Article Figures & Data
Figures
- Figure 1.
Proteinuria trend versus time for seven patients who achieved remission with adrenocorticotropic hormone (ACTH). Time zero demarcates the end of the ACTH treatment course for each patient. Patients 15 and 23 experienced relapse after ACTH therapy, with patient 15 not meeting remission criteria because of worsened renal function, and patient 23 attaining remission in proteinuria with a repeat course of Stanford Protocol ACTH but not meeting criteria for remission based on serum creatinine. Additional immunosuppressive therapy used during follow-up period: patient 3, mycophenolate mofetil and tacrolimus; patient 12, mycophenolate mofetil; patient 23, cyclosporine and ACTH.
Tables
Patient Age/(yr)/Sex Race/Ethnicity FSGS Morphology Previous Immunosuppression Steroid Response Category Time from Diagnosis to Treatment (mo) Serum Creatinine (mg/dl) eGFR (ml/min per 1.73 m2) Proteinuria (mg/g) Columbia patients 1 42/Male WNH NOS Steroids, MMF, tacrolimusa SR 2b 2.7 28 7000 2 38/Female BNH NOS Steroids, MMF, cyclosporine SR 56 2.3 31 5046 3 21/Female WNH Tip Steroids, tacrolimus, cyclosporine SD 12 1.1 67 5200 4 40/Male WNH Tip Steroids, tacrolimus, cyclosporine, MMF SR 96 1.2 71 2100 5 18/Male BNH Tip Steroids, tacrolimus, MMF SR 13 2.6 42 11,200 6 46/Male WNH NOS Steroids, cyclophosphamide, MMF, tacrolimus SD 30 3.2 23 16,800 7 64/Female WH Cellular Steroids, MMF, tacrolimus, SD 61 1.6 35 10,300 8 60/Female WNH Cellular Steroids, tacrolimus, MMF, cyclosporine SR 23 2.3 23 2300 9 66/Male WNH Tip Steroids, cyclosporine, MMF SR 132 1.0 66 1600 10 36/Male WNH NOS Steroids, cyclosporine SR 96 0.8 116 2200 11 30/Female WNH Tip Steroids, MMF, cyclosporine, tacrolimus SR 13 3.4 17 8800 12 47/Female BH Tip Steroids, tacrolimusa SR 4 3.6 17 15,200 Stanford patients 13 66/Male WH NOS Steroids SR 14 2.2 30 2020 14 65/Male WNH Cellular None NA 8 2.7 24 3170 15 64/Male WNH NOS Steroids SD 22 1.04 76 7240 16 27/Male WH NOS Steroids, cyclosporine SR 28 2.99 35 2200 17 58/Male WNH Tip None NA 4 2.2 32 3550 18 29/Male BNH Collapsing Steroids, MMF SR 6 3.3 28 23,800 19 28/Female WH Tip Cyclosporine NA 19 0.6 124 4860 20 40/Male WH NOS Steroids SD 29 1.1 84 2320 21 39/Female WH Tip Steroids, MMF SR 24 1.7 37 1710 22 69/Female WNH Tip Steroids, tacrolimus SR 94 0.6 93 4330 23 56/Female WNH Cellular Steroids SR 2 1.1 56 6560 24 39/Male WH Tip Steroids, cyclosporine SD 29 1.0 94 2000 eGFR, estimated GFR; WNH, white non-Hispanic; NOS, not otherwise specified; MMF, mycophenolate mofetil; SR, steroid resistant; BNH, black Non-Hispanic; SD, steroid dependent; WH, white Hispanic; BH, black Hispanic; NA, not applicable.
↵a Patients 1 and 12 also underwent plasma exchange therapy.
↵b Patient 1 had undergone four prior renal transplants for recurrent FSGS and was diagnosed with another recurrence 2 months before starting ACTH.
Characteristic Value Mean age ± SD (yr) 45.3±15.8 Sex (n) Male 14 Female 10 Race/ethnicity (n) White non-Hispanic 13 White Hispanic 7 Black non-Hispanic 3 Black Hispanic 1 FSGS morphology (n) Tip 11 NOS 8 Cellular 4 Collapsing 1 Previous immunomodulatory drugs used (n) 0 2 1 5 2 7 3 5 4 5 Serum creatinine (mg/dl) 2.0 (1.1–2.7) eGFR (ml/min per 1.73 m2) 36 (28–78) Proteinuria (mg/g) 4595 (2200–8020) Time from diagnosis to treatment (mo) 23 (10–43) Steroid response category (n) SDNS 6 SRNS 15 NA 3 Values with ranges in parentheses are medians and interquartile ranges. NOS, not otherwise specified; eGFR, estimate GFR; SDNS, steroid-dependent nephrotic syndrome; SRNS, steroid-resistant nephrotic syndrome; NA, not applicable.
- Table 3.
Treatment regimens, data pre- and post-adrenocorticotropic hormone, and outcome category for patients with FSGS treated with adrenocorticotropic hormone
Patient Treatment Regimen IMD during ACTH Therapy Duration of ACTH Therapy (wk) Change in Laboratory Values before/after ACTH Treatment Outcome Variable Initial Final Columbia patients 1 Columbia Tacrolimus, mycophenolic acida,b 15 Scr (mg/dl) 2.7 3.7 Failed Proteinuria (mg/g) 7000 2841 Albumin (g/dl) 3.7 3.7 2 Columbia None 24 Scr (mg/dl) 2.3 1.3 Partial Proteinuria (mg/g) 5046 963 Albumin (g/dl) 2.1 3.4 3 Individual Tacrolimus 36 Scr (mg/dl) 1.1 0.7 Partial Proteinuria (mg/g) 5200 1500 Albumin (g/dl) 1.9 3.4 4 Columbia None 24 Scr (mg/dl) 1.2 1.4 Complete Proteinuria (mg/g) 2100 272 Albumin (g/dl) 3.7 4.0 5 Individual Cyclosporine 34 Scr (mg/dl) 2.6 1.1 Failed Proteinuria (mg/g) 11,200 7789 Albumin (g/dl) 1.3 2.3 6 Columbia None 12 Scr (mg/dl) 3.2 2.9 Failed Proteinuria (mg/g) 16,800 25,000 Albumin (g/dl) 1.6 1.4 7 Individual None 56 Scr (mg/dl) 1.6 1.2 Failed Proteinuria (mg/g) 10,300 5380 Albumin (g/dl) 2.0 3.3 8 Columbia None 28 Scr (mg/dl) 2.3 1.7 Failed Proteinuria (mg/g) 2300 5620 Albumin (g/dl) 2.9 3.6 9 Columbia None 23 Scr (mg/dl) 1.0 1.0 Failed Proteinuria (mg/g) 1600 1900 Albumin (g/dl) 3.6 3.8 10 Columbia Cyclosporine 19 Scr (mg/dl) 0.8 0.9 Failed Proteinuria (mg/g) 2200 3847 Albumin (g/dl) 2.9 2.4 11 Individual MMF 36 Scr (mg/dl) 3.4 1.5 Failed Proteinuria (mg/g) 8800 9500 Albumin (g/dl) 2.3 1.6 12 Individual Noneb 36 Scr (mg/dl) 3.6 1.3 Partial Proteinuria (mg/g) 15,200 891 Albumin (g/dl) 2.1 3.4 Stanford patients 13 Stanford None 16 Scr (mg/dl) 2.2 2.2 Failed Proteinuria (mg/g) 2020 1620 Albumin (g/dl) 3.8 3.9 14 Stanford None 16 Scr (mg/dl) 2.7 2.9 Failed Proteinuria (mg/g) 3170 2240 Albumin (g/dl) 2.9 NA 15 Stanford None 16 Scr (mg/dl) 1.0 1.1 Partial Proteinuria (mg/g) 7240 1940 Albumin (g/dl) NA 3.3 16 Stanford None 16 Scr (mg/dl) 3.0 2.7 Failed Proteinuria (mg/g) 2200 2240 Albumin (g/dl) 4.8 NA 17 Stanford None 16 Scr (mg/dl) 2.2 2.1 Failed Proteinuria (mg/g) 3550 4580 Albumin (g/dl) 4.3 3.6 18 Stanford None 16 Scr (mg/dl) 3.3 3.9 Failed Proteinuria (mg/g) 23,800 18,600 Albumin (g/dl) 1.7 2.8 19 Stanford None 16 Scr (mg/dl) 0.6 NA Lost to follow-up Proteinuria (mg/g) 4860 NA Albumin (g/dl) 2.4 NA 20 Stanford None 16 Scr (mg/dl) 1.1 1.1 Failed Proteinuria (mg/g) 2320 1570 Albumin (g/dl) 3.4 3.8 21 Stanford None 16 Scr (mg/dl) 1.7 2.0 Failed Proteinuria (mg/g) 1710 3230 Albumin (g/dl) 3.2 3.9 22 Stanford None 16 Scr (mg/dl) 0.6 0.7 Complete Proteinuria (mg/g) 4330 270 Albumin (g/dl) 1.7 3.6 23 Stanford None 16 Scr (mg/dl) 1.1 1.2 Partial Proteinuria (mg/g) 6560 2120 Albumin (g/dl) 2.2 2.1 24 Stanford None 16 Scr (mg/dl) 1.0 1.7 Failed Proteinuria (mg/g) 2000 6100 Albumin (g/dl) 3.3 NA Patient 19 was lost to follow-up. Columbia protocol: 80 units subcutaneously (SC) twice weekly for 24 weeks. Stanford protocol: 40 units subcutaneously weekly for 2 weeks, 80 units SC weekly for 2 weeks, then 80 units SC twice weekly to complete 16 weeks of therapy. Individual treatment regimens: Patient 3: 80 units twice weekly for 18 weeks, then 80 units weekly for 18 weeks; patient 5: 80 units twice weekly for 16 weeks, then three times weekly for 18 weeks; patient 7: 80 units twice weekly for 24 weeks, then 50 units twice weekly for 10 weeks, then 50–80 units weekly for 22 weeks; patient 11: 80 units twice weekly for 20 weeks, then 40 units twice weekly for 16 weeks; patient 12: 80 units twice weekly for 13 weeks, then 80 units weekly for 21 weeks. IMD, immunosuppressive drugs; ACTH, adrenocorticotropic hormone; Scr, serum creatinine, MMF, mycophenolate mofetil; NA, not applicable.
↵a Patient 1 had undergone four prior renal transplants for recurrent FSGS and was diagnosed with another recurrence 2 months before starting ACTH.
↵b Patient 1 underwent plasma exchange therapy; patient 12 completed plasma exchange therapy during week 1 of ACTH therapy.
- Table 4.
Baseline characteristics and treatment regimen, organized by response category during adrenocorticotropic hormone therapy
Variable Steroid Response Category (n) Age (yr) Sex (n) Race/Ethnicity (n) FSGS Morphology (n) Time from Diagnosis to Treatment (mo) Previous IMD (n) Creatinine (mg/dl) eGFR (ml/min per m2) Proteinuria (mg/d) IMD use during ACTH (n) Treatment Regimen (n) Complete remission (n=2) SR: 2 55 (40–69) M: 1 F: 1 WNH: 2 Tip: 2 95 (94–96) 2: 1 4: 1 0.9 (0.6–1.2) 82 (71–93) 3215 (2100–4330) Yes: 0 No: 2 Stanford (1) Columbia (1) Partial remission(n=5) SR: 3 SD: 2 43 (21–64) M: 1 F: 4 WNH: 3 BNH:1 BH: 1 NOS: 2 Tip: 2 Cellular: 1 12 (2–56) 1: 2 2: 1 3: 2 1.8 (1.1–3.6) 49 (17–76) 7849 (5046–15,200) Yes: 2 No: 3 Stanford (2) Columbia (1) Individual (2) Failed (n=17) SR: 9 SD: 5 NA: 3 44 (18–66) M: 12 F: 5 WNH: 8 BNH: 2 WH: 7 NOS: 6 Tip: 7 Cellular: 3 Collapsing: 1 31 (2–132) 0: 2 1: 3 2: 5 3: 4 4: 3 2.1 (0.6–3.4) 49 (17–124) 3170 (1600–23,800) Yes: 2 No: 15 Stanford (9) Columbia (5) Individual (3) Values with ranges in parentheses are medians and interquartile ranges. IMD, immunosuppressive therapy; ACTH, adrenocorticotropic hormone; SR, steroid resistant; SD, steroid dependent; M, male; F, female; WNH, white non-Hispanic; BNH: black non-Hispanic; BH, black Hispanic; NOS, not otherwise specified; WH: white Hispanic.
Steroid-like Adverse Events Events (n) Patients Experiencing Event (n) Swelling/edema/volume overload/weight gain 5 5 Increased energy 4 4 Mood alteration/anxiety/increased energy 4 4 Elevated BP 3 3 Acne 2 2 Dyspepsia/bloating 2 2 Increased appetite 1 1 Face swelling 1 1 Hyperglycemia/diabetes (reversible) 1 1 Other reported adverse events Sinus congestion/rhinorrhea/upper respiratory tract symptoms 4 4 Muscle cramps 4 4 AKI 2 1 Pain/bruising at injection site 2 2 Tanning of skin 2 2 Rash 2 2 Fatigue 1 1 Vertigo 1 1 Pneumoniaa 1 1 Slow wound healing 1 1 Redness of face on injection days 1 1 Nausea 1 1 Headache 1 1 Polyuria 1 1 Palpitations 1 1 Intermittent chest discomfort 1 1 Loose stools 1 1 Shortness of breath after injection 1 1 Total events (n) 52 ↵a Patient 6 was clinically diagnosed with pneumonia and treated empirically with azithromycin. No chest radiography was performed.
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