Case 15: Steroid-resistant nephrotic syndrome in a patient who wishes to become pregnant.
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.
Joseph KupfermanCase Presenter | Beth Israel Deaconess Medical Center, Boston, USA | No conflict of interest reported
Helmut RennkeNephropathology Discussant | Brigham and Women's Hospital, Boston, MA, USA | No conflict of interest reported
Samer NasserNephrology Discussant | Beth Israel Deaconess Medical Center, Boston, USA | No conflict of interest reported
Adib KhouzamiHigh Risk Maternal Fetal Medicine Discussant | Johnstown, PA, USA | No conflict of interest reported
Patient is a 39-year-old female with a history of nephrotic syndrome who wishes to become pregnant and is planning IVF. Several years ago, she developed nephrotic syndrome with anasarca, serum albumin of 1.8 g/dl, nephrotic range proteinuria, and serum creatinine level of 0.6 mg/dl.
She had a kidney biopsy at the time of initial presentation many years ago: none of the glomeruli were sclerotic, there was diffuse foot process effacement. The diagnosis was consistent with minimal change.
She was treated with prednisone 60 mg/day, but she did not have a good response to therapy.
Few months after steroid initiation, her serum albumin declined further to 1.7 g/dl with a urine protein/Cr ratio of >10; creatinine was 0.7 mg/dl.
Approximately 6 months later, she presented with anasarca (~20 kg weight gain) and pulmonary edema. She had a complicated one-month hospital admission, where she was treated with diuretics and glucocorticoids but her serum albumin remained low at 1.7 and serum creatinine now was elevated at 1.8 mg/dl. Her admission was complicated by infection and other severe steroid side effects.
A few months later she was rehospitalized.
Labs on admission included: Serum albumin 1.5 mg/dl, creatinine 2.2 mg/dl, urine protein 8 grams/day; She was diagnosed with steroid-induced diabetes, and on the exam with anasarca, including massive ascites. She was given prednisolone but this was gradually tapered and cyclosporine was started.
She was also treated with losartan, spironolactone, atorvastatin, and warfarin. She also underwent LDL apheresis. At this point the presumed diagnosis was FSGS.
She took different doses of prednisolone and cyclosporine over the years. ARB and warfarin were stopped 2 years later.
More recently she has been off all medications.
She feels well.
She continues to check her urine by dipstick at home and has had little or no proteinuria recently.
Medical history: Steroid induced diabetes Bacteremia Obesity
Physical Exam: Currently BP 130/82. Exam essentially normal. No edema.
Laboratory and other data: Normal white and red blood cell count, normal electrolytes Creatinine 0.7 mg/dl Total cholesterol 181 mg/dl Serum albumin 4.1 g/dl C3: 80 mg/dL (normal 90-160); C4 20 mg/dL (normal 14-30) Urine protein to creatinine ratio 0.5 g/g
Pathology images pending
Minimal change disease with high degree foot process effacement, without immune complex deposition.
Questions posed & summary of key discussion points
1. The advice to give on the risks of pregnancy in light of kidney disease? 2. How to monitor the patient throughout the pregnancy? 3. Discussions around use of ACEi/ARB in childbearing age.
Author(s) of case summary:
Case summary pending
If you would like to be a contributing author for the creation of didactic materials and case summaries, please contact us at [email protected].