[Diffuse panbronchiolitis with myeloperoxidase-specific antineutrophil cytoplasmic antibody-related vasculitis].
A 46-year-old woman was referred to our department in July 1996 with complaints of fever and myalgia in her calves. She had a 20-year history of purulent sputum; diffuse panbronchiolitis had been diagnosed in 1983. Physical examination revealed low-pithed rhonchi over the lung fieldis and hypesthesia of the right leg. She had a white blood cell count of 16,100/mm3, including 4% eosinophils, and a platelet count of 80.0 x 10(4)/mm3. The serum IgE level was 2,200 U/ml, and the cold hemagglutinin titer was high. Pulmonary-function tests showed mixed ventilatory dysfunction, and arterial blood gas analysis revealed a PaO2 of 55.8 Torr on room air. Pseudomonas aeruginosa was cultured from her sputum. A chest X-ray film and CT scan showed diffuse nodular shadows and bronchiectatic changes with mild hyperinflation. An infiltrative lesion in right S6 area could also be seen. Administration of broad-spectrum antibiotics did not alleviate her symptoms. The level of myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA) in serum was 245 EU/ml, and 67Ga scintigraphy showed marked accumulation in the abdomen. Abdominal angiography demonstrated a bead-like appearance and irregularities in the peripheral branches of the hapatic artery, the splenic artery, the cystic artery, and the superior mesenteric artery. Because of the high MPO-ANCA level and the angiographic abnormalities, MPO-ANCA-related vasculitis was diagnosed. She was treated with 1 g of methylprednisolone daily for 3 days, followed by 60 mg of prednisolone and 50 mg of cyclophosphamide daily. Her condition improved dramatically, and the MPO-ANCA level became almost normal. During treatment, her blood pressure rose markedly with a normal serum creatinine level and normal urinalysis. Plasma renin activity was 13.3 ng/ml/hr. Renal angiography showed stenoses and irregularities in the peripheral branches of renal arteries bilaterally. These findings led to a diagnosis of renovascular hypertension due to vasculitis. Her blood pressure was controlled with an angiotensin-converting enzyme inhibitor and a calcium antagonist. Vasculitis associated with chronic supportive lung disease has occasionally been reported, which suggests a casual relation between chronic respiratory infection and ANCA-related vasculitis. Systemic vasculitis should be taken into account as a potential complication of chronic suppurative lung disease.
Miyamoto
D
D
First Department of Internal Medicine, Tokyo Medical College, Japan.
Ichinose
Y
Y
Kikawada
M
M
Kusumoto
H
H
Yanagisawa
N
N
Kanai
E
E
Minemura
K
K
Yonemaru
M
M
Toyama
K
K
D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis*; D001988:Bronchiolitis / Q000150:complications* / Q000188:drug therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D006978:Hypertension, Renovascular / Q000188:drug therapy / Q000209:etiology; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008875:Middle Aged; D009195:Peroxidase / Q000276:immunology*; D011239:Prednisolone / Q000008:administration & dosage; D014657:Vasculitis / Q000188:drug therapy / Q000209:etiology*
D000893:Anti-Inflammatory Agents; D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone; D009195:Peroxidase