PMC6514513_01
Clinical course after the onset of anti-GBM glomerulonephritis. PSL, prednisolone; CRP, C-reactive protein.
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F1
Immediately after recognizing the anti-GBM antibody-positive status, we started methylprednisolone pulse therapy (1 g/day for 3 days) and plasma exchange (seven sessions for 3 weeks) (Fig. 1). As shown in Figure 2f, g, h, i, IgG1 subclass is predominant in the glomeruli. However, with the daily administration of 40 mg oral prednisolone, macrohematuria appeared again and the patient's body temperature and CRP were gradually elevated within 2 weeks (Fig. 1). These abnormalities were rapidly reversed after the second course of steroid pulse therapy, but again relapsed with 2 weeks of oral prednisolone (Fig. 1). The titer of anti-GBM antibody and serum creatinine was gradually decreased from 87.3 U/mL and 6.33 mg/dL to 12.2 U/mL and 3.01 mg/dL, respectively, at the time of discharge (Fig. 1).
Histological findings from renal biopsy. a Periodic acid-Schiff (PAS) staining image of a low-power field (magnification x10). b PAS staining of a high-power field (magnification x20). All the glomeruli were involved by "cellular" crescents (black arrows), which suggested that all the crescents were formed very recently. c Masson trichrome staining image (magnification x10). d Periodic acid methenamine silver staining (magnification x10). White arrows indicate interstitial fibrosis and tubular atrophy. Inflammatory cell infiltration was detected in over 50% of the renal interstitium with moderate to severe interstitial fibrosis and tubular atrophy. e Immunostaining for immunoglobulin G (IgG) of a frozen section (magnification x20). f-i Immunostaining for IgG subclass. IgG1 is positive (f), IgG2 and -3 are borderline positive (g, h), and IgG4 is negative (i).
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F2
Renal biopsy specimens included 16 glomeruli of which six were global sclerosis and four were segmental sclerosis; cellular crescents were present in all glomeruli with or without glomerular sclerosis (Fig. 2a and b). Inflammatory cell infiltration was detected in over 50% of the renal interstitium with moderate to severe interstitial fibrosis and tubular atrophy (Fig. 2c and d). Linear IgG depositions were detected along the glomerular capillaries, which was compatible with anti-GBM glomerulonephritis (Fig. 2e). As shown in Figure 2f, g, h, i, IgG1 subclass is predominant in the glomeruli. In this case, linear IgG deposition on GBM (Fig. 2e, f, g, h, i) was not associated with diabetic nephropathy, in which faint linear IgG deposition on GBM is sometimes observed.