French National Cohort MATRIX "Renal and Systemic Thrombotic Microangiopathy"

Last updated: August 10, 2023
Sponsor: University Hospital, Tours
Overall Status: Active - Recruiting

Phase

N/A

Condition

Anemia

Renal Failure

Kidney Disease

Treatment

Collecting datas

Clinical Study ID

NCT05991245
RNI/MATRIX
2022-59
F20221110095846
  • Ages > 18
  • All Genders

Study Summary

Thrombotic microangiopathies (TMAs) are a diverse, rare but serious group of diseases. Progress has been made regarding the epidemiology of TMA (Bayer CJASN 2019). It has been shown that secondary TMAs account for 95% of cases, whereas primary TMAs (atypical hemolytic syndromes (HUS) and thrombotic thrombocytopenic purpura (TTP)) account for only about 5%.

However, in many cases, the pathophysiology, optimal management and prognosis of TMA remains unclear and it has been shown that patients with TMA may have renal-limited TMA or renal and hematological TMA (ie. With (mechanical anemia, thrombocytopenia, elevated LDH, decreased haptoglobin, schistocytes). In most studies, kidney biopsies are not performed and the diagnostic workup is uncomplete.

As this is a rare disease, only a multicenter approach (>20 centers) over a long period of time (>10 years), with adequate diagnostic workup including kidney biopsies can help us to answer these questions (investigators in the present are usually members of the CNR-MAT (a network of the TMA centers in France).

Eligibility Criteria

Inclusion

Inclusion Criteria:

  1. Adult patients 18 years of age or older
  2. Who have undergone renal biopsy of the native kidney for impaired renal functionbetween 2009 and July 2022.
  3. Presence of classically defined systemic MAT (most of the following parameters:elevated LDH, decreased haptoglobin, schizocytes, thrombocytopenia and anemia) AND/ORpresence of arteriolar or glomerular renal MAT as indicated by the pathologist (including endothelial turgor, mesangiolysis, double contours, presence of thrombi,fibrinoid necrosis of the arterial wall).

Exclusion

Exclusion Criteria:

  1. Kidney transplantation

Study Design

Total Participants: 1000
Treatment Group(s): 1
Primary Treatment: Collecting datas
Phase:
Study Start date:
January 01, 2023
Estimated Completion Date:
January 31, 2025

Study Description

Thrombotic microangiopathies (TMAs) are a diverse, rare but serious group of diseases. Their epidemiology has recently been elucidated thanks to work published by our team. It has been shown that secondary TMAs account for 95% of cases, whereas primary TMAs (atypical hemolytic syndromes (HUS) and thrombotic thrombocytopenic purpura (TTP)) account for only about 5%.

However, many epidemiologic problems remain. First, many but not all patients with TMA as classically defined (mechanical anemia, thrombocytopenia, elevated LDH, decreased haptoglobin, schizocytes) have impaired renal function. If a renal biopsy is performed (which is not always the case, as TMA is a risk factor for bleeding after renal biopsy), the renal lesions do not always show "renal-limited" TMA. We also do not know which of the causes of systemic TMA are associated with renal TMA and which are not.

Conversely, in patients with renal biopsy, we can find stigmata of renal-limited TMA in the absence of systemic TMA. Why do some patients have systemic TMA but not renal TMA and others have renal TMA but not systemic TMA? Most studies are based on a few small clinical cases and the literature reviews that report them.

The vital, renal, and cardiovascular prognosis of patients with TMA obviously depends on the cause, the clinical presentation of the patients, and their management. However, the renal, systemic, and vital outcomes of renal-only vs. systemic-only vs. systemic and renal TMA, regardless of the cause and severity of TMA, are currently unknown.

As this is a rare disease, only a multicenter approach (>20 centers) over a long period of time (>10 years), including highly recruited university and general hospitals, with experienced and motivated investigators, can help us to answer these currently unanswered questions (these investigators usually belong to the competence centers of the national reference center).

Connect with a study center

  • Department of Nephrology, University Hospital of Tours

    Tours, 37044
    France

    Active - Recruiting

Not the study for you?

Let us help you find the best match. Sign up as a volunteer and receive email notifications when clinical trials are posted in the medical category of interest to you.