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Two cases of idiopathic steroid-resistant nephrotic syndrome complicated with thrombotic microangiopathy

Kentaro Nishi¹, Mai Sato¹, Masao Ogura¹

¹Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.

|August 5, 2020

Abstract

Background:

Thrombotic microangiopathy (TMA) is a histopathological entity associated with microangiopathic hemolytic anemia, thrombocytopenia, and end-organ ischemic damage. Although TMA is caused by various diseases, there have been few reports regarding children with idiopathic nephrotic syndrome (NS) and TMA. Here we report two 1-year-old infants with steroid-resistant NS (SRNS) who presented with severe hypertension, acute kidney injury (AKI), and TMA.

Case Presentation:

The diagnosis of NS was complicated with anemia, AKI, and hypertension. Maximum blood pressure was 150/70 mmHg in Case 1 and 136/86 mmHg in Case 2. There was no thrombocytopenia during their clinical course in both cases. Renal biopsy showed the features of TMA, including endothelial cell swelling, capillarectasia or marked mesangiolysis, along with mesangial proliferation in Case 1 and TMA with minor glomerular abnormalities in Case 2. Hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, and secondary TMA other than that caused by hypertension were excluded. Oral prednisolone therapy, frequent infusion of albumin and diuretics, and multiple anti-hypertensive drugs were initiated. Blood pressure was controlled after 6 and 7 days from initiation of multiple anti-hypertensive drugs and lisinopril was added due to persistent mild proteinuria and mild hypertension after improvement of renal function in both cases. Proteinuria resolved completely 4 months after admission with daily oral prednisolone for 4 weeks followed by alternative daily oral prednisolone for 4 weeks in Case 1. Proteinuria resolved completely 10 months after admission with initial prednisolone treatment for 4 weeks followed by cyclosporine A and intravenous methylprednisolone pulse therapy in Case 2. The follow-up biopsy showed no TMA findings in both patients. Because the patient in Case 1 subsequently developed frequent relapsing NS, cyclosporine A was commenced after the second biopsy and he did not have any flares for 2 years. Renal function was normal in Case 1 and mildly decreased in Case 2 at last follow-up (creatinine-eGFR of 136.2 mL/min/cm² in Case 1 and 79.5 mL/min/cm² in Case 2).

Conclusion:

Severe hypertension and AKI can be signs of TMA in patients with SRNS. Strict anti-hypertensive therapy might improve renal outcomes.

Insights

Severe hypertension and acute kidney injury (AKI) in infants with steroid-resistant nephrotic syndrome (SRNS) may indicate thrombotic microangiopathy (TMA). Prompt antihypertensive therapy is crucial for managing TMA and improving kidney outcomes in children.

Area of Science:

Pediatric Nephrology
Hematology
Pathology

Background:

Thrombotic microangiopathy (TMA) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and ischemic organ damage.
Reports of TMA in children with idiopathic nephrotic syndrome (NS) are rare.
This study details two infant cases of steroid-resistant NS (SRNS) presenting with severe hypertension, AKI, and TMA.

Observation:

Two 1-year-old infants with steroid-resistant nephrotic syndrome (SRNS) presented with severe hypertension, acute kidney injury (AKI), and TMA.
Despite the absence of thrombocytopenia, renal biopsies confirmed TMA features, including endothelial cell swelling and mesangiolysis.
Treatment involved antihypertensive drugs, albumin, diuretics, and corticosteroids, leading to blood pressure control and proteinuria resolution.

Findings:

Severe hypertension and AKI were key indicators of TMA in these pediatric SRNS cases.
Aggressive antihypertensive therapy was essential for managing the clinical presentation and improving renal function.
Complete resolution of TMA findings on follow-up biopsy was observed in both patients.

Implications:

Early recognition of severe hypertension and AKI as potential TMA signs in pediatric SRNS is critical.
Strict adherence to antihypertensive regimens may significantly improve renal outcomes in children with SRNS-associated TMA.
This case series underscores the importance of prompt diagnosis and management of TMA in infants with steroid-resistant nephrotic syndrome.

Read full analysis

Keywords:

Acute kidney injury (AKI)Hypertension Steroid-resistant nephrotic syndrome (SRNS)Thrombotic microangiopathy (TMA)

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