case_images: PMC11810895_01
case_images: Response to treatment with combined rituximab and daratumumab in Patient 1. (A) Proteinuria levels (green line) and SLEDAI scores (yellow line) during the observation period. (B-D) PAS staining of the three kidney biopsies performed at recurrence.
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case_images: PMC11810895_01_fimmu-16-1525892-g001.jpg
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case_images: Therefore, she was admitted to our Nephrology Unit with a diagnosis of SLE and a Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score of 38 ( Figures 1A, B ). IV+V, characterized by diffuse mesangial expansion and endocapillary hypercellularity, and early signs of focal and segmental sclerosis ( Figure 1C ). Immunofluorescence (IF) staining was positive for a "full house" pattern (IgA ++, IgG +++, IgM ++, C3 +++, C4 +, C1q ++, glomerular C4d +++). A kidney biopsy largely confirmed the previous histological findings, in accordance with LN class IV+V ( Figure 1B ). IF staining showed a partial remission of the "full-house" pattern (IgA ++, IgG +, IgM -, C3 -, C4 -, C1q +), consistent with an activity index score of 5 and chronicity index score of 4 ( Figure 1D ). At 2 years of follow-up, she is receiving standard therapies with low-dose glucocorticoids (prednisone 5 mg/day), MMF, and hydroxychloroquine, and proteinuria is stable at 2.6 g/day, with a SLEDAI-2K score of 4, marking the longest remission period since disease onset ( Figure 1A ).
case_images: PMC11810895_02
case_images: Response to treatment with combined rituximab and daratumumab in Patient 2. (A) Proteinuria levels (green line) and SLEDAI scores (yellow line) during the observation period. (B-D) PAS staining of the three kidney biopsies performed at recurrence.
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case_images: PMC11810895_02_fimmu-16-1525892-g002.jpg
case_images: f2
case_images: IU/mL (SLEDAI-2K=19)( Figure 2A ). A kidney biopsy was performed, revealing mesangial expansion and proliferation ( Figure 2B ). IF staining showed IgA +, IgG +++, IgM -, C3 ++, C4 +, C1q ++, and glomerular C4d ++. A second kidney biopsy was performed, which showed mesangial and endocapillary hypercellularity in approximately 70%-80% of the glomeruli, with cellular crescents ( Figure 2C ). However, over the following 2 years, the patient continued to exhibit signs and symptoms consistent with refractory SLE, including headache, photophobia, low complement levels (C3 49-70 mg/dL, C4 6-15 mg/dL), and elevated anti-dsDNA antibodies (169-240 IU/mL) with SLEDAI-2K scores between 10 and 12 ( Figure 2A ), leading to a poor quality of life. Glomerulosclerosis was present in approximately half of the glomeruli ( Figure 2D ). Proteinuria remains negative and renal function normal (0.8 mg/dl) and the patient's SLEDAI-2K score is 0 ( Figure 2A ).