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Table 1 Summary of reported EGPA patients with occlusion of arteries in the extremities

From: A delayed diagnosis of eosinophilic granulomatosis with polyangiitis complicated with extensive artery occlusion of lower extremities in children: case report and literature review

Author, year Braunberger T, 2016 Waseda K, 2011 Otani Y, 2003 Inui S, 2001 Abbas MA, 2000 Present study, 2018
Country USA Japan Japan Japan UK China
Sex Male Female Male Male Female Female
Age, years 72 37 59 69 49 10.8
Clinical manifestations
 Asthma +a + + + + +
 Eosinophilia -b + + + + +
 Neuropathy + + + + +
 Pulmonary + +
 Skin lesions + + + +
Extrapulmonary Weight loss, fever Fever Liver infarction Atrial fibrillation, fatigue, fever, weight loss Microhaematuria, proteinuria, arthralgia, Arthralgia and myalgia
Peak level of eosinophils Normal 1.9 × 109/L 12.9 × 109/L 15.8 × 109/L 13.1 × 109/L 7.3 × 109/L
ANCAs Negative Negative Negative Negative Negative Negative
Presentation of vasculitis Occluded right anterior tibial artery Narrowing of the dorsalis pedis artery and of the peripheral arteries of her right leg Narrowing of the main arteries in the forearms and lower legs; narrowing and occlusion of the hepatic vein Stenosis of the medium sized arteries in both legs Digital vasculitis affecting the left index and middle finger Extensive stenosis and/or occlusion of shank and foot arteries with formation of collateral vessels
Treatment CTX, methylprednisolone, maintaining with prednisone Prednisolone, CSA, CTX, Antithrombotic agents, azathioprine Prednisolone, intravenous PG1 Prednisolone Methylprednisolone, CTX Methylprednisolone maintain with prednisone, methotrexate, antithrombotic agents
Prognosis Right partial second toe amputation, alive Plastic surgery, alive Amputate, alive Alive Alive Alive
  1. Abbreviations: ANCAs antineutrophil cytoplasmic antibodies, including p-ANCA and c-ANCA, CSA cyclosporin A, CTX cyclophosphamide, PG1 prostaglandin E1
  2. aPositive, bNegative