Differentiating scleroderma renal crisis from other causes of thrombotic microangiopathy in a postpartum patient.

Clinical Nephrology
Muaz AbudiabSuzanne M Norby

Abstract

Thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), and scleroderma renal crisis (SRC) all present with features of thrombotic microangiopathy. Distinguishing among these entities is critical, however, as treatments differ and may be mutually exclusive. We describe the case of a 25-year-old woman with an undefined mixed connective tissue disease who presented 6 weeks post-partum with fever, transient aphasia, thrombocytopenia, hemolytic anemia, and acute kidney injury eventually requiring initiation of hemodialysis. Renal biopsy revealed thrombotic microangiopathy. Renal function did not improve despite immediate initiation of plasma exchange, and an angiotensin-converting enzyme (ACE) inhibitor was initiated following discontinuation of plasma exchange. At last follow up, she remained dialysis dependent. Due to the myriad causes of thrombotic microangiopathy and potential for diagnostic uncertainty, the patient's response to therapy should be closely monitored and used to guide modification of therapy.

Citations

Jan 1, 2012·Annals of Vascular Diseases·Akihiro IshizuYoshiharu Amasaki
May 12, 2019·Internal and Emergency Medicine·Tim MontriefBrit Long
May 26, 2018·Jornal brasileiro de nefrologia : ʹorgão oficial de Sociedades Brasileira e Latino-Americana de Nefrologia·Roman ZuckermanTushar Vachharajani
Nov 21, 2020·Internal Medicine Journal·Yun Hui Sheryl WongJohan Rosman
Oct 27, 2020·KI Reports·Lilian Monteiro P PalmaSanjeev Sethi

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Methods Mentioned

BETA
biopsy
light microscopy

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