Kidney Week

Abstract: PUB401

Light Deposited All Over (Lighted All Over)

Session Information

• Publication Only

Category: Glomerular Diseases

• 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

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

Fnu Pariya, MD

• 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

• 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

Light-chain deposition disease (LCDD) is the deposition of monoclonal light chains in multiple organs. It is a rare disease characterized by deposition of nonamyloid immunoglobulin light chains, which do not stain with Congo red and do not exhibit a fibrillar structure when examined ultra structurally. The incidence of LCDD is unknown.

Case Description

76-year-old man with history of HTN, HLD, CVA, CKD prostate cancer s/p radical prostatectomy, and severe IPF with light smoking history. He came to ED for raised creatinine thought due to chlorthalidone use. Systemic review revealed nocturia, four times a night, more than usual. Vitals were normal. Labs showed BUN/Cr of 41/2.7, no electrolyte abnormality, ALP 499, AST 151, ALT 232, GGT 770, CBC normal except eosinophils at 9%, ANA positive 1: 80, negative for Hepatitis A, B, C, AMA, AntiSm, Urine protein initially 30. He was discharged with outpatient follow ups. GI considered DILI but with worsening LFT requested liver biopsy. Biopsy showed mild hepatitis with biliary ductal inflammation, sclerosing cholangitis with portal and peri portal fibrosis. Other labs showed Bence Jones proteins on UPEP, k/l ratio of 19, no monoclonal spike. UPCR 197g/day. Renal ultrasound was normal. MRCP showed no liver or biliary pathology but slight prominence of pancreatic duct in the head of pancreas. Fat pad biopsy was negative for amyloid. Renal labs and LFT continued to be elevated hence tried on ursodiol. Rheumatology followed for eosinophilia without asthma or allergy. Autoimmune work up including ACE, Vitamin D ratio, lysozyme, SSA, SSB, centromere, Scl70, C3, C4, dsDNA was negative. Extensive infectious work up was negative. A bone marrow biopsy showed 15% plasma cell with kappa restriction. Heme started him on high dose steroids and Bortezomib with prophylaxis. LFT Down trended with chemotherapy however, the kidney function continued to worsen, and patient had to be initiated on hemodialysis.

Discussion

Symptomatic extrarenal LCDD is rare. We present a case with involvement of multiple organ systems. Liver with biliary ductal involvement with fibrosis. Pulmonary with interstitial fibrosis. Cardiac with diastolic dysfunction. Other systems likely involved include pancreas with a ductal prominence, and possible peripheral neuropathy. To our knowledge, this is the first ever case involving so many organ systems.