Kidney Week

Abstract: PUB372

Type B Insulin Resistance Syndrome Associated with Lupus Nephritis

Session Information

• Publication Only

Category: Glomerular Diseases

• 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

• Qasim, Muhammad, University of Kentucky College of Medicine, Lexington, Kentucky, United States

Muhammad Qasim, MD

• Mustafa, Kanza, Yusra Medical and Dental College, Islamabad, Pakistan

Kanza Mustafa, MBBS

• Finke, Ann R., University of Kentucky College of Medicine, Lexington, Kentucky, United States

Ann R. Finke, MD

• Sims, Tyler, University of Kentucky College of Medicine, Lexington, Kentucky, United States

Tyler Sims, MD, CDE

• Alagusundaramoorthy, Sayee Sundar, University of Kentucky College of Medicine, Lexington, Kentucky, United States

Sayee Sundar Alagusundaramoorthy, MD, MBBS, FASN

• Ayach, Taha, University of Kentucky College of Medicine, Lexington, Kentucky, United States

Taha Ayach, MD, FASN

• Cornea, Virgilius, University of Kentucky College of Medicine, Lexington, Kentucky, United States

Virgilius Cornea, MD

Introduction

Type B insulin resistance(TBIR) syndrome is a rare disease entity caused by IgG polyclonal Ab that antagonize the insulin receptor. Systematic review in 2020, 119 cases described in literature. 50% have coexisting autoimmune disorder. 35% associated with systematic lupus erythematosus (SLE).

Case Description

33 yo M with no past medical history presented with 1 week of hematuria, fatigue, polyuria, polydipsia admitted for new onset diabetes. Progressed to DKA with uncontrolled hyperglycemia. Labs C3 34, C4 7, CRP 15.5 mg/L, ANA Speckled 1:1280. RF <10. Anti DNA Screen 1:80, Smith Ab IgG 61, SmRNP Ab IgG 143. Glutamate Decarboxylase Ab <5. Insulin Ab 0.4 [0-0.4 U/mL]. HgbA1c 8.6%. Developed AKI Stage III; Cr 0.74 mg/dL on admit, peaked at 2.49 mg/dL. Renal biopsy(Fig 1):mesangial proliferative lupus nephritis(LN). Revised lupus classification, Class II. NIH protocol initiated, 40mg dexamethasone. Required up to 215,000 U Insulin/day, then dexamethasone held for hyperglycemia. Initiated cyclophosphamide(CYC)15 mg/kg. Plasmapheresis(PLEX) initiated as per NIH recommendations. Rituximab administered with PLEX session 3 and 6. 11 days of PO CYC + 2^nd pulse dose dexamethasone. Relapsed 1 month after PLEX with persistent hyperglycemia and required 5 more PLEX sessions. He had 2 more courses of dexamethasone. 5 months post diagnosis remains stable on outpatient management: U500 Insulin 1400 U 3x daily.

Discussion

We present the complexity of managing a rare case of TBIR syndrome coalesced with overlapping SLE/LN and the challenges related to pulse dose steroids, plasmapheresis and immunosuppressants. Renal biopsy of LN strengthened our suspicion for TBIRS. Inability to tolerate glucocorticoids due to hyperglycemia. Treatment also consisted of CYC, PLEX, Rituximab. AKI resolved with CYC, PLEX x 6, 1 dose of Rituximab and dexamethasone pulse.

Fig1
A-H&E ATN, B-PAS Hypercellular mesangium, C-D: EM Mesangial + Subendothelial dense deposit. E-I: IF Full House