Review of literature of previously described cases of WAS with large vessel vasculopathy
| Study (year) | No. of patients | Age at diagnosis | Age at developing vasculitis | Symptoms and signs | Type of vasculitis | Infections | Treatment | Outcome | Relapse |
|---|---|---|---|---|---|---|---|---|---|
| Ilowite et al. (1986) (4) | 1 | 9 mo | 12 years | Fever, chest pain, dyspnea, differential pulses | Lymphomatoid granulomatosis pulmonary vasculitis, calcified aortic ring, mitral and aortic regurgitation, partially obstructed left subclavian artery | EBV | Methylprednisolone, gentamycin, acyclovir, oral cyclophosphamide | Death after 2 years following pneumonia | |
| Lau et al. (1992) (5) | 1 | 4.5 years | 5.5 years | Abdominal pain, hypertension, hypertensive encephalopathy, differential renal sizes | Abdominal aortic aneurysm, stenosis of bilateral renal arteries | Nil | Renal autotransplant | Death at 5.5 years due to ventricular arrhythmia | |
| Son et al. (1995) (6) | 1 | – | 23 years | Incidentally detected mediastinal widening in a chest roentgenogram | Aortic insufficiency, ascending and descending aorta, t horacic dilatation | Nil | Surgical repair | Recovered | Relapse at 9-year follow-up |
| Cluggage et al. (1999) (7) | 1 | – | 24 years | Abdominal pain, melena, hematemesis, hypertension, gastrointestinal hemorrhage | Multiple aneurysms from distal branches of right and left hepatic arteries, superior mesenteric artery, perirenal hematoma | Nil | Nephrectomy | Recovered, well after 7 wk | |
| Johnston et al. (2001) (8) | 1 | 6 years | 17 years | Signs of aortic regurgitation | Dilatation of the ascending aorta, aortic arch, and proximal descending aorta | Nil | Surgical repair | Recovered, well after 2 years of follow-up | |
| Narayan et al. (2004) (9) | 1 | 6 years | 24 years | Shortness of breath | Severe aortic regurgitation, dilatation of the aortic root and ascending aorta | Nil | Surgical repair | Recovered, well in 1-year follow-up | At 3 years, requiring second surgery |
| Bernabeau et al. (2007) (10) | 1 | 15 years | 33 years | Incidentally detected mediastinal widening in a chest roentgenogram | Thoracic aortic aneurysm involving ascending aorta, arch, and descending thoracic aorta | Nil | Surgical repair | Recovered, well in 10-mo follow-up | |
| Faganello 2008 (11) | 1 | NA | 27 years | – | Aortic root, ascending aorta | NA | Two-staged surgical repair | Developed aortobronchial fistula and massive hemoptysis and succumbed | |
| Ono et al. (2009) (12) | 1 | 5 years | 7 years | Incidentally detected in a routine chest roentgenogram | Ascending aortic aneurysm and moderate aortic regurgitation | Nil | Surgical repair | Recovered, well at 5-mo follow-up | |
| Pellier et al. (2010) (13) | 5 | | Mean age: 12.6 years | 4 asymptomatic 1 acute chest pain | Case 1: arch and descending thoracic aorta Case 2: descending thoracic aorta Case 3: abdominal aorta Case 4: descending thoracic aorta and another 3 cm lower of aortic wall Case 5: ascending thoracic aorta | Case 5: VZV, EBV, and HHV-6 in the aortic material | Case 1: HSCT Case 2: HSCT Case 3: nil Case 4: nil Case 5: surgical repair, steroids and methotrexate for 18 mo | Case 1: progression of aneurysm, asymptomatic after 5 years Case 2: death due to herpes simplex viral encephalitis after 4 years of diagnosis Case 3: died sue to lymphoma after 2 years Case 4: asymptomatic, aneurysm size stable over 12 years of follow-up Case 5: dysplastic aorta without clinical symptoms at 6 years of follow-up | |
| Önalan et al. (2018) and Önalan et al. (2023) (14, 15) | 1 | 10 years | 12 years | Chest pain and dyspnea | Descending aorta at 12 years of age At 21 years (9 years following HSCT), ascending aortic aneurysm with aortic valve insufficiency | Nil presence of an aortitis with granulomatous inflammatory response and multinucleated cells | Surgical repair | Ascending aortic aneurysm at 21 years, surgically operated Alive at 21 years of age | Nil |
| Study (year) | No. of patients | Age at diagnosis | Age at developing vasculitis | Symptoms and signs | Type of vasculitis | Infections | Treatment | Outcome | Relapse |
|---|---|---|---|---|---|---|---|---|---|
| Ilowite et al. (1986) ( | 1 | 9 mo | 12 years | Fever, chest pain, dyspnea, differential pulses | Lymphomatoid granulomatosis pulmonary vasculitis, calcified aortic ring, mitral and aortic regurgitation, partially obstructed left subclavian artery | EBV | Methylprednisolone, gentamycin, acyclovir, oral cyclophosphamide | Death after 2 years following pneumonia | |
| Lau et al. (1992) ( | 1 | 4.5 years | 5.5 years | Abdominal pain, hypertension, hypertensive encephalopathy, differential renal sizes | Abdominal aortic aneurysm, stenosis of bilateral renal arteries | Nil | Renal autotransplant | Death at 5.5 years due to ventricular arrhythmia | |
| Son et al. (1995) ( | 1 | – | 23 years | Incidentally detected mediastinal widening in a chest roentgenogram | Aortic insufficiency, ascending and descending aorta, t | Nil | Surgical repair | Recovered | Relapse at 9-year follow-up |
| Cluggage et al. (1999) ( | 1 | – | 24 years | Abdominal pain, melena, hematemesis, hypertension, gastrointestinal hemorrhage | Multiple aneurysms from distal branches of right and left hepatic arteries, superior mesenteric artery, perirenal hematoma | Nil | Nephrectomy | Recovered, well after 7 wk | |
| Johnston et al. (2001) ( | 1 | 6 years | 17 years | Signs of aortic regurgitation | Dilatation of the ascending aorta, aortic arch, and proximal descending aorta | Nil | Surgical repair | Recovered, well after 2 years of follow-up | |
| Narayan et al. (2004) ( | 1 | 6 years | 24 years | Shortness of breath | Severe aortic regurgitation, dilatation of the aortic root and ascending aorta | Nil | Surgical repair | Recovered, well in 1-year follow-up | At 3 years, requiring second surgery |
| Bernabeau et al. (2007) ( | 1 | 15 years | 33 years | Incidentally detected mediastinal widening in a chest roentgenogram | Thoracic aortic aneurysm involving ascending aorta, arch, and descending thoracic aorta | Nil | Surgical repair | Recovered, well in 10-mo follow-up | |
| Faganello 2008 ( | 1 | NA | 27 years | – | Aortic root, ascending aorta | NA | Two-staged surgical repair | Developed aortobronchial fistula and massive hemoptysis and succumbed | |
| Ono et al. (2009) ( | 1 | 5 years | 7 years | Incidentally detected in a routine chest roentgenogram | Ascending aortic aneurysm and moderate aortic regurgitation | Nil | Surgical repair | Recovered, well at 5-mo follow-up | |
| Pellier et al. (2010) ( | 5 | | Mean age: 12.6 years | 4 asymptomatic | Case 1: arch and descending thoracic aorta | Case 5: VZV, EBV, and HHV-6 in the aortic material | Case 1: HSCT | Case 1: progression of aneurysm, asymptomatic after 5 years | |
| Önalan et al. (2018) and Önalan et al. (2023) ( | 1 | 10 years | 12 years | Chest pain and dyspnea | Descending aorta at 12 years of age | Nil presence of an aortitis with granulomatous inflammatory response and multinucleated cells | Surgical repair | Ascending aortic aneurysm at 21 years, surgically operated | Nil |
EBV, Epstein-Barr virus; VZV, varicella-zoster virus; HHV-6, human herpesvirus 6; HSCT, hematopoietic stem cell transplantation.