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KMID : 0882419930440020263
Korean Journal of Medicine
1993 Volume.44 No. 2 p.263 ~ p.272
Anti-Neutrophil Cytoplasmic Antibodies(ANCA) Positive Wegener's Granulomatosis(WG) Mimicking Goodpasture's Syndrome
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À̼öÇü/¹Ú¼º¹è/±èÇöö/¹Ú°ü±Ô/ÀÌ»ó¼÷
Abstract
G is a syndrome characterized by necrotizing granulomatous lesions in the upper and
lower respiratory tracts, glomerulonephritis, and a generalized vasculitis involving both arteries
and vein. Recently ANCA has been reported to be a highly specific test for the diagnosis of
WG. We have experienced three patients with ANCA positive WG whose initial clinical
presentation mimicked Goodpasture's syndrome. All three patients were female with mean
age of 30 years (16-53). The major presenting signs and symptoms were hemoptysis, fever,
general weakness, gastrointestinal symptoms, and conjunctivitis. Persisten rhinorrhea was
seen in only one patient. All patients had lung, renal, and eye involvement, but none of the
patients had signs of ear, heart or nervous system involvement. All three patients had
moderate anemia, elevated ESR and urinary abnormality.
One patient had leukopenia, and no patient had thrombocytopenia. All three patients were
positive for rheumatoid factor and ANCA. None were positive for antinulear antibody,
anti-GBM antibody, or cryoglobulin. Serum complement levels were normal in all three
cases. Radiographic studies of chest shouwed multiple bilateral patches and nodular
infilerates in all three patients. Cavitation was seen in one case and pleural effusionn in one
case. Two patients were treated with steroid pulse therapy, one of them showed dramatic
improvement, the other patient expired due to progression of underlying illness. One patient
whose renal function was normal at the time of admission recovered, but the other two
patients who required hemodialysis treatment expired.
In conclusion, earlier diagnosis of WG facilitated by ANCA tests may help reduce the
higher morbidity and mortality seen in this type of patient.
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