Informations générales (source: ClinicalTrials.gov)
Identification of Biomarkers Predictive of Worse Prognosis in Henoch Schonlein Purpura
Observational
Assistance Publique - Hôpitaux de Paris (Voir sur ClinicalTrials)
avril 2010
juillet 2014
29 juin 2024
Henoch Schonlein Purpura (HSP), vasculitis of small vessels with deposits of IgA, is
considered by many authors as the systemic form of Berger's disease (IgA-N). IgA-N is
characterized by IgA1 deposits in mesangial areas associated with mesangial
proliferation. These two diseases remain the leading cause of ESRD by primitive
glomerulopathy in Western countries. In recent years, considerable progress has been made
in understanding the pathophysiological mechanisms of IgA-N. However, only a high rate of
proteinuria at one year or the presence of severe glomerular inflammation on renal biopsy
remain predictors of long term renal function. Moreover, the high variability of HSP
clinical expression, from few purpura skin lesions that evolve favourably spontaneously,
to rapidly progressive renal failure, remains so far unexplained but suggests the
existence of individual genetic susceptibility.
In the first part of the study, we will study key factors based on physiopathological
data obtained by our laboratory as well as by other groups. The second part of the study
concerns genetic factors. Although the candidate genes that may confer a particular
susceptibility to the disease, to progress to ESRD or respond to treatment are many, the
genes involved in inflammation or controlling renin-angiotensin system are of particular
interest.
We will apply these results by studying patients with HSP showing three distinct
phenotypes (HSP with isolated cutaneous purpura or associated with minimal or severe
renal disease) at diagnosis and after clinical remission.
The purpose of this study is to assess whether the phenotype at diagnosis is associated
with the physiological markers and if one of them predicts a pejorative evolution of
renal disease at 1 year. Meanwhile, study of polymorphism of selected genes of interest
could allow identification of patients with specific genetic susceptibility or with bad
prognosis factors who would be thus eligible for specific treatment.
Etablissements
| Les établissements hors Île-de-France dont les données sont issues de ClinicalTrials.gov Origine et niveau de fiabilité des données | |||||
|---|---|---|---|---|---|
| Nephrology Unit - Hôpital St Louis - 75010 - Paris - France | Evangeline Pillebout, MD PhD | Contact (sur clinicalTrials) | |||
Critères
Tous
Inclusion Criteria:
- Patients with HSP whose diagnosis was confirmed by histology of an active skin
lesion, who signed the informed consent form or for minors, signature of the holders
of parental authority.
- Patients with HSP whose diagnosis was confirmed by histology of an active skin
lesion, who signed the informed consent form or for minors, signature of the holders
of parental authority.
- Patients who do not have skin lesions; Patients receiving immunosuppressive drugs or
steroids for more than 2 weeks; Patients with another diagnosis
(platelets<100,000/mm3, bacterial purpura, other systemic disease);
- Patients unable to understand the protocol, refusing to sign the information form or
unable to comply with regular follow-up consultation.