The case

A 28-year old woman presented with a new-onset nephrotic syndrome at 16 weeks of gestation.

History

Medical history was unremarkable except for infertility. Her birthweight was within the normal range.

Physical investigations

Her height was 156 cm, blood pressure was not high, and she weighed 53.3 Kg. She had remarkable oedema in her lower extremities. She did not have sclerodactyly.

Examinations

Laboratory tests were done; serum total protein and albumin levels were very low; serum creatinine and uric acid were slightly high owing to her gestation. Serum total cholesterol, low density lipoprotein cholesterol, and triglycerides were 510, 329, 439 mg/dL, respectively. C-reactive protein was 0.37 mg/dL, and urine protein content was 5.6 g/day, with no haematuria. Antinuclear antibody was low titre and anti-centromere antibody was positive at 29.3 IU/mL. In addition, anti-dsDNA, anti-β-2-glycoprotein I antibody, and anti-phospholipid IgG antibody, lupus anticoagulant wasnegative. Further protein S activity was in the normal range.

Biopsy

5 Weeks after delivery, a kidney biopsy was performed. The core kidney tissue contained 21 glomeruli without global sclerosis. Two of the 21 glomeruli showed focal segmental sclerotic lesions, with epithelial cell hyperplasia and foam cells in the tubular poles. Additionally, hyaline arteriosclerosis and intimal thickening in the interlobular arteries were not found.

What is your answer?

1. What diagnostic criteria does this patient meet?
2. What would be the management strategy considering she is pregnant?

Case sourced from: Suzuki T, Ichikawa D, Nakata M, Watanabe S, Han W, Kohatsu K, Sayuri S, Imai N, Koike J, Shibagaki Y. Nephrotic syndrome due to preeclampsia before 20 weeks of gestation: a case report. BMC Nephrology. 2020; 21; Article number:240. https://doi.org/10.1186/s12882-020-01876-9