Study of One Protein Implicated in Wegener Disease

NCT01167491 | Completed

• 1 other identifier: P081238
• Study Type: interventional
• Target: 138
• Locations: 1 country
• Sites: 1

Brief Summary

The investigators recently showed an abnormal expansion of NK-like CD4+ T cells in Wegener's granulomatosis (WG), mainly in the diffuse vasculitis presentation. These cells expressed an assortment of activating NK cell receptors and their signaling partners, in particular DAP12. The investigators hypothesize that DAP12, or a downstream signaling target of DAP12, is the missing link between the different cell components involved in WG (neutrophils, macrophages, CD4 T cells).

Conditions

Wegener's Granulomatosis

Keywords

Wegener; CD4+ T cells; DAP12; NKG2D

Outcome Measures

Primary Outcomes (1)

• level of mRNA expression of DAP12

  Measure:level of mRNA expression of DAP12 by RT-PCR in CD4+T cells, macrophages and neutrophils

  less than 24 hours

Secondary Outcomes (1)

• level of expression of DAP 12 downstream signalling proteins

  Less than 24 hours

Study Arms (1)

Disease group

OTHER

Physiopathology

Biological: Physiopathology Endpoint Classification

Interventions

Physiopathology Endpoint Classification BIOLOGICAL

Blood samples will be collected during a routine medical visit.

Disease group

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• Group 1: WG, with granulomatous lesions limited to upper airway or lungs and no evidence of generalized vasculitis ,± biopsy, ± anti-PR3 ANCA
• Group 2: WG with granulomatous lesions plus vasculitis expression (renal, neurological, skin, gut or heart involvement), ± biopsy, ± anti-PR3 ANCA
• Group 3: Necrotizing vasculitis with no granulomatous lesions, ± PAUCI immune glomerulonephritis, ± anti-MPO ANCA
• Group 4: clinical presentation compatible with sarcoidosis, ± biopsy, ± ECA elevated ± tuberculin anergy

You may not qualify if:

• \<18 years
• Pregnancy or breastfeeding
• Absence of signed informed consent
• No affiliation to insurance

Sponsors & Collaborators

• Assistance Publique - Hôpitaux de Paris: lead
• URC-CIC Paris Descartes Necker Cochin: collaborator

Study Sites (1)

Medecine Interne Hôpital Saint Louis

Paris, 75010, France

Location

MeSH Terms

Conditions

Granulomatosis with Polyangiitis

Condition Hierarchy (Ancestors)

Lung Diseases, Interstitial; Lung Diseases; Respiratory Tract Diseases; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Systemic Vasculitis; Vasculitis; Vascular Diseases; Cardiovascular Diseases; Skin Diseases, Vascular; Skin Diseases; Skin and Connective Tissue Diseases; Autoimmune Diseases; Immune System Diseases

Study Officials

• Mathilde De Menthon,, MD, PhD

  Assistance Publique Hopitaux de Paris

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NON RANDOMIZED
• Masking: NONE
• Purpose: OTHER
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: July 12, 2010
• First Posted: July 22, 2010
• Study Start: May 1, 2010
• Primary Completion: November 1, 2013
• Study Completion: September 1, 2014
• Last Updated: March 31, 2026

Record last verified: 2026-03

Locations

FR (1)