Study was updated and issued a new NCT number.

Treatment with combination ACTHar gel and Tacrolimus therapy or ACTHar gel therapy alone in lowering urinary protein to creatinine (UP/Cr) ratios

This is a multicenter, Phase 4, prospective, open labeled study to compare the safety, tolerability, and efficacy of a 12month course of ACTHar Gel and Tacrolimus therapy or ACTHar gel therapy alone in lowering urinary protein to creatinine (UP/Cr) ratios.

The addition of tacrolimus patients exhiba partial to ACTH resulted in a further reduction in Fibrillary.

Spe #1-will randomize with biopsy proven DNA-JB9 positive Fibrillary glomerulopathy to receive course of ACTHar gel alone at 80 units per week or in combination with oral Tacrolimus at 1 mg BID

Hypothesis Treatment with ACTHar Gel with oral Tacrolimus will result in a higher eGFR after 24 months of follow up than patients randomized to ACTHar gel therapy alone.

Hy3: Combination therapy of ACTHar Gel and Tacrolimus in patients with DNA-JB9 positive Fibrillary will lead to significant markers of podocyte injury compared ACTHar Gel alone.

Histologic Inclusion Criteria:

All patients with a diagnosis of The Fibrillary GN wbe classified according to the Nasr nomenclature:

1. Renal biopsy demonstrating JB9 Positive staining within 3 years of study randomization
2. Mesangial expansion with/without glomerular sclerosis diffuse sclerosis

4) Crescents or capillary proliferation Note: Patients with > 50% interstitial fibrosis will not be eligible for study

Inclusion Criteria:

1. Female ag
2. Biopsy proven Fibrillary GN within 3 years of study randomization
3. Stable Maximum RAAS inhibition X 40 weeks prior to randomization Note: Maximum inhibition will be the discretion of the site PI
4. eGFR > 25 mls/min
5. UP/Cr ratio > 2000 mg/gm 5 Note: IF UP/Cr less than 2000 mg/gm, a formal urine collection for total protein can be performed. The total 24-hour will need to >/= 2000mg.
6. Blood pressure targeted to < 140 at the time of randomization
7. Patients with MGUS without history of myeloma WILL be eligible.
8. Patients with monoclonal staining for fibrillary fibers will be excluded
9. Patients with Type II non-insulin dependent diabetes WILL be eligible provided the renal biopsy does not show nodular Kimmelstiel Wilson lesions

Exclusion

1. Patients with MGUS and history of myeloma WILL be eligible
2. Patients with active viral production of either B or C as evidence by historical PCR test positive for active viral shedding
3. HIV seropositivity
4. Renal biopsy data with > 5Interstitialxxxx Fibrosis
5. Patient with active or a known history
6. Patients with insulin Dependent diabetes mellitus will be excluded Note: Patients diabetes and are well controlled without the need for insulin WILL be eligible for the study.
7. Patients with Type non-insulin diabetes WILL be eligible provided the renal biopsy does not s nodular Kimmelstiel Wilson lesions.
8. Patients receiving steroids, MMF, cyc, Azathioprine or other immunosuppressive agent with of study random Note: Wash medications will be allowed at the screening visit
9. Patients having received Rituximab or cell modifying biologic bbtherapy within 60 months of randomiza