Table 1 (abstract P371). See text for description
From: Proceedings of the 28th European Paediatric Rheumatology Congress (PReS 2022)
Mean Age at Onset | 8.2 years |
Female | 14 |
Males | 6 |
Predominantly Large Vessel Vasculitis – Takayasu Aortoarteritis | 6/20(30%) |
Predominantly Medium Vessel Vasculitis – Polyarteritis Nodosa | 8/20(40%) |
(Includes Monogenic Vasculitis – DADA 2 syndrome) | 6 |
Predominantly Small Vessel Vasculitis – Granulomatosis with Polyangiitis (GPA) | 4/20(20%) |
Isolated CNS Angiitis | 2/20(10%) |
Fever at Diagnosis | 16/20(80%) |
Constitutional symptoms including weight loss | 14/20(70%) |
Hypertension | 12/20(60%) |
Central Nervous System | |
 Stroke | 5/20(25%) |
 PRES | 5/20 (25%) |
 Peripheral neuropathy | 6/20(30%) |
 Cranial Neuropathy | 2/20(10%) |
 Neuropsychiatric Manifestations | 1/20(5%) |
Respiratory System | |
 ENT Disease | 3/20(15%) |
 Diffuse alveolar haemorrhage | 1/20(5%) |
 Pulmonary Hypertension | 1/20(5%) |
Cardiovascular system | |
 Left Ventricular Dysfunction | 3/20(15%) |
 Coronary Aneurysms | 1/20(5%) |
Gastrointestinal system | |
 Severe abdominal pain | 10/20(50%) |
 GI Ulcers | 5/20 (25%) |
 GI Perforation | 3/20(15%) |
Renal System | |
 Perfusion or functional defect | 8/20(40%) |
 Nephritis | 3/20(15%) |
Musculoskeletal system | |
 Myalgia or myositis | 3/20(15%) |
 Arthralgia or arthritis | 4/20(20%) |
Skin involvement | |
 Livedo reticularis | 6/20(30%) |
 Nodular skin lesions | 4/20(20%) |
 Vasculitic Rash | 4/20(20%) |
 Other Rash | 2/20(10%) |
Eye involvement | |
 Hypertensive Retinopathy | 2/20(10%) |
 Scleritis | 1/20(5%) |
 Ptosis | 1/20(5%) |
Elevated CRP +/- ESR at onset | 14/20(70%) |
Evidence of Vasculitis by | |
 MR Angiography | 7/20(35%) |
 CT Angiography | 8/20(40%) |
 PET | 2/20(10%) |
 Biopsy | 4/20(20%) |
Response to cDMARD | |
 Intravenous Cyclophosphamide | 9/12(75%) |
 Methotrexate | 0/5(0%) |
 Oral Azathioprine | 11/15(73%) |
 Oral MMF | 1/3(33%) |
Response to bDMARD | |
 Intravenous Rituximab | 1/1(100%) |
 Subcutaneous Adalimumab | 0/2(0%) |
 Intravenous Infliximab | 0/2(0%) |
 Intravenous Tocilizumab | 3/3(100%) |
Follow Up Details | |
 Inactive disease off steroids | 10/20(50%) |
 Inactive disease on minimum steroids | 4/20(20%) |
 Persistent low disease activity | 2/20(10%) |
 Persistent disease activity and damage | 2/20(10%) |
 Death | 2/20(10%) |