A 60-year-old man had experienced
cough, bloody sputum, and a 38 °C
fever for 1.5 months. He visited an outpatient clinic and received
antibiotics and nonsteroidal anti-inflammatory drugs. However, because the symptoms continued, he visited our hospital. The past medical history included chronic
sinusitis,
hypertension, and
diabetes mellitus. A chest
x-ray film and computed tomography showed
multiple pulmonary nodules with cavities. Macrohematuria had developed 3 days before admission, and renal function had deteriorated (
creatinine, 2.45 mg/dL) in 2 weeks. He was admitted to our hospital because of rapidly progressive
glomerulonephritis (RPGN) and
multiple pulmonary nodules. On admission, the clinical diagnosis was suspected to be
granulomatosis with polyangiitis (Wegener's) (GPA), although tests for proteinase-3
anti-neutrophil cytoplasmic antibody (PR3-ANCA) were negative.
Antibiotics were administered for 5 days. After renal biopsy,
methylprednisolone pulse
therapy and
cyclophosphamide pulse
therapy were performed. The pathological diagnosis on the basis of the renal biopsy was glomerular and interstitial
hemorrhage, possibly associated with
vasculitis. After the treatment, the pulmonary symptoms,
multiple pulmonary nodules, and severe inflammatory reactions in the peripheral blood were resolved. However, renal dysfunction progressed to
end-stage renal disease 1 month after renal biopsy.
Hemodialysis was started, and the
steroid therapy was continued. During
hemodialysis, a second renal biopsy was performed and led to a diagnosis of pauci-immune focal segmental crescentic
glomerulonephritis. Renal function gradually recovered, and
hemodialysis was discontinued. This case was (double)
ANCA-negative GPA which presented prominent glomerular and interstitial
hemorrhage, may be associated with small vessel
vasculitis, but without active necrotizing and crescentic glomerular lesions, in the rapidly progressive
glomerulonephritis.