Abstract
Biopsy diagnosis of lupus nephritis with no extra-renal features of systemic lupus erythematosus,
normal
complement levels, negative or weekly positive anti-nuclear antibody and negative
other serology is a
rare entity known as “Renal Limited Lupus Like Nephritis (RLLLN). So far, only 39 cases of RLLLN in
adults have been reported in literature. The prognosis of these patients varied depending on their clinical
presentation. Herein, we report a case of an elderly
Arab female with RLLLN who presented with massive
proteinuria and acute kidney injury with
crescents, however had a good outcome contrary to reported cases
with similar presentations. We also reviewed all reported cases thus far.
Resume
Le diagnostic par biopsie de la néphropathie lupique dépourvue de caractéristiques extra-rénales du lupus
érythémateux disséminé, de niveaux de complément normal, d'anticorps anti-nucléaire négatifs ou
hebdomadaires positifs et d'une autre sérologie est une entité rare appelée « Néphrite Rénale Limitée
semblable au Lupus » (RLLLN). Jusqu'à présent, seuls 39 cas de RLLLN chez les adultes ont été rapportés
dans la littérature. Le pronostic de ces patients variait en fonction de leur présentation clinique. Ici, nous
rapportons le cas d'une femme arabe âgée souffrant de RLLLN qui présentait une protéinurie massive et une
lésion rénale aiguë accompagnée de croissants, mais dont le résultat était satisfaisant contrairement aux cas
rapportés avec des présentations similaires. Nous avons également examiné tous les cas signalés jusqu'à
présent.
Keywords: Renal-Limited Lupus-Like Nephritis, Lupus Nephritis, Full-House pattern
About the Authors
Jumana Amir is a Resident in Internal Medicine at McGill University. Salwa Sheikh is Chief of Pathology - Pathology Services Division
at Johns Hopkins Aramco Healthcare. Abdulrazack Amir is a Nephrologist and Head of Academic Affairs, at Johns Hopkins
Aramco Healthcare.
Correspondence can be directed to: jumana.amir@mail.mcgill.ca
Submitted: March 24, 2018. Accepted: July 5, 2018. Published: February 12, 2019. DOI: 10.22374/cjgim.v14i1.281
Renal Limited Lupus-Like Nephritis – A Case
Report and Review of Literature
Jumana Amir, Salwa Sheikh, Abdulrazack Amir
Lupus nephritis is a common feature of systemic lupus
erythematosus (SLE) usually occurring in young females and
diagnosed by renal biopsy.
1
Only 10 cases have been reported in
adults where patients with lupus nephritis have negative serology
and no extra-renal SLE manifestations. This rare entity is known
as Renal Limited-Lupus-Like Nephritis (RLLLN). Reported
outcomes varied from good to poor. We herein report a case of
an elderly female with Renal-Limited Lupus-Like Nephritis who
presented with massive proteinuria, gross hematuria and acute
kidney injury with crescents. However, she had a good outcome
unlike previous cases with similar presentation.
Case Report
A 63-year-old Arab female with no previous history of renal or
autoimmune disease presented in November 2014 to an outside
facility with 1-day history of gross hematuria and decreased urine
output. On arrival, she was found to have proteinuria. 2days after
presentation, she became anuric with signs of volume overload,
severe hyponatremia, and had worsening renal function requiring
urgent hemodialysis. She was referred to our facility 1 week
after initial presentation for further care and management. The
patient had no history of joint pain/swelling, rash, oral ulcers,
Case Study
Canadian Journal of General Internal Medicine
Volume 14, Issue 1, 2019 29