Kidney Week

Abstract: PUB399

A Rare Presentation of Primary Systemic Lupus Erythematosus (SLE) Tubulointerstitial Nephritis (TIN)

Session Information

• Publication Only

Category: Glomerular Diseases

• 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

• Sasidharan, Sandeep Raja, SUNY Downstate Health Sciences University, New York City, New York, United States

Sandeep Raja Sasidharan, MD

• Jatoi, Tahir Ahmed, SUNY Downstate Health Sciences University, New York City, New York, United States

Tahir Ahmed Jatoi, MBBS

• Pariya, Fnu, SUNY Downstate Health Sciences University, New York City, New York, United States

Fnu Pariya, MD

• Grossman, Susan, VA New York Harbor Healthcare System, New York, New York, United States

Susan Grossman, MD

• Michel, Marie-Alex, VA New York Harbor Healthcare System, New York, New York, United States

Marie-Alex Michel, MD

Introduction

SLE is an inflammatory disease with systemic effects, and 50% of them present with signs of nephropathy, which involve the parenchyma's glomerular, vascular, and tubular aspects.TIN is a well-recognized feature of lupus nephritis (LN) in 66% of SLE kidney biopsies (KB). SLE TIN is recognized in two forms, i.e., secondary, coexisting with lupus glomerulonephritis, and primary: develops with no or mild glomerular or vascular involvement. We present a rare case of primary SLE TIN in an elderly male.

Case Description

A 76-year-old male with a history of MI with PCI, HTN, COPD, h/o CVA, resolved DM2, h/o AKI d/t prerenal/over diuresis, and BPH.
He presented with 3 days of intense red rash on his face and bilateral hands associated with pruritis and facial puffiness. No h/o NSAID use, beta lactams. Hydrochlorothiazide was stopped as a source. However, he continued to have persistent proteinuria > 2 g/d. The lab was positive for ANA 1:320, EBV IgG>600, EBV nuclear antigen 544, ESR 74, Beta2microglobulin 2.7, and IgG 544, but IgG subclasses normal, SPEP showed the faint band in the gamma region, kappa 27.2,, lambda 20.4, ratio 1.33, rest of nephritic panel was normal. UPCR started at 1.2g/d and trended up to 4.5g/d.

KB showed mild mesangial hypercellularity, moderate foot process effacement, and chronic interstitial nephritis with associated tubular atrophy. Before a skin biopsy, he had another rash episode in sun-exposed areas of bilateral shin with persistent proteinuria. He was started on prednisone with a diagnosis of SLE TIN, with improvement in his renal function and proteinuria.

Discussion

Primary SLE TIN is rare, with only 16 reported cases in the literature. In our patient, the diagnosis was challenging, with differential including AIN, Schnitzler syndrome, dermourticariopathy with renal dysfunction, and IgG4 nephropathy. Finally diagnosed with SLE after fulfilling 4 out of 11 criteria per ACR. To our knowledge, our patient is the first to have nephrotic range proteinuria, with KB showing both foot process effacement and mesangial hypertrophy. As seen with our patient, primary SLE TIN is known to have a good response to steroids with a good prognosis as seen with our patient. We report this case to increase awareness of this unique diagnosis.