Abstract

Background: Early diagnosis of thrombotic thrombocytopenic purpura (TTP) versus hemolytic and uremic syndrome (HUS) is critical for the prompt initiation of specific therapies. Objective: To evaluate the diagnostic performance of the proteinuria/creatininuria ratio (PU/CU) for TTP versus HUS. Patients/Methods: In a retrospective study, in association with the “French Score” (FS) (platelets < 30 G/L and serum creatinine level < 200 µmol/L), we assessed PU/CU for the diagnosis of TTP in patients above the age of 15 with thrombotic microangiopathy (TMA). Patients with a history of kidney disease or with on-going cancer, allograft or pregnancy were excluded from the analysis. Results: Between February 2011 and April 2019, we identified 124 TMA. Fifty-six TMA patients for whom PU/CU were available, including 35 TTP and 21 HUS cases, were considered. Using receiver–operating characteristic curves (ROC), those with a threshold of 1.5 g/g for the PU/CU had a 77% sensitivity (95% CI (63, 94)) and a 90% specificity (95% CI (71, 100)) for TTP diagnosis compared with those having an 80% sensitivity (95% CI (66, 92)) and a 90% specificity (95% CI (76, 100) with a FS of 2. In comparison, a composite score, defined as a FS of 2 or a PU/CU ≤ 1.5 g/g, improved sensitivity to 99.6% (95% CI (93, 100)) for TTP diagnosis and enabled us to reclassify seven false-negative TTP patients. Conclusions: The addition of urinary PU/CU upon admission of patients with TMA is a fast and readily available test that can aid in the differential diagnosis of TTP versus HUS alongside traditional scoring.

Highlights

  • Our understanding of the mechanisms driving thrombotic microangiopathies (TMA)pathology has greatly improved over the past two decades

  • TMA subtypes has played a central role in the development of therapies, with eculizumab used as first-line therapy for CM-hemolytic and uremic syndrome (HUS) [4], compared with a combination of plasma exchange, rituximab and caplacizumab for the treatment of acquired thrombocytopenic purpura (TTP) [5,6,7]

  • ADAMTS13-detectable patients werefurther classified as either STEC-HUS for patients who tested positive for Shigatoxin in stool or positive Shigatoxin-secreting E.coli serology or complement-mediated HUS (CM-HUS) in cases of confirmed or suspected abnormal regulation of alternative complement pathway [13]

Read more

Summary

Introduction

Our understanding of the mechanisms driving thrombotic microangiopathies (TMA)pathology has greatly improved over the past two decades. TMA subtypes has played a central role in the development of therapies, with eculizumab used as first-line therapy for CM-HUS [4], compared with a combination of plasma exchange, rituximab and caplacizumab for the treatment of acquired TTP [5,6,7]. Given these divergent treatments and the pace of disease progression, the ability to differentiate. Diagnosis of thrombotic thrombocytopenic purpura (TTP) versus hemolytic and uremic syndrome (HUS) is critical for the prompt initiation of specific therapies

Methods
Results
Conclusion
Full Text
Published version (Free)

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call