ASN's Mission

ASN leads the fight to prevent, treat, and cure kidney diseases throughout the world by educating health professionals and scientists, advancing research and innovation, communicating new knowledge, and advocating for the highest quality care for patients.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on Twitter

Kidney Week

Abstract: PO1951

Pauci-Immune Lupus Nephritis: A Case Report

Session Information

Category: Trainee Case Report

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Suryadevara, Bharath, Dr. Pinnamaneni Siddhartha Institute of Medical Sciences & RF, Gannavaram, Andhra Pradesh, India
  • Anne, Anvita, Dr. Pinnamaneni Siddhartha Institute of Medical Sciences & RF, Gannavaram, Andhra Pradesh, India
  • Bandi, Varun kumar, Dr. Pinnamaneni Siddhartha Institute of Medical Sciences & RF, Gannavaram, Andhra Pradesh, India
Introduction

Systemic lupus erythematosus (SLE) is an autoimmune disease with multisystem involvement, and Lupus nephritis (LN) typically shows immune deposits on biopsy. Pauci-immune LN is a rare entity.

Case Description

A 35 year old female presented with pedal edema, reduced urine output & yellowish discoloration of eyes since 20 days.No vomiting, dyspnea, joint pains, rashes, or hematuria. She had similar episodes in 2011 and 2013 & was given blood transfusion and oral steroids. She is a hypertensive for 10 years and diabetic for 2 years. She had 2 normal vaginal deliveries with no obstetric complications. On examination she had pallor, icterus and generalized edema, blood pressure of 170/80mmHg. Rest of the examination was normal.
She had severe anemia, renal failure, positive ANA, dsDNA and direct Coomb’s test, with normal complements. There was no evidence of active hemolysis. SLE with Auto immune hemolytic anemia (AIHA) and probable lupus nephritis (LN) was diagnosed. Steroid pulse was started with stabilization of renal function and hemoglobin.
Renal biopsy showed necrotising crescentic glomerulonephritis with no endocapillary proliferation. Immunofluorescence did not show any immune deposits. A diagnosis of pauci immune LN was made, and was started on cyclophosphamide. She had partial renal recovery with creatinine of 1.5 mg/dl, no hematuria, no hemolysis.

Discussion

Renal biopsy in SLE patients can reveal varied pathologies like ANCA or lupus vasculitis etc. In our case, renal biopsy was similar to ANCA vasculitis, however systemic features favored SLE. With a diagnosis of SLE, and absence of endocapillary proliferation, a diagnosis of pauci-immune LN was made.

Hemoglobin(g/dl)4.9
WBC count(cells/mm3)10400
Platelets(lakh/mm3)3.3
Urinalysis3+ protein, 8 RBC, 14 WBC
Creatinine(mg/dl)4.4
T.Bili(mg/dl)1.1
Albumin(g/dl)3.5
LDH(U/I)191
Reticulocyte count6%
TSH(mIU/L)3.7
C3(mg/dl)103
C4(mg/dl)23
Anti-dsDNA (IU/ml)682.7
ANA53.81
Ultrasound abdomenHepatosplenomegaly with cholelithiasis