Case Report

Aortic Aneurysm as a Complication of Granulomatosis with Polyangiitis Successfully Treated with Prednisolone and Cyclophosphamide: A Case Report and Review of the Literature

Table 1

Case of aortic involvement in granulomatosis with polyangiitis.

CaseAge/gender (years)Affected siteAntibodiesDuration of complaintTreatmentRuptureOutcome

1 [1]38/MTAAAnti-neutrophil cytoplasmic antibodies × 128NSSurgery (J-graft) + PSL 15 mg/dayYesGood
2 [13]51/MDistal part of aorta (3.8 cm)Anti-proteinase-3 antibodies > 530 kU/L2 monthsSteroid pulse + PSL 1 mg/kg + CY 2 mg/kg/dayNoGood
3 [14]45/MAorta, extending to the right iliac arteryANCA + NS5 daysRight ureterolysis + immunosuppressive therapyNoGood
4 [15]33/MAAAAntiproteinase-3 (>1/10)3 weeksIJV graft + PSL + CYNoGood
5 [16]42/MAAAAntiproteinase-3 157 AU/l1 monthAortoiliac graft and high-dose PSL + CY 2 mg/kg/dayNoGood
6 [17]63/MAAA (5.4 cm)p-ANCA 1/80 and MPO 28 U/l2 monthsSurgery + PSL 1 mg/kg + CY 2 mg/kgNoGood
7 [18]50/FTAAp-ANCA (1 : 320) antimyeloperoxidase 440 U/ml2 monthsPSL + CYYesDeath
8 [19]43/MInfrarenal aorta (3.1 × 3.5 cm)NS1 weekPSL + surgeryNoGood
9 [20]29/MBranches of hepatic and renal arteriesAnti-PR3 ANCA 15 IU/mLNSCoil embolization + steroid pulse + PSL 60 mg + MMF 2.5 gNoGood
10 [21]34/MAnterior choroidal arteryPR3 ANCA 457 EU1 yearClipping + steroid pulse + PSL 40 mg + CYYesGood
11 [22]67/MSuperior pancreaticoduodenal arteryC-ANCA 1 : 512 PR3 ANCA 88 IU/L11 monthsIVCY (0.7 g/m2) every 3 weeks + steroid pulse + PSL 1 mg/kg/dayYesDeath
12 [23]58/FSubclavian aneurysmP-ANCA 68 units2 monthsPSL 1 mg/kg + CY 2 mg/kg + stent graftNoGood
13 [24]56/MLeft gastric arteryC-ANCA positive1 monthNoneYesDeath
14 [25]55/MHepatic arteryC-ANCA 1 : 803 weeksmPSL + CYYesDeath
15 [26]24/MBilateral renal arteryNS6 weeksPSL 30 mg + CY 150 mg/dayYesGood
16 [27]59/MAortaC-ANCA 158 SLI units9 monthsCoronary artery bypass + steroid pulse + PSL + CY 3 mg/kg + plasmapheresisNoGood
17 [28]35/MHepatic, renal, splanchnicC-ANCA positive6 weeksSteroid pulse + PSL + IVCY 750 mgYesGood
18 [29]30/MRenal arteryNS1 monthPSL 1 mg/kg/day and CY 2 mg/kg/dayNoGood
19 [30]53/FRenal arteryNS20 daysPSL 1 mg/kg/day + CY 2 mg/kg/day + hemodialysisNoGood
20 [30]79/MTAAPR3-ANCA 1180 EU8 monthsSteroid pulse PSL 60 mg + IVCY 300 mgYesDeath
Present case58/MAAAPR3 ANCA 187 IU/ml2 weeksSteroid pulse + PSL 55 mg + IVCY 500 mgNoGood

TAA: thoracic aortic aneurysm; AAA: abdominal aortic aneurysm; F: female; M: male; NS: not stated; PSL: prednisolone; CY: cyclophosphamide; mPSL: methylprednisolone; MMF: mycophenolate mofetil; IVCY: intravenous cyclophosphamide; IJV: internal jugular vein.