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Primary Gougerot-Sjögren's syndrome is a systemic autoimmune disease belonging to the group of connectivities, whose physiopathology remains largely unknown. Quantification and characterization of epithelial and endothelial circulants in Gougerot-Sjögren's syndrome could reflect the intensity of the epithelial aggression, and thus possibly constitute a biomarker.
Primary Gougerot-Sjögren's syndrome is a systemic autoimmune disease belonging to the group of connectivities.
The criteria for classification of the disease include dry syndrome, positive salivary gland biopsy and detection of anti-Sjögren's-syndrome-related antigen A (anti-SSA) and anti-Sjögren's-syndrome-related antigen B (anti-SSB) antibodies. The presence of antibodies is thus important for the diagnosis but not essential, because in some patients the salivary gland biopsy is positive and the antibodies are absent. Therefore, the identification of new biomarkers could be very useful to confirm the diagnosis of primary Gougerot-Sjögren's syndrome and to identify subgroups of patients.
The pathophysiology of the disease remains largely unknown. Currently, primary Gougerot-Sjögren's syndrome is thought to originate from inflammation of the epithelial tissue of the salivary glands. However, it is currently unknown whether this autoimmune epithelitis is accompanied by a contingent of circulating epithelial cells, the characterization of which might be accessible by liquid biopsy.
So far, the circulating epithelial cells that have been identified have been identified in the context of cancer: in this case they are called circulating tumor cells. Their detection during primary Gougerot-Sjögren's syndrome could reflect the intensity of epithelial aggression, and thus possibly constitute a biomarker.
In other connectivities, data on circulating cells have already been published. In systemic scleroderma, another connectivitis affecting mainly middle-aged women, circulating progenitor cells have already been detected and are thought to have the capacity to differentiate into endothelial cells, thus playing a potentially important role in the pathophysiology of the disease.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Experimental group | Experimental | Patients with Gougerot-Sjögren's syndrome meeting American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) disease definitions |
|
| Positive control group | Active Comparator | Patients with diffuse systemic scleroderma meeting American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) disease definitions |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Blood sampling | Other | A blood sampling will be performed during the inclusion visit (day 0). |
|
| Measure | Description | Time Frame |
|---|---|---|
| Circulating epithelial cell detection rate | Rate of patients with at least 1 circulating epithelial cell in the peripheral blood | day 0 |
| Measure | Description | Time Frame |
|---|---|---|
| Number of circulating epithelial cells | day 0 | |
| Number of circulating epithelial cells expressing human epidermal growth factor receptor 2 (HER2) | day 0 | |
| Number of circulating epithelial and endothelial cells |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Philippe GUILPAIN, MD, PhD | University Hospital, Montpellier | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Montpellier University Hospital | Montpellier | France |
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This a single-center prospective comparative non-randomized cohort pilot study, with 2 parallel groups (experimental group : patients with Gougerot-Sjögren's syndrome and positive control group : patients with diffuse systemic scleroderma).
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| day 0 |
| ID | Term |
|---|---|
| D045743 | Scleroderma, Diffuse |
| D012859 | Sjogren's Syndrome |
| D012595 | Scleroderma, Systemic |
| D001327 | Autoimmune Diseases |
| D003240 | Connective Tissue Diseases |
| ID | Term |
|---|---|
| D017437 | Skin and Connective Tissue Diseases |
| D012871 | Skin Diseases |
| D001172 | Arthritis, Rheumatoid |
| D001168 | Arthritis |
| D007592 | Joint Diseases |
| D009140 | Musculoskeletal Diseases |
| D012216 | Rheumatic Diseases |
| D014987 | Xerostomia |
| D012466 | Salivary Gland Diseases |
| D009059 | Mouth Diseases |
| D009057 | Stomatognathic Diseases |
| D015352 | Dry Eye Syndromes |
| D007766 | Lacrimal Apparatus Diseases |
| D005128 | Eye Diseases |
| D007154 | Immune System Diseases |
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| ID | Term |
|---|---|
| D001800 | Blood Specimen Collection |
| ID | Term |
|---|---|
| D013048 | Specimen Handling |
| D019411 | Clinical Laboratory Techniques |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
| D011677 | Punctures |
| D013514 | Surgical Procedures, Operative |
| D008919 | Investigative Techniques |
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