Factors Associated With Rapid Progression to Endstage Kidney Disease in Lupus Nephritis

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Figure 1.
(A) Glomerulus displays endocapillary hypercellularity: Capillary loops are retracted; retraction and periglomerular fibrosis are seen; adhesions to the Bowman’s capsule are identified; 40× PAS stain. (B) EM (same sample): Capillary loops show subendothelial widening (white star); there is endothelial swelling; findings are suggestive of thrombotic microangiopathy; EM direct magnification 12000×. (C) Two glomeruli displaying retraction of the glomerular tuft with hypertrophy and hyperplasia of the podocytes; PAS 20×. (D) EM (same sample): Marked retraction of the glomerular tuft with podocyte hypertrophy and hyperplasia; there is segmental scarring at the tip area (opposite to the vascular pole); findings are suggestive of collapsing glomerulopathy; EM direct magnification 12000×. EM: electron microscopy; PAS: Periodic acid–Schiff.

Table 1.

Demographic, histopathologic, and initial renal function characteristics of the patients.

	Values
Sex, female:male	8:2
Age at LN diagnosis, yrs	34.2 ± 7.3
Disease duration, yrs	2.2 ± 2.5
Race/ethnicity, n
Black	5
Hispanic	2
White	2
Asian	1
LN histopathologic class
III	1
IV	5
IV/V	2
V	2
Activity index	5.7 ± 4.9
Chronicity index	3.3 ± 3.0
Serum creatinine, μmol/L	82.1 ± 15.5
eGFR, mL/min/1.73 m²	90.2 ± 24.9
Anemia, n (%)^{^a}	3 (30%)
Proteinuria, g/24 h	2.7 ± 1.04
Nephrotic syndrome, n (%)	5 (50%)
Serum albumin, g/L	32.5 ± 3.7
Hypertension, n (%)^{^b}	5 (50%)
Time to ESKD, months	19.2 ± 12.4
Rate of renal function decline, mL/min/1.73 m²/yr, median	43.3

↵^aDefined as hemoglobin < 120 g/L for females and < 130 g/L for males.
↵^bDefined as blood pressure > 130/80 mmHg. eGFR: estimated glomerular filtration rate; ESKD: endstage kidney disease; LN: lupus nephritis.

Table 2.

Main histologic and treatment characteristics in patients with catastrophic progression to ESKD.

Sex/Race/Age, yrs	ISN/RPS Class	eGFR	Time to ESKD, months	Treatment	Factors Associated With ESKD
F/H/42	V	110	36	GC, AZA, AM, ACEI	Patient achieved complete remission for 24 months and then discontinued all medications to follow a naturopathic approach
M/W/26	III	137	23	GC, MMF, AM, ACEI	Patient achieved complete remission at 12 months and then discontinued all medications to follow alternative treatments
F/B/41	IV/V	82	31	GC, CYC, AM, MMF, ACEI	Severe interstitial inflammation and tubuloreticular inclusions
F/A/44	IV/V	61	11	GC, AZA, AM, ACEI	Patient discontinued all medications after 3 months
F/B/26	V	88	25	GC, MMF, AM, ACEI	Collapsing glomerulopathy
F/B/33	IV	111	12	GC, CYC, AM, MMF	Patient did not achieve remission and discontinued all medications after 6 months
F/B/38	IV	81	6	GC, MMF, AM, RTX	Extensive thrombotic microangiopathy
F/B/31	IV	61	36	GC, CYC, MMF	Severe interstitial inflammation and tubuloreticular inclusions
M/W/24	IV	93	6	GC, CYC, AM, TPE	Anti-GBM nephropathy (anti-GBM antibodies detected at disease onset)
F/H/37	IV	81	6	GC, MMF, AM, RTX	Thrombotic microangiopathy (catastrophic APS)

A: Asian patients; ACEI: angiotensin-converting enzyme inhibitor; AM: antimalarials; APS: antiphospholipid syndrome; AZA: azathioprine; B: Black patients; CYC: cyclophosphamide; eGFR: estimated glomerular filtration rate; ESKD: endstage kidney disease; F: female; GBM: glomerular basement membrane; GC: glucocorticoids; H: Hispanic patients; ISN/RPS: International Society of Nephrology/Renal Pathology Society; M: male; MMF, mycophenolate mofetil; RTX: rituximab; TPE: therapeutic plasma exchange; W: White patients.