PMC7902059_01
The clinical course for the C1q nephropathy patient. (A) Selected urinalysis results and clinical course for the patient. Proteinuria and urinary occult blood have been negative for >18 months since administration of rituximab, indicating long-term remission. No relapse occurs after withdrawing maintenance dose of corticosteroid. (B) The course of steroid treatment is shown for corresponding time points in (A). (C) Urinary protein/creatinine ratios during the care provided by us.
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F1
Instead, the patient was switched to traditional Chinese medicine (ingredients unknown) for nearly 4 months (Figure 1A). During this time, she was also treated with methylprednisolone (2 mg/kg daily) and aldehyde oxystarch, without significant efficacy (Figures 1A,B). After 2 weeks of rituximab treatment, B cells were completely depleted, and renal function was significantly improved with a GFR of 93 ml/min (Figure 1A). The treatment courses and selected laboratory results are shown in Figure 1.
Pathology evaluation for the C1q nephropathy patient. (A) Hematoxylin-eosin staining shows increased number of cells in glomerulus. No glomerular crescent, focal segmental glomerular sclerosis, and fibrosis are noted. However, renal tubule dilation and vacuolar degeneration of epithelial cells are present, and renal interstitium is slightly infiltrated with inflammatory cells, indicating a minimal change disease (MCD). (B) Periodic acid-Schiff (PAS) staining shows no remarkable cellular proliferation of mesangium and stroma. (C) Periodic Schiff-methenamine (PASM) staining shows normal capillary loops and no significantly thickened basement membrane. (D) Immunofluorescent staining confirms large amount of C1q deposition. (E,F) Transmission electron microscopy shows vacuolar degeneration of epithelial cells, podocyte foot processes effacement, proliferation of mesangium and stroma, and thin basement membrane.
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F2
Due to frequency of relapses, renal biopsy was discussed again with the patient's parents for which consent was given in May 2018, and microscopy results were consistent with C1q nephropathy (Figures 2A-F).