Kidney Week

Abstract: FR-OR085

Treatment Response in Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits

Session Information

• Glomerular Diseases: Clinical, Outcomes, and Trials
  October 26, 2018 | Location: 24A, San Diego Convention Center
  Abstract Time: 05:54 PM - 06:06 PM

Category: Glomerular Diseases

• 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

• Bhutani, Gauri, University of Wisconsin, Madison, Wisconsin, United States

Gauri Bhutani, MBBS

• Lau, Wai Lang, Columbia University College of Physicians and Surgeons, Scarsdale, New York, United States

Wai Lang Lau,

• Drosou, Maria Eleni, Mayo Clinic, Rochester, Minnesota, United States

Maria Eleni Drosou,

• Nasr, Samih H., Mayo Clinic, Rochester, Minnesota, United States

Samih H. Nasr,

• Astor, Brad C., University of Wisconsin, Madison, Wisconsin, United States

Brad C. Astor,

• Appel, Gerald B., Columbia University College of Physicians and Surgeons, Scarsdale, New York, United States

Gerald B. Appel,

• Leung, Nelson, Mayo Clinic, Rochester, Minnesota, United States

Nelson Leung,

Background

Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits (PGNMID) usually results from an occult hematologic clone, making treatment challenging.

Methods

The Mayo Clinic pathology and Columbia U. Glomerular Ctr database were used to identify patients with PGNMID. Response to each treatment (Rx) regimen for native renal disease was evaluated. "Renal response" was defined as decrease in proteinuria (UPRT) by >50% without worsening of serum creatinine (Scr); "complete remission" as return to within 10% of normal Scr with <1 g/d UPRT; "renal progression" as reaching ESRD, death or need for another Rx. Chi-square, Fisher's exact and Log-rank test were utilized for statistical analysis.

Results

Rx outcomes in 83 PGNMID patients were evaluated. Most had no known hematologic clone (87%). Median Scr at presentation was 1.6 mg/dL (1.2-2.3) with UPRT 4.12 g/d (2.71-8.07). Over a median follow up period of 2.8 yrs, 30% (25/83) reached ESRD, 5% (4/83) died and a median of 2 (1-3) and total 139 Rx were received.
"Renal response" differed significantly between the Rx regimens (p=0.012). This was primarily due to worse outcomes with conservative Rx and a superior response with cyclophosphamide (Table).
“Renal progression” occurred with 60% Rx regimens over a median time of 216 days (104-545). Time to progression was lesser with conservative vs immunosuppressive Rx (p=0.012) but not different among the 5 immunosuppresive regimens (p=0.369). Anti-plasma cell Rx was less often used as 1^st Rx as compared to other regimens (p=0.011).

Conclusion

PGNMID results in poor renal outcomes especially when immunosuppression is not used. Cyclophosphamide was most likely to lead to renal remission in our cohort but surprisingly, anti-plasma cell Rx, which is most appropriate targeted therapy in expert opinion, was not found to have improved outcomes. This may be due to use of this Rx later in disease course. Further prospective studies are needed to identify the best Rx in PGNMID.