1, female* | WG diagnosed age 44 Respiratory, joint, renal Cyclophosphamide, corticosteroids | cANCA | R RAS diagnosed 4 years after vasculitis | Positive lupus anticoagulant on more than two occasions Cardiac valve lesions, renal thrombotic microangiopathy |
2, male | Churg-Strauss diagnosed age 46 Cerebral vasculitis, cardiomyopathy, asthma, sinusitis, necrotising glomerulonephritis Corticosteroids, azathioprine, methotrexate, cyclophosphamide | pANCA | L RAS diagnosed 18 years after vasculitis | Positive lupus anticoagulant on two occasions 1 Cerebrovascular accident 1 Deep vein thrombosis |
3, female | WG diagnosed age 47 Pulmonary haemorrhage, crescentic glomerulonephritis, peripheral neuropathy Azathioprine, cyclophosphamide, co-trimoxazole | cANCA | Bilateral RAS and coeliac artery stenosis diagnosed 7 years after onset of vasculitis | Positive lupus anticoagulant and anticardiolipin antibodies on two occasions Miscarriages, superficial thrombophlebitis |
4, male | WG diagnosed age 56 Pulmonary haemorrhage, epistaxis, and scleritis Methotrexate, cyclophosphamide, prednisolone, co-trimoxazole | cANCA | L RAS, superior mesenteric artery stenosis diagnosed 4 years after onset of vasculitis | Lupus anticoagulant positive on two occasions No thromboses |
5, female | Primary idiopathic systemic vasculitis diagnosed age 42 Inflammatory arthropathy, coronary arteritis Azathioprine, methotrexate, prednisolone | Not present | Bilateral RAS diagnosed 15 years after onset of vasculitis | Positive lupus anticoagulant on two occasions |