Atypical Wegener's granulomatosis with positive cytoplasmic antineutrophil cytoplasmic antibodies, ophthalmologic manifestations, and slowly progressive renal failure without respiratory tract involvement.
A 68-year-old woman had microscopic hematuria and proteinuria since the age of 50. She also had hearing impairment, arthralgia, retinal embolism, peripheral arterial occlusion of the right foot and chronic renal failure during the course. At the age of 68, she had progressive renal failure and nephrotic syndrome with high titers of serum cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA). No evidence of respiratory tract involvement was found. Methylprednisolone pulse therapy and low dose cyclophosphamide therapy ameliorated the renal failure and reduced the serum c-ANCA level. She, however, died on July 19, 1998 due to pulmonary fungal and pneumocystis carinii infection.
Kakizawa
T
T
Department of Geriatrics, Shinshu University School of Medicine, Matsumoto, Nagano.
Ichikawa
K
K
Yamauchi
K
K
Takeda
T
T
Nagai
M
M
Mori
J
J
Yumita
W
W
Miyamoto
T
T
Nagasawa
T
T
Hashizume
K
K
D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D003520:Cyclophosphamide / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000097:blood / Q000150:complications* / Q000175:diagnosis; D006311:Hearing Disorders / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008171:Lung Diseases / Q000209:etiology; D008775:Methylprednisolone / Q000627:therapeutic use; D051437:Renal Insufficiency / Q000209:etiology; D012164:Retinal Diseases / Q000209:etiology; D014657:Vasculitis / Q000209:etiology
D000893:Anti-Inflammatory Agents; D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone