Successful Remission of Non-Lupus Near Full-House Nephropathy with Voclosporin: A Novel Therapeutic Strategy in a Pregnancy Planning Context

 

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https://storage.unitedwebnetwork.com/files/1099/4cd2d955cfd53dcc947a2bbe77ced9e6.pdf
Successful Remission of Non-Lupus Near Full-House Nephropathy with Voclosporin: A Novel Therapeutic Strategy in a Pregnancy Planning Context

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Chisato
Takahashi
Chisato Takahashi c-taka@dokkyomed.ac.jp Dokkyo Medical University Nephrology and Hypertension Tochigi Japan *
Erika Hishida e-hishida@dokkyomed.ac.jp Dokkyo Medical University Nephrology and Hypertension Tochigi Japan -
Kazutoshi Ono r1506ok@jichi.ac.jp Jichi Medical University Division of Nephrology, Department of Internal Medicine Tochigi Japan -
Yuko Ono o-yuko@dokkyomed.ac.jp Dokkyo Medical University Pathology Tochigi Japan -
Tatemitsu Rai t-rai@dokkyomed.ac.jp Dokkyo Medical University Nephrology and Hypertension Tochigi Japan -
Daisuke Nagata nagatad@jichi.ac.jp Jichi Medical University Division of Nephrology, Department of Internal Medicine Tochigi Japan -
 
 
 
 
 
 
 
 
 

Non-lupus full-house nephropathy (NLFHN) is characterized by lupus-like immune complex deposition in the kidneys without systemic lupus erythematosus (SLE). Recent systematic analyses (Clin J Am Soc Nephrol 2024; 19: 743–754) hae identified NLFHN as a distinct clinicopathologic entity, often exhibiting "near full-house" patterns lacking IgA and with weak IgM. Evidence for the use of voclosporin in SLE-negative kidney-limited disease is limited, and management can be challenging when pregnancy is planned because of the teratogenicity of mycophenolate mofetil (MMF). This case illustrates that voclosporin is a safer alternative for pregnancy-oriented immunosuppression. 

A 37-year-old woman presented with nephrotic-range proteinuria during routine screening. Urinalysis showed protein 7.17 g/gCr, positive occult blood, and 10–19 RBCs/HPF, with normal renal function. The results for autoantibodies, cryoglobulins, and hepatitis B/C antigens were negative. Complement C3/C4 levels were normal, with CH50 mildly decreased. A kidney biopsy (day 0) revealed diffuse endocapillary hypercellularity and subepithelial deposits resembling class IV/V lupus nephritis. Immunofluorescence demonstrated strong IgG, C3, and C1q; weak IgM; and negative IgA, consistent with a "near full-house" pattern. Methylprednisolone (500 mg/day × 3 days; days 64–66) was followed by oral prednisolone (25 mg/day from day 67) plus MMF 1000 mg/day, escalated to 1500 mg/day on day 86. Proteinuria declined rapidly, achieving complete remission by day 70 (UPCR < 0.5 g/gCr). The PSL dosage was tapered to 20 mg (day 121), 15 mg (day 149), 12.5 mg (day 177), 10 mg (day 212), 5 mg (day 261), and 2.5 mg (day 365). voclosporin (7.9 mg twice daily) was introduced on day 247 to facilitate pregnancy planning, followed by gradual MMF dose reduction aiming for complete withdrawal, while voclosporin maintained remission as a bridge therapy. 

After voclosporin initiation, proteinuria remained suppressed, and complete remission persisted for > 12 months. Renal function and complement levels remained stable, without autoantibody seroconversion. Cryoglobulin negativity and the absence of secondary causes supported idiopathic, kidney-limited NLFHN. No treatment-related hypertension, neurotoxicity, or infection occurred. Serial follow-up confirmed sustained UPCR reduction and stable eGFR, demonstrating the long-term safety of voclosporin during MMF tapering. These observations support recent reports that "near full-house" patterns represent a distinct lupus-like glomerulopathy with non-systemic behavior under targeted immunosuppression. 

This SLE-negative case (IgA–, IgM±) achieved early and sustained complete remission (day 70, UPCR < 0.5 g/gCr) through a pregnancy-conscious sequential regimen. voclosporin enabled progressive MMF reduction and safe transition toward preconception while preserving renal stability with low-dose steroids. These findings align with the recent systematic review that defines NLFHN as a distinct entity (Clin J Am Soc Nephrol 2024; 19: 743–754), highlighting voclosporin as a pregnancy-compatible, durable immunosuppressive option for rare glomerular diseases.

Kewords