shaped or round hypoechoic or echo-free

ulomatosis

Comments

Autopsy findingAutopsy findingNecrosis of the spleen except for a medial rim (CT).Autopsy finding1: intrasplenic nodules (US, confirmed by MRI)2: enlarged spleen with multiple hypodense areas (CT)Splenic inhomogeneity with patency of the splenic

artery and vein (US)Lobulation and shrinkage of the spleen with return to

normal attenuation (CT)Diffusely heterogeneous spleen appearance (US)Multiple curly hypodense areas within the spleen, patent

blood vessels (CT)Large region of predominantly low attenuation centrally,

a rim of normally enhancing tissue peripherally; withinthe low-attenuation region, small scattered focal areasof increased attenuation appeared to surroundbranches of the splenic artery (CT)

Autopsy finding

Inhomogeneous and hypodense spleen with nodularand cystic areas (CT)

Granulomatous necrotizing vasculitis found on histologicstudy at biopsy after splenectomy

Slightly enlarged spleen with widespread abnormalechogenicity (US)

Diffuse low signal intensity of the splenic parenchyma,on T1-weighted images, with a thin rim ofenhancement; on T2-weighted, very high signalintensity of the organ, while an irregular peripheralportion had mixed intermediate and high signalintensity (MRI)

Heterogeneous density consistent with infarction (CT)1: Voluminous splenic hematoma (CT)2: Splenic infarction and hematoma (CT)Hypodense area occupying 80% of the spleen due to an

infarct (CT)Intrasplenic inhomogeneous area with a stop of blood

flow in the lienalis artery and no parenchymalperfusion (US)

Splenic infarction with remnant capsular blood supplyvia extrasplenic vessels (CT)

Splenomegaly with predominantly low-attenuationareas; higher attenuation areas in the splenic hilumand capsule (CT)

Well-defined areas of low attenuation within the spleen,consistent with infarction, with small areas ofenhancement within larger hypodense lesions (CT)

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nfarction (16). Standard ultrasound combined with du-lex ultrasonography of splenic blood vessels may be par-icularly helpful in defining infarction by showing a vas-ular filling defect. On a note of caution, it should beorne in mind that the appearance of a splenic infarctionay closely mimic other conditions such as abscess, neo-

lasm, and hematoma not only on ultrasonography, butlso on CT scan and MRI (12). Therefore, a full clinicalvaluation should be performed in addition to imagingtudies to arrive at a confident diagnosis.

Rarely, 111Indium scintigraphy has been used to inves-igate spleen involvement in WG. With specific regard topleen infarction, ischemic spleen areas are characterizedy decreased tracer uptake (14,15).Although Howell-Jolly bodies were absent in our pa-

ient, their presence may suggest splenic infarction. How-ver, these bodies are not a very sensitive sign, particularlyn cases of mild hyposplenism (18,19).

In some patients, splenic infarction may lead to com-lications such as abscess, bleeding, or even spontaneousupture (20-23). In addition, splenic infarction resultingn functional hyposplenism has been linked to an in-reased risk of developing serious infections with encap-ulated and intraerythrocytic organisms, with an esti-ated incidence of 7 cases per 1000 person-years (24).his risk increases with the use of immunosuppressiverugs (24-26). To lower the risk of such infections, allatients should receive pneumococcal, meningococcal,aemophilus influenza type B, and annual influenza vac-ination (25). Other strategies include patient education,mmunoprophylaxis, and chemoprophylaxis (25).

Infarction of solid organs other than the spleen havelso been reported in WG but appear to occur less fre-

Table 2 Published Reports on Spleen Involvement other th

Reference, YearNo. ofCases Abdominal Symptoms or Signs

(5), 1954 28 Not reported

(7), 1983 6 Not reported(36), 1991 1 Not reported(20), 1993 1 Acute abdominal pain

(31), 1996 5 1: Abdominal pain

2: Absent

3: Vomiting and abdominal pain

4: Absent

5: Absent

(21), 1998 1 Absent(23), 2002 1 Painless abdominal swelling

uently. The reason for the particular susceptibility of the 1

pleen to infarction has not been fully elucidated (26).he frequency of splenic involvement, particularly infarc-

ion, is probably underestimated in WG. Therefore, aigh index of suspicion should be maintained and appro-riate tests ordered to document splenic lesions.

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arction in Wegener Granulomatosis

Histological Findings

omegaly with capsular adhesions, granulomatosis lesions,ular involvement (arteriolitis, trabeculitis, capsulitis,cular necrosis, etc)omegaly; splenic vasculitistizing granulomasophilic infiltration at the site of splenic tear and in thecapsular zone without splenic arteritisenomegaly, necrotizing granuloma, fibrinoid necrosis ofd vessels, infarction, thrombosis, congestion

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enomegaly, vascular congestion, extramedullaryatopoiesisminent vascular hyalinization, vascular congestion, mildosiderin deposition

al arteritis and segmental necrosisic rupture (CT); histologically normal

an Inf

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2

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2

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A. Ghinoi et al. 333

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