To preserve patient privacy and for didactic purposes, case descriptions and pathology reports have been anonymized and partially fictionalized. The pathology images are representative images from a mixture of similar cases.

Invited Speakers

Franco Cabeza-Rivera
Franco Cabeza-RiveraCase Presenter | University of Mississippi Medical Center, Jackson, MS, USA | No conflict of interest reported
Satya Bhupathi
Satya BhupathiCase Presenter | University of Mississippi Medical Center, Jackson, MS, USA | No conflict of interest reported
Jorge Castaneda
Jorge CastanedaCase Presenter | University of Mississippi Medical Center, Jackson, MS, USA | No conflict of interest reported
Helmut Rennke
Helmut RennkeNephropathology Discussant | Brigham and Women’s Hospital, Boston, MA, USA | No conflict of interest reported
Manish Maski
Manish MaskiNephrology Discussant | Sanofi Genzyme, Boston, MA, USA | No conflict of interest reported

Case

Patient is a 30-year-old female who was noted to have increased proteinuria during an outpatient visit with Rheumatology. She has known history of SLE, no prior renal biopsy was done. Patient being controlled on Hydroxychloroquine and Azathioprine. Patient was on Prednisone 40 mg daily for lupus related myositis (elevated CK and Aldolase). 

Medical history:
SLE (dx in 2001, ANA +, SSA +)
Myositis
Hx of PE (Lupus Anticoagulant +)
Asthma
Chronic Allergic Rhinitis
Hypertension
Microscopic Hematuria

Medications:
Azathioprine
Diltiazem CD
Fluticasone-Salmeterol inhaler
Hydrochlorothiazide
Hydroxychloroquine
Prednisone 40 mg po daily
Warfarin

Family History:
Multiple family members with SLE
HTN
No known kidney disease

Physical Exam:
T: 99F / 38C, BP: 127/88, HR: 76, RR: 16, SpO2: 99% on RA
Weight: 246lb / 111.6kg
General: NAD, Moderately Obese
Eyes: EOMI, PERRL, No Icterus
ENT: No Ulcers, Dentition normal
Neck: Normal ROM, No Thyromegaly/Lymphadenopathy
CV: RRR, No m/r/g
Lungs: CTAB, No Wheeze
Abdomen: Soft, non-tender, no masses
Neuro: AAO, Strength: UE 5/5, LE: 4/5 Proximal, 5/5 Distal
MSK: No synovitis, Normal ROM all joints w/o effusions
Ext: 1+ Edema, symmetric

Laboratory and other data:
WBC: 12 k/microliter, Hb: 15 mg/dl, Hct: 44 %, Platelets: 250 k/microliter
BUN: 14, serum creatinine: 0.8 mg/dl
Serum albumin: 3.5 g/dl
C3 – 133 (90-180 mg/dL)
C4 – 20 (10-40 mg/dL)
ANA+
dsDNA, SM Ab, HIV, Hep B/C Negative
CK 1356 (26-140 U/L)
SPEP & IFE negative
Serum kappa 10 (3.3-19.4 mg/L); Lambda 9 (5.7 -26.3 mg/L); ratio 1.2 (normal ratio)
Urinalysis:
SG: 1.027, Moderate Small, Protein >300mg/dL, no WBC, RBC: 50-100
>Urinary protein to creatinine ratio: 1.4 g/g
24 Hour Urine Protein: 2.4 gm/day
Renal US: Unremarkable

Short term follow up and additional work up:
Hydroxychloroquinine stopped
Urinary protein to creatinine ratio 0.5 g/g
Plasma globotriaosylsphingosine (lysoGb3) levels normal
Genetic testing negative

Kidney Pathology

Pathology images pending

Fabry’s Disease like lesions (myeloid bodies / zebra bodies)

Few mesangial deposits in occasional mesangial area, rare subepithelial deposits with rare TRIs compatible with minimal (Class I LN).

Questions posed & summary of key discussion points

1. How would you approach further diagnosis & management of this patient?

Author(s) of case summary: