PMC5313425_01
Histopathology of the kidney. The microscopic findings of the glomerulus showed cellular crescent formation and disruption of the Bowman's capsule (A: periodic acid-Schiff staining; original magnification, x400). There were few changes in the glomerular basement membrane and mesangial area. High disease activity was indicated by fibrinoid necrosis in the lobular artery (B: Masson's trichrome staining; original magnification, x400). The immunofluorescence studies revealed no glomerular deposition (C: IgG, D: IgA, E: IgM, F: C3, and G: C1q).
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On the day following admission, percutaneous renal biopsy was performed (Fig. 1).
Clinical course after admission. After admission, methylprednisolone pulse therapy was initiated to treat rapidly progressive glomerulonephritis due to anti-nuclear cytoplasmic antibodies. However, diffuse alveolar hemorrhaging occurred, and the respiratory condition rapidly worsened on Day 5 after admission. Plasma exchange was immediately performed (seven times for two weeks). There was an improvement in the respiratory condition, and the patient was successfully extubated on Day 14. Her serum creatinine level decreased from a maximum of 2.99 mg/dL to 1.48 mg/dL, and the MPO-ANCA titer also decreased to the normal range.
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As shown in Fig. 2, intravenous methylprednisolone pulse therapy was administered every 24 hours for 3 consecutive days; however, on the day following the pulse therapy (Day 5), the patient's respiratory condition suddenly worsened, and she required ventilatory support.
Chest computed tomography. Chest computed tomography on the sudden onset of dyspnea showed broad infiltrative shadows and ground glass opacity in the lungs (left panel). Diffuse alveolar hemorrhaging and acute deterioration of interstitial pulmonary fibrosis was diagnosed. The therapy with plasma exchange and low-dose prednisolone was dramatically effective, and these shadows were considerably attenuated after one month (right panel).
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DAH was diagnosed on bronchoscopy and chest computed tomography, which showed broad infiltrative shadows and ground glass opacity in her lungs (Fig. 3).