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

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

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

Abbreviations: ANCAs antineutrophil cytoplasmic antibodies, including p-ANCA and c-ANCA, CSA cyclosporin A, CTX cyclophosphamide, PG1 prostaglandin E1
^aPositive, ^bNegative

ISSN: 1546-0096