Eosinophilia and hyperimmunoglobulinemia E as the presenting manifestations of Wegener's granulomatosis.
Eosinophilia has long been known as a hallmark of Churg-Strauss syndrome but has rarely been reported in Wegener's granulomatosis (WG). Here we describe a patient with WG who had skin, kidney and lung involvement as well as striking peripheral eosinophilia and hyperimmunoglobulinaemia E (hyper-IgE). The patient's clinical picture was complicated by intra-alveolar haemorrhage resulting in severe anaemia and respiratory failure. The pulmonary symptoms recovered completely, but the renal involvement evolved into end-stage renal failure despite intensive immunosuppressive treatment, intravenous immunoglobulin and plasmapheresis. We suggest that the presence of eosinophilia and hyper-IgE might contribute to the development of different disease patterns in WG.
Kamali
S
S
Department of Internal Medicine, Division of Rheumatology, Istanbul University, Istanbul Medical Faculty, Istanbul, Turkey.
Kasapoglu
E
E
Aktürk
F
F
Gül
A
A
Inanc
M
M
Ocal
L
L
Aral
O
O
Konice
M
M
D000328:Adult; D001774:Blood Chemical Analysis; D003520:Cyclophosphamide / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007073:Immunoglobulin E / Q000378:metabolism; D007589:Job Syndrome / Q000175:diagnosis*; D008775:Methylprednisolone / Q000008:administration & dosage; D011657:Pulmonary Eosinophilia / Q000175:diagnosis*; D013902:Radiography, Thoracic; D018570:Risk Assessment; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D007073:Immunoglobulin E; D003520:Cyclophosphamide; D008775:Methylprednisolone