Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

Figures & data

Figure 1 Typical computed tomography findings in idiopathic RPF. (A) Periaortic lesion (arrow) with aneurysmal change of the affected abdominal aorta and left hydronephrosis. (B) Right renal pelvic mass (arrow). (C) Right periureteral mass (arrow). (D) Placoid lesion (arrow) in the pelvis.

Figure 2 Proposed algorithm for the management of idiopathic RPF.

Abbreviations: GC, glucocorticoids; IS, immunosuppressants.

Table 1 Main Clinical Pictures, Laboratory Findings, Treatment, and Outcomes of Patients with IRPF in Different Clinical Series

References	Kermani et al^Citation26	van Bommel et al^Citation17	Brandt et al^Citation30	Moriconi et al^Citation27	Zhao et al^Citation25	Wallwork et al^Citation68	Urban et al^Citation69	Wang et al^Citation5	Wang et al^Citation5	Mizushima et al^Citation31
	185 Pts	53 Pts	204 Pts	43 Pts	155 Pts	26 Pts	20 Pts	85 Pts	47 Pts	99 Pts
IgG4 or non-IgG4	NA	NA	NA	Mixed	Mixed	Mixed#	Non-IgG4	Non-IgG4	IgG4	IgG4
Age, years	58 ± 12	64 ± 11	56 ± 12	55 ± 11	58 ± 12	55 (43–58)	58 (53–67)	55 ± 13	59 ± 12	67 ± 10
Male, %	61	77	68	63	71	85	65	63	83	85
Clinical Pictures at Diagnosis
Pain (abdominal, flank, back), %	40	92	66	62	57	73	85	55	36	23
Fever, %	9	17	NA	NA	14	NA	NA	6	4	8
Limb edema, %	13	8	17	5	34	NA	NA	8	11	4
Hydronephrosis, %	57	55	96	49	95	46	65	59	74	21
Unilateral, %	NA	40	40	17	38	NA	40	22	34	15
Bilateral, %	56	15	56	32	57	NA	25	36	40	6
Impaired renal function, %	42	66	NA	NA	62	NA	NA	NA	NA	NA
Laboratory Findings at Diagnosis
ESR, mm/h	32 ± 29	45 (23–76)	NA	NA	45 (17–154)	28 (17–48)	47 (29–71)	30 (12–57)	39 (18–72)	NA
CRP, mg/dL	2.1 ± 2.6	2.3 (0.7–4.4)	NA	2.2 ± 1.7	2.6 (0.8–23.1)	0.6 (0.3–1.1)	1.2 (0.6–3.6)	1.0 (0.3–2.0)	0.6 (0.2–1.6)	0.3 (0.1–1.0)
eGFR, mL/min/1.73m^2	NA	NA	NA	77 ± 20	NA	NA	64 (28–86)	NA	NA	NA
Serum creatinine, mg/dL	1.3 (1.1–2.1)	1.4 (1.1–1.9)	NA	NA	4.5 (1.2–21.4)	NA	NA	1.1 (0.8–1.8)	1.0 (0.7–1.4)	NA
Treatments
PSL monotherapy, %	12	NA	57	70	10	0	0	7	11	NA
PSL + tamoxifen, %	NA	NA	2	30	NA	0	0	13	2	NA
Tamoxifen monotherapy, %	NA	NA	13	0	NA	0	0	0	0	NA
PSL + IS, %	NA	NA	21	0	NA	0	0	47	62	NA
PSL + RTX, %	NA	NA	0	0	NA	27	80	1	2	NA
RTX monotherapy, %	NA	NA	0	0	NA	73	20	0	0	NA
No medication, %	12	NA	7	0	14	0	0	23	15	NA
Ureteral stent, %	57	NA	12	51	71	39	45	46	34	NA
Nephrostomy, %	6	NA	2	NA	18			14	4	NA
Ureterolysis, %	28	NA	24	7	12	0	0	16	8	NA
Outcomes
Follow-up duration, months	48 (25–80)	NA	NA	90 ± 56	45 (0.1–169)	3.6 (2.9–4.2)	38 (17–61)	24 (12–48)	20 (12–36)	NA
Remission, %	54	NA	NA	60	NA	NA	75	NA	NA	NA
Relapse, %	12	NA	NA	40	18	NA	15	NA	21	NA
ESRD, %	0	NA	NA	8	0	4	0	NA	NA	NA
CKD, %	32*	NA	NA	NA	47**	NA	NA	NA	NA	NA
Death, %	6	NA	NA	0	9	0	5	NA	NA	NA

Notes: Conversion factor for Cr: mg/dL to μmol/L, ×88.4. Data are presented as mean ± standard deviation or median [interquartile range (IQR1-IQR3)] for continuous variables. *Defined as serum creatinine levels higher than upper limit of normal. **Defined as eGFR <60 mL/min/1.73m².

Abbreviations: CKD, chronic kidney disease; CRP, C-reactive protein; eGFR, estimated glomerular filtration rate; ESRD, end stage renal disease; IgG, immunoglobulin G; IgG4, immunoglobulin G4; IgE, immunoglobulin E; IQR, interquartile range; IS, immunosuppressant; NA, not available; PSL, prednisolone; Pts, patients; RTX, rituximab; SD, standard deviation; #IgG4:73%, non-IgG4:27%.

Figure 3 Aneurysm formation at the periaortic lesion of the abdominal aorta after glucocorticoid therapy. Before (A) and after (B) glucocorticoid therapy, the periaortic lesion is markedly ameliorated, while an aneurysm has developed at the same site.

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