Adrenocorticotropic hormone analog use for podocytopathies

Figures & data

Table 1 Baseline characteristics and previous therapy

	Patient number	Age, years	Race	Duration prior to Acthar Gel	Sex	Proteinuria, mg/g or mg/d	Serum creatinine, mg/dL	Serum albumin, g/dL	Serum TC, mg/dL	Initial IS therapy	Response to initial therapy
MCD	1	32	Asian	6 months	F	16,465 mg/d	0.84	0.8	506	Prednisone 60 mg/d	CR
	2	73	C	6 months	M	4,060 mg/d; 3,100 mg/g	1.2	4.1	159	Prednisone 60 mg/d	NR
	3	6	C	16 years	M	12,400 mg/g	1.3	2.2	NA	Prednisone	NR
										Azathioprine
										MMF
										Cyclophosphamide
FSGS	4 Tip	44	C	6 months	F	8,430 mg/d	0.84	2.3	290	Acthar Gel	NR
	5 Coll	33	C	10 months	F	7,920 mg/d	0.99	1.9	404	Prednisone 60 mg/d	NR
										PE/Dexa/MMF	NR
										Abatacept	PR
	6	38	C	10 years	F	5,800 mg/d	1.2	3.3	NA	Acthar Gel	NR
	7 Coll	17	AA	10 months	M	36,000 mg/g	1.79	1.2	359	Prednisone 60 mg/d	NR
										Cys A 150 mg bid
	8 NOS	65	C	4 years	M	7,700 mg/g	1.93	3.6	189	MMF 1,500 mg bid	NR
	9 NOS	19	AA	3 years	F	16,100 mg/g	1.48	2.2	251	Prednisone 60 mg/d	CR
										Cys A 150 mg bid	CR
										Prednisone 60 mg/d	PR
	10 Coll	54	AA	8 months	F	11,090 mg/g	1.34	2.2	289	Prednisone 80 mg/d	Initial PR, then side effects
	11	52	AA	9 years	F	6,700 mg/g	6.3	1.2	NA	Prednisone	NR
										Cyclophosphamide
										Cys A
										MMF
										Tacrolimus
										Rituximab
	12	33	C	10 years	F	3,100 mg/g	0.9	3.3	NA	Prednisone	NR
										Cyclophosphamide
										MMF
										Azathioprine
										Pentoxifylline
	13	43	C	4 years	M	5,900 mg/g	0.9	4.2	NA	Prednisone	PR

Note: The laboratory values in this table are those obtained prior to any IS therapy.

Abbreviations: AA, African American; bid, twice daily; C, Caucasian; Coll, collapsing FSGS; CR, complete remission; Cys A, cyclosporine A; Dexa, dexamethasone; F, female; FSGS, focal segmental glomerulosclerosis; IS, immunosuppressive; M, male; MCD, minimal change disease; MMF, mycophenolate mofetil; NA, not available; NOS, FSGS not otherwise specified; NR, no response; PE, plasma exchange; PR, partial remission; TC, total cholesterol; Tip, glomerular tip lesion.

Table 2 Response to Acthar Gel

Patient	Acthar Gel regimen	Acthar Gel duration	Follow-up after Acthar Gel initiation	Concomitant therapy	Serum creatinine, pre-/post-Acthar Gel initiation, mg/dL	Proteinuria, pre-/post-Acthar Gel initiation	Outcome	Adverse events
1	80 U twice weekly, then weekly	Ongoing	17 months	RASB Statin MMF 1,000 mg bid	0.7/0.51	84,300 mg/d/1,860 mg/d	PR	None
2	80 U twice weekly	6 months	16 months	Statin	1.2/1.39	3,119 mg/g/918 mg/g	PR	None
3	80 U twice weekly	10 months	26 months	RASB MMF	1.3/1.2	12,400 mg/g/270 mg/g	CR	None
4	40 U twice weekly	5 months	18 months	RASB	0.84/0.9	8,430 mg/d/10,825 mg/d	NR	Weight gain, myalgia, worsening diabetes, and hypertension
5	80 U twice weekly	9 months	16 months	RASB Abatacept	3.05/0.83a	Albumin/creatinine: >30,000 mg/g/255 mg/ga	CR	Weight gain
6	80 U twice weekly	2 months	16 months	RASB	2.4/ESRD	NA	NR	None but noncompliant
7	80 U twice weekly	10 months	15 months	Vitamin D Statin	2.44/3.61	15,700 mg/g/2,700 mg/g	NR (rise in creatinine)	None
8	80 U twice weekly	1 month	7 months	RASB Vitamin D Statin	3.02/2.3	8,400 mg/g/15,600 mg/g	NR	Weight gain, hypertension, edema, and fatigue
9	80 U twice weekly	3 months	20 months	Vitamin D	1.01/1.01	1,100 mg/g/67 mg/g	CR	None
10	40 U twice weekly, then weekly	Ongoing	7 months	RASB Statin	1.55/1.71	2,700 mg/g/624 mg/g	PR	Myalgia, weakness, and hyperpigmentation
11	80 U twice weekly	7 months	14 months	RASB	6.3/6.5	6,700 mg/g/12,800 mg/g	NR	Skin pigmentation, weight gain, worsening diabetes, and Cushingoid features
12	80 U twice weekly	7 months	17 months	RASB	0.9/1.2	3,100 mg/g/2,100 mg/g	NR	None
13	80 U twice weekly	7 months	18 months	RASB	0.9/1.0	5,900 mg/g/1,700 mg/g	PR	None

Note:

a Post-Acthar Gel initiation laboratory values for this patient are from her 7-month follow-up.

Abbreviations: CR, complete remission; ESRD, end-stage renal disease; MMF, mycophenolate mofetil; NA, not available; NR, no response; PR, partial remission; RASB, renin–angiotensin system blockade; U, units.

Table 3 Case series, cohort studies, and case reports of various glomerulopathies treated with ACTH preparations

Study	Preparation	MN	FSGS	MCD	DN	IgAN	MPGN	Other	Discontinued due to AEs
Berg et al^Citation13	T	9/5a (5)b							0/5
Berg and Arnadottir^Citation16	T	10 (10)	1 (1)	2 (2)	2 (2)		6 (6)	2 (1)	0/23
Picardi et al^Citation15	T	7 (5)							2/7
Ponticelli et al^Citation14	T	16 (14)							2/16
Rauen et al^Citation17	T	4 (4)							0/4
Lindskog et al^Citation18	T	5 (4)							0/5
Bomback and Radhakrishnan^Citation19	Gel	11 (9)	1 (1)c	1 (0)		1	4 (0)	3	0/21
Bomback et al^Citation20	Gel	5 (2)	3 (1)c	2 (1)		5			3/15
Hogan et al^Citation21	Gel		24 (7)						0/24
Tumlin et al^Citation22	Gel				14 (8)				9 of 23 initially randomizedd
Hladunewich et al^Citation23	Gel	20 (12)							0/20
Lorusso et al^Citation24	T	9 (4)	2 (2)	2 (1)			2 (1)		1 of 18 initially randomized
Gigante et al^Citation29,e	T	1 (1)
Watson^Citation30,e	Gel	1 (1)
Anwar et al^Citation31,e	Gel		1 (1)
Madan^Citation32,e	Gel		1 (1)
This study	Gel		10 (4)	3 (3)					2/13
Total		94	39	10	16	6	12	5
Overall response		67 (71%)	16 (41%)	7 (70%)	10 (63%)		7 (58%)

Notes:

a Five were treated for 1 year and are included in the overall total, nine just for the short term;

b overall number attaining complete or partial remission in parentheses;

c included among the study of Hogan et al²¹ and not counted separately in total;

d includes one withdrawing due to need for injections and two with scheduling conflicts;

e eisolated case reports.

Abbreviations: ACTH, adrenocorticotropic hormone; AEs, adverse events; DN; diabetic nephropathy; FSGS, focal segmental glomerulosclerosis; Gel, Acthar Gel; Ig, immunoglobulin; IgAN, IgA nephropathy; MCD, minimal change disease; MN, membranous nephropathy; MPGN, membranoproliferative glomerulonephritis; T, tetracosactide (long-acting), ACTH_1–24.

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