Coexistence of anti-glomerular basement membrane disease and IgA nephropathy: an illustrative case and comprehensive literature review

Figures & data

Figure 1. Pathological results of kidney biopsy and clinical course. (A) Light microscopy showing the formation of large cellular crescents (PASM staining, × 200). (B) Immunofluorescence showing linear deposition of IgG along the GBM. (C) IgG subclass shows capillary lineal deposition of IgG3. (D) Immunofluorescence showing lumpy deposition of IgA in the mesangium. (E) Clinical course of patient. GBM, glomerular basement membrane; Ig, immunoglobulin; Scr creatinine; HD, hemodialysis; PE, plasma exchange; MethyI-PD, Methylprednisolone; IV, intravenous; CYC, cyclophosphamide; w. week; m, month.

Table 1. Clinical characteristics of anti-GBM disease combined with IgAN (2005–2023).

Author		Gender	Age (yr)	Country	Oliguria/anuria	Hemoglobin (g/dL)	Proteinuria	Serum creatinine on diagnosis (mg/dL)	Level of anti-CBM antibody	Crescent ratio (%)	Immunofluorescence on GBM	Hemodialysis on diagnosis	Immunosuppressive therapy	Follow-up time (month)	Outcome – kidney
1	Our study	Female	68	China	Yes	8.2	2+	11.8	184.94 U/mL	69%	IgG 2+	Yes	IV Methyl-PD + oral PD + PE + IV CYC	22.0	Improved renal function
2	Takahiro Matsuno (2023) [2]	Male	76	Japan	NR	10.1	3+	5.2	485.7 U/mL	NR	NR	Yes	IV Methyl-PD + PE + IV CYC + oral PD + oral azathioprine	2.6	MHD
3	Yu Chen (2023) [13]Cong-rong Shen (2023) [8]	Female	35	China	Yes	10.6	3 +	4.7	Positive	55%	IgG 3+	No	IV Methyl-PD + PE + IV CYC + IV immunoglobulin + oral PD	3.0	Improved renal function
4		Female	49	China	Yes	6.8	2+	8.0	59 RU//mL	91%	IgG	Yes	IV Methyl-PD + PE + Immunosuppressant + oral PD	0.9	ESKD
5		Male	52	China	No	9.3	2+	8.5	142 RU//mL	79%	IgG, IgM	Yes	IV Methyl-PD + PE + Immunosuppressant + oral PD	19.6	ESKD
6		Female	49	China	No	8.3	3+	8.4	45 RU//mL	87%	IgG, IgM	No	IV Methyl-PD + PE + Immunosuppressant + oral PD	1.1	ESKD
7		Male	60	China	Yes	10.7	1+	10.1	200 RU//mL	86%	IgG, IgM	Yes	IV Methyl-PD + PE + Immunosuppressant + oral PD	1.1	ESKD
8		Male	16	China	No	12.8	4+	4.0	189 RU//mL	80%	IgG, IgM	No	IV Methyl-PD + PE + Immunosuppressant + oral PD	45.8	Improved renal function
9		Female	35	China	Yes	7.7	2+	9.3	86 RU//mL	90%	IgG	No	IV Methyl-PD + PE + Immunosuppressant + oral PD	1.0	ESKD
10		Male	58	China	No	9.3	3+	8.5	25 RU//mL	62%	IgG	No	IV Methyl-PD + PE + oral PD	1.2	ESKD
11		Male	70	China	No	8.8	4+	3.1	25 RU//mL	77%	IgG, IgM	No	PE + Immunosuppressant + oral PD	27.5	Improved renal function
12		Male	37	China	No	12.0	4+	6.0	200 RU//mL	91%	IgG	No	IV Methyl-PD + PE + Immunosuppressant + oral PD	24.4	Improved renal function
13		Female	31	China	No	9.9	3+	2.5	200 RU//mL	52%	IgG, IgM	No	IV Methyl-PD + PE + Immunosuppressant + oral PD	17.6	Improved renal function
14		Female	43	China	No	9.9	1+	1.7	29 RU//mL	53%	IgG, IgM	No	IV Methyl-PD + PE + Immunosuppressant + oral PD	16.3	Improved renal function
15		Female	54	China	No	10.4	3+	5.1	200 RU//mL	93%	IgG	No	IV Methyl-PD + PE + Immunosuppressant + oral PD	15.5	Improved renal function
16		Male	76	China	No	8.3	2+	9.6	148 RU//mL	78%	IgG	No	IV Methyl-PD + PE + Immunosuppressant + oral PD	9.6	Improved renal function
17		Male	54	China	No	7.8	1+	6.2	160 RU//mL	77%	NR	No	IV Methyl-PD + PE + Immunosuppressant + oral PD	0.9	ESKD
18		Male	17	China	No	8.4	2+	2.2	200 RU//mL	86%	IgG	No	IV Methyl-PD + PE + Immunosuppressant + oral PD	2.9	Improved renal function
19	Chuan Guo (2022) [14]	Male	49	China	No	10.8	2+	1.5	68 RU//mL	70%	IgG 3+	No	IV Methyl-PD + IV CYC + oral PD	7.0	Improved renal function (Scr, 1.19–1.39 mg/dL)
20	Huihui Chen (2022) [15]	Female	40	China	No	9.9	1+	3.6	192.4 IU//mL	77%	IgG 3+	No	PE + oral CYC + oral PD	12.0	Improved renal function (1.17 mg/dL)
21	Fu Shaojie (2022) [16]	Female	26	China	NR	NR	3+	1.9	142 RU/ mL	48%	IgG	No	IV Methyl-PD + PE + IV CYC + oral PD	12.0	Remain within the normal range
22	Varun Bajaja (2022) [17]	Female	13	India	Yes	7.4	2+	3.4	Positive	56%	IgG1 (2 ∼ 3+), IgG4 (1 ∼ 2+)	Yes	IV Methyl-PD + PE + IV CYC + oral PD	NR	Improved renal function (Scr, 1.26 mg/dL)
23	Manyu Zhang (2021) [18]	Female	41	China	Yes	9.1	2+	4.6	100.8 RU/mL	> 50%	NR	Yes	IV Methyl-PD + PE + IV CYC + IV rituximab + oral PD	6.5	Scr level fluctuated from 1.33–2.05 mg/dL
24	Candice Khor (2021) [19]	Female	66	Australia	NR	10.0	1+	3.6	79 U/mL	40%	IgG 3+	No	IV Methyl-PD + PE + IV CYC + oral PD	12.0	Improved renal function
25	Malviya PB (2021) [20]	Male	29	India	Yes	11.0	3+	12.7	Positive	100%	IgG	Yes	IV Methyl-PD + PE + IV CYC + oral PD	NR	ESKD
26	Kwang-Sun Suh (2019) [21]	Female	38	Korean	NR	NR	1+	5.5	187.2 U/mL	69%	IgG	No	IV Methyl-PD + IV CYC + oral PD	3.0	Improved renal function (Scr, 2.08 mg/dL)
27	Tadasu Kojima (2019) [22]	Female	66	Japan	Yes	11.3	4+	4.5	116 IU/mL	58%	IgG 2+	Yes	IV Methyl-PD + PE + oral PD	NR	MHD
28	Longano Anthony (2019) [10]	Male	22	Australia	No	9.0	1+	0.9	Positive	18%	IgG	No	PE + oral PD + oral CYC + oral azathioprine	4.0	Scr remained within the normal range
29	Yashita Gupta (2019) [23]	Male	20	India	Yes	NR	3+	13.1	Positive	100%	IgG (3 ∼ 4+)	No	IV Methyl-PD + PE + IV CYC + oral PD	NR	Nondialysis-dependent chronic renal failure
30	Jing Shan (2018) [24]	Male	40	China	No	9.1	2+	4.7	141 RU/mL	NR	NR	No	IV Methyl-PD + PE + IV immunoglobulin + IV CYC + oral PD	1.0	Improved renal function (Scr, 1.48 mg/dL)
31	Ishwarya Annamalai (2017) [25]	Female	22	India	Yes	8.6	3+	3.2	96 U/mL	70%	IgG 2+	No	IV Methyl-PD + oral PD	NR	Improved renal function
32	Dechao Xu (2016) [26]	Female	50	China	No	9.9	1+	2.62	258.3 EU/mL	89%	IgG 3+	No	IV Methyl-PD + oral mycophenolate + oral PD	20.0	Scr remained within the normal range
33	Yating Ge (2015) [27]	Male	24	China	Yes	8.2	4+	15.7	Positive	58%	IgG	Yes	IV Methyl-PD + PE + IV CYC + oral PD	2.9	MHD
34	Weifeng Shang (2015) [28]	Female	54	China	Yes	8.1	3+	10.0	Positive	19%	IgG	Yes	IV Methyl-PD + PE + IV immunoglobulin + IV CYC + oral PD	3.0	MHD
35	Hiroshi Yamaguchi (2013) [29]	Male	46	Japan	NR	11.9	1+	6.6	214 EU/mL	94%	IgG	No	IV Methyl-PD + oral PD	NR	MHD
36	Aifeng Wang (2012) [30]	Female	31	China	NR	NR	3+	3.3	93.5 RU/mL	82%	IgG 3+	No	IV Methyl-PD + oral CYC + oral PD + PE	1.0	Improved renal function
37	Bixia Gao (2009) [31]	Female	38	China	No	6.1	2+	6.6	237.6 EU/mL	28%	IgG 2+	No	IV Methyl-PD + oral PD + IV CYC	4.0	Improved renal function (Scr, 2.19mg/dL)
38	Zhao Cui (2006) [32]	Male	27	China	NR	NR	NR	15.2	Positive	100%	IgG +	NR	NR	NR	MHD
39	Yan Wang (2005) [33]	Male	34	China	NR	11.4	4+	8.6	Positive	63%	IgG 3+	Yes	oral mycophenolate	NR	ESKD

Abbreviations: GBM, glomerular basement membrane; IgAN, IgA nephropathy; Scr, serum creatinine; HD, hemodialysis; PE, plasma exchange; Methyl-PD, Methylprednisolone; IV, intravenous; CYC, cyclophosphamide; MHD, maintained hemodialysis; ESKD, end-stage kidney disease; NR, not reported.

Table 2. Factors associated with renal survival of patients.

Variables	Non-ESKD (24)	ESKD (15)	p value
Age (yr)	39.3 ± 17.8	47.5 ± 15.1	0.612
Country	24	15	0.597
China, n (%)	18 (75.0%)	11 (73.3%)
Foreign country, n (%)	6 (25.0%)	4 (26.7%)
Oliguria/anuria	20	11	0.069
Yes, n (%)	6 (30.0%))	7 (63.6%)
No, n (%)	14 (70.0%)	4 (36.4%)
Hemoglobin (g/dL)	20	14	0.643
<9 g/dL, n (%)	13 (65.0%)	8 (57.1%)
≥9 g/dL, n (%)	7 (35.0%)	6 (42.9%)
Proteinuria	24	14	0.789
1+, n (%)	6 (25.0%)	3 (21.4%)
2+, n (%)	8 (33.3%)	3 (21.4%)
3+, n (%)	7 (29.2%)	5 (35.7%)
4+, n (%)	3 (12.5%)	3 (21.4%)
Serum creatinine on diagnosis (mg/dL)	24	15	<0.001
<4 mg/dL, n (%)	13 (54.2%)	0 (0.0%)
≥4 mg/d, n (%)	11 (45.8%)	15 (100.0%)
Crescent ratio (%)	23	14	0.362
<50%, n (%)	4 (17.4%)	1 (7.1%)
≥50%, n (%)	19 (82.6%)	13 (92.9%)
Hemodialysis on diagnosis	24	14	0.002
Yes, n (%)	3 (12.5%)	9 (64.3%)
No, n (%)	21 (87.5%)	5 (35.7%)

Abbreviations: Scr, serum creatinine; ESKD, end-stage kidney disease. Bold values are statistically significant.

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