Table 1 Characteristics of patients with aortitis and aortic dissection due to granulomatosis with polyangiitis
First author /year | Gender/Age (y) | Country | Location | Manifestation | Antibodies | Aneurysmal symptoms | Duration of aneurysmal symptoms | Aneurysmal symptoms from GPA diagnosis | Therapy | Rupture | Outcome |
|---|---|---|---|---|---|---|---|---|---|---|---|
Sieber/ 1990 | Male/59 | American | Aorta | Aortic aneurysm | c-ANCA positive | Abdominal pain | 9 months | 9 months before | Coronary artery bypass + steroid pulse + PSL + CY 3 mg/kg + plasmapheresis | No | Good |
Fink/1994 | Male/45 | United Kingdom | Aorta | Aortitis | ANCA positive | Malaise and intermittent right abdominal pain | 5 months | 5 months after | Right ureterolysis + immunosuppressive therapy | No | Good |
Blockmans/2000 | Male/42 | Belgium | Aorta | Aortic aneurysm + aortic dissection | c-ANCA 1: 1280, Antiproteinase-3157 AU/l | Abdominal pain | 1 week | 3 weeks after | Aortoiliac graft and high-dose steroids + CY 2 mg/kg/day | No | Good |
Chirinos/2004 | Female/50 | Spain | Thoracic aorta | Thoracic aortic aneurysm | p-ANCA (1: 320) antimyeloperoxidase 440 U/ml | Abdominal pain with radiation to the back | 2 weeks | Concomitant | mPSL pulse CY | Yes | Death |
Carels/2005 | Male/63 | Belgium | Abdominal aorta | Abdominal aortic aneurysm | p-ANCA 1:80 and MPO 28 U/l | Low back pain, fever | 2 months | Concomitant | Surgery +PSL 1 mg/kg + CY 2 mg/kg | No | Good |
Minnee/2009 | Male/51 | Netherlands | Aorta | Aortic aneurysm | ANCA positive, anti-proteinase-3 antibodies > 530 kU/L | Lower back pain | 2 months | Concomitant | Steroid pulse + PSL 1 mg/kg + CY 2 mg/kg/day | No | Good |
Durai/2009 | Male/33 | Netherlands | Abdominal aorta | Abdominal aortic aneurysm | Antiproteinase-3 positive (> 1/10) | Abdominal discomfort in the upper abdomen | 3 weeks | 3 weeks after | Internal jugular vein graft + PSL+ CY | No | Good |
Unlü/2011 | Male/43 | Netherlands | Infrarenal aorta | Infrarenal aortitis with aneurysm | NS | Abdominal pain and generalized malaise | 1 week | 11 years after | PSL + surgery | No | Good |
Toda /2011 | Male/79 | Japan | Thoracic aorta | thoracic aortic aneurysm | Proteinase 3-ANCA 1180 EU | Back pain | 8 months | 8 months after | Steroid + PSL 60 mg + intravenous CY 300 mg | Yes | Death |
Amos /2012 | Male/64 | Greek | Aorta | Aortitis | ANCA positive | Fever, intermittent pleuritic chest pain | 4 weeks | Concomitant | CY 1.5 mg/kg/day and intravenous mPSL 500 mg/day | No | Good |
Ohta /2013 | Male/38 | Japan | Thoracic aorta | Thoracic aortic aneurysm | ANCA positive(× 128) | Back pain and lossofconsciousness | NS | 22 years after | Surgery (J-graft) + PSL 15 mg/day | Yes | Good |
Miyawaki/2017 | Male/60 | Japan | Aorta | Aortitis | Proteinase 3-ANCA 1500 U/mL | Fever and cough | NS | 26 years after | mPSL 1 mg/kg daily and CY 2 mg/kg daily | No | Good |
Niimi/2018 | Male/57 | Japan | Abdominal aorta | Abdominal aortic aneurysm | Proteinase 3-ANCA 187 IU/ml | Back pain and fever | 1 month | Concomitant | Steroid + PSL 55 mg + intravenous CY 500 mg | No | Good |
Kim /2018 | Male/58 | South Korea | Ascending arta | Aortitis and intramural hematoma | ANCA positive | Mid-sternal pain | NS | Concomitant | steroid treatment | No | Good |
Parperis/2019 | Female/71 | Spain | Aorta | Aortitis | p-ANCA positive, MPO antibody 159 | Chronic headache | 20 years | 20 years after | PSL 60 mg daily and methotrexate 20 mg weekly with folic acid 1 mg daily | No | Good |
Present case | Male/28 | China | Aorta | Aortitis and aortic dissection | c-ANCA positive | Chest pain | 4 h | 1 years after | Aortoiliac graft + mPSL 40 mg twice daily and CY 2 mg/kg daily | No | Good |