Immunoglobulin G4-Related DiseaseNCT05901207
Diagnostic and Prognostic Biomarkers for IgG4-related Disease.
An observational study of Immunoglobulin G4-Related Disease, run by Fundació Institut de Recerca de l'Hospital de la Santa Creu i Sant Pau at 1 site in Spain.
Trial summary
IgG4-immunoglobulin-related disease (IgG4-IRD) is a relatively new pathology, characterized by intense inflammation, fibrosis, infiltration and elevated IgG4 levels in peripheral blood. Despite the interest in the disease, these diagnostic criteria are not without discrepancies and false negatives. Read more about this study
- Who is it for?
- Ages 18 years and older
- Conditions the study is for
- Immunoglobulin G4-Related Disease
- What is being tested?
- blood test
- How many people
- 50 participants
- Where
- 1 siteSpain
- Study dates
- 2 May 2024to 1 Dec 2024
- Phase
- Observational
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 years and older
- All sexes
- No healthy volunteers
- Adult, Older adult
Study population: In the monographic consultations of systemic autoimmune disease of a tertiary university, Hospital Universitari Sant Pau
Who may be able to join
- Patients over 18 years of age
- Diagnosed of IgG4-related disease (possible, probable or definite) according to the revised Umehara diagnostic criteria
Who cannot join
- Patients with cancer
- secondary causes of fibrosis
- Granulomatosis
- Castleman's disease
- seropositive Sjögren's syndrome
- Primary sclerosing cholangitis.
- Patients who have received treatment with prednisone ≥ 5 mg, immunosuppressive or biologic treatment in the last 6 months.
Trial locations and contacts
1 site in 1 country.
Spain1 site, 1 recruiting
- Recruiting
Hospital de la Santa Creu i Sant Pau
Barcelona, 08025
Patricia Moya, doctor, 639196767, pmoyaa@santpau.cat
About this study
IgG4-immunoglobulin-related disease (IgG4-IRD) is a relatively new pathology, characterized by intense inflammation, fibrosis, infiltration and elevated IgG4 levels in peripheral blood.
Despite the interest in the disease, these diagnostic criteria are not without discrepancies and false negatives. In fact, despite the fact that elevated serum IgG4 concentrations can provide an important clue for the diagnosis of the disease,described that the specificity and positive predictive value of elevated serum IgG4 concentrations are 60 % and 34 % respectively. And, when they increased the cut-off values to double to improve specificity, the sensitivity of IgG4 levels drops to 35 %.
Read more
In 2014, was described the presence of elevated concentrations of plasmablasts (CD19 low, CD20 -, CD38+ and CD27+) in the serum of patients with active ER-IgG4, even in patients with normal IgG4 levels, compared with healthy patients and with other autoimmune pathologies.
Furthermore, several studies have shown that follicular T helper (Thf) cells are increased in both peripheral blood and affected tissue of patients with ER-IgG4. These cells appear responsible for the development of germinal centers in lymph nodes and for the production of interleukins that drive IgG4 class switching, and creation of IgG4-secreting plasmablasts and plasma cells. This suggests that interleukins IL4, IL5, IL 10, IL13 might also be relevant in discerning ER-IgG4 from other immune-mediated processes with similar symptoms.
ACTION GOALS:
- To evaluate the diagnostic validity of plasmablast count and other immunological markers (B lymphocyte differentiation stage, follicular T helper lymphocytes (Thf) and IL-4, IL5, IL-10 and IL-13) in peripheral blood for IgG4-Related Disease.
- To evaluate the correlation of these biomarkers with inflammatory activity, clinical manifestation and diagnostic certainty (possible, probable or definite) of IgG4-Related Disease.
- To evaluate whether high counts or concentrations of these biomarkers at diagnosis are prognostic factors for relapse during the first 12 months of follow-up in patients with IgG4-Related Disease.
Read the detailed description
- Project design The project will consist of a first diagnostic study (objectives 1-2) of cross-sectional analytical design to assess the validity of the biomarkers. Subsequently, a second prospective cohort study (objective 3) will be carried out to evaluate whether these biomarkers are prognostic factors for relapse in patients with IgG4-related disease after treatment.
- Scope of the project The project will be carried out at the Hospital Sant Pau which is a tertiary referral center of the AIS Dreta (Área Integral de Salud) of Barcelona. The hospital covers a population of 407,550 inhabitants. IgG4-Related Disease is a rare disease that requires diagnostic tests and treatments for hospital use, so almost all cases are referred and followed up at the hospital. At the Functional Autoimmune Unit of the Hospital de Sant Pau the investigators perform monographic consultations on systemic vasculitis, the investigators are an active part of the Aortic Pathology Unit (source of patients with aortitis and retroperitoneal fibrosis), and the investigators maintain a close relationship with the other services involved in this pathology (otorhinolaryngology, digestive and nephrology).
- Period of performance and recruitment procedure Patients will be selected by sequential sampling between April 2023 and December 2023 in the Systemic Autoimmune Diseases monographic consultations.
- Sample size IgG4-related disease is a rare disease and has therefore been defined under a pragmatic and realistic approach. A total of 50 participants will be recruited at Hospital Sant Pau in Barcelona. It is planned to recruit 50 patients with IgG4 disease which would be the totality of newly diagnosed patients during 12 months in our center and 50 patients with osteoarthritis or soft tissue pathology. In a previous study (doi: 10.1097/MD.00000000000000003785) the correlation of serum IgG4 concentration with active disease was r=0.17 while the correlation of IgG4+ plasmablasts (10.1136/annrheumdis-2014-205233) with active disease was r=0.47. The expected size to detect a difference (r1-r) of 0.28 is 84 patients with a beta of 20% and alpha of 5%.
- Data collection Data will be collected from the electronic medical record of the hospital database.
- Demographic and disease assessment: Collection of demographic (age, sex) and disease information (onset, duration of symptoms, treatments). Disease onset will be defined as the time at which ER- IGg4 diagnostic counseling is performed according to the Umehara criteria. The status of the outcome event (disease relapse) and the date on which it occurs will be collected. The start date of follow-up, date of outcome event, last follow-up visit or last contact with the patient in the case of lost subjects will be collected.
- Clinical evaluation: The clinical evaluation will be carried out according to medical criteria, taking into account the combination of the patient's clinical status and the complementary radiological and serological examinations, classifying the patient as active or inactive.
- Radiological studies: Data from radiological examinations in the form of CT and/or FDG18F-PET/CT performed in the diagnosis and/or evolutionary control of the patients will be collected. The organs affected and the increase of 18F FDG in case of PET-CT will be taken into account. In those patients who have scans at diagnosis and control visits, a qualitative comparative study of the images will be performed (no change, improvement, worsening).
- Laboratory studies: Plasmablasts are one of the differentiation stages of B lymphocytes. At the peripheral blood level, the investigators can identify them by labeling against molecules on their surface. Plasmablasts are defined as CD45+ CD19+ IgM- IgD- CD27+ CD38high cells. The use of these markers also allows us to identify other differentiation stages such as naïve B lymphocytes (CD45+ CD19+ IgD+ CD27-), marginal zone (CD45+ CD19+ IgD+ CD27+), class non-switched (CD45+ CD19+ IgM+ IgD+ IgD+ CD27+ CD38-), class switched memory (CD45+ CD19+ IgM- IgD- CD27+ CD38-), transitional (CD45+ CD19+ IgM+ IgD+ CD27- CD38high CD24high), CD19low (CD45+ CD19low CD27+) or CD21low (CD45+ CD19+ CD21low CD38-). To analyze the differentiation stages of B lymphocytes, the investigators will perform a flow cytometry analysis. Briefly, from peripheral blood in EDTA, the investigators will perform a wash and subsequent staining with the different monoclonal antibodies directed against the surface molecules of interest labeled with fluorochromes. After a 15-minute incubation, the red blood cells are lysed, washed again and analyzed in the BD FACSLyric cytometer. For the determination of follicular T helper (Thf) lymphocytes and Thf1 and Thf2 subpopulations the investigators will also perform cytometry analysis with monoclonal antibody staining, defining Tfh1 as CD4+ CD45RA- CXCR5+ CCR6- CXCR3+ T lymphocytes and Tfh2 as CD4+ CD45RA- CXCR5+ CCR6- CXCR3- T lymphocytes. The analysis of interleukins IL-4, IL-5, IL-10 and IL-13 will be performed, from our serum, by individualized ELISAs for each of them. The analysis of the biosamples and images will be performed at the research center (Hospital Sant Pau de Barcelona).
- Description of variables 6.1. Baseline study
- Dependent or response variables: Disease activity, prevalence of IgG4-related disease according to different degrees of diagnostic certainty, type of clinical manifestation, number of organs affected, IGg4 concentration and acute phase reactants.
- Independent variables: plasmablast count, differentiation stages of B lymphocytes and Thf lymphocytes in peripheral blood and serum concentration of interleukins (IL-4, IL-5, IL-10 and IL-13). 6.2. Longitudinal study
- Response-dependent variables: Disease relapse and persistence of disease activity will be studied according to medical criteria.
- Independent variables: plasmablast count, differentiation stages of B lymphocytes and Thf lymphocytes in peripheral blood and serum concentration of interleukins (IL-4, IL-5, IL-10 and IL-13).
- Potentially confounding variables: Demographics (age, sex, race), IgG4-related disease subtype according to organ specificity, number of organs affected, time from onset of symptoms and time to initiation of treatment, accumulated corticosteroid dose, immunomodulatory or biological treatment.
- Follow-up At the baseline visit, demographic and disease information will be collected in a standardized manner. Blood sampling is performed within 30 days as per standard clinical practice. Imaging tests such as CT or PET-CT are usually performed 1 month before or after the baseline visit. Follow-up visits will be performed every 4 months per usual clinical practice without requiring additional visits except for those presenting complications or adverse effects to the treatments.
- Statistical analysis Logarithmic transformation of biomarker levels will be performed if they do not follow a normal distribution.
- Objectives 1 and 2: Sensitivity, specificity and area under ROC curve will be calculated. Pearson's or Spearman's correlation coefficient will be used according to applicability conditions.
- Objective 3: Univariate Cox regression will be performed to evaluate the prognostic value of biomarkers for predicting relapse. Survival will be defined as the time between the baseline visit at diagnosis to the date of the outcome event (relapse or persistence of inflammatory activity). Median follow-up will be calculated according to the inverse Kaplan Meier method. Other factors that may be related to survival (biological treatment, cumulative corticosteroid dose, age, acute phase reactants) will be added. Binary logistic analysis will also be performed to assess the association between biomarkers and dependent variables. The significance level will be set at 5% bilaterally. The statistical program Stata 14.0 for Windows will be used.
- Ethics The study will be conducted in strict accordance with international ethical recommendations for medical research in humans. The investigator will be responsible for ensuring that the study is conducted in accordance with the Bioethical Research Law 14/2007. Before starting the study, the Ethics Committee of the investigating Hospital must approve the study protocol, the information to be given to the subject and the informed consent form to be used. The CEIC will be informed of any subsequent amendments to the protocol and must be asked for its opinion in the event that a new evaluation of the ethical aspects of the study is necessary. Regarding the confidentiality of the study data, the provisions of the Organic Law 3/2018 of December 5, "Protection of Personal Data" will be followed. Your name will not appear in any of the study reports and your identity will not be disclosed to any person except to fulfill the purposes of the study, and in the case of medical emergency or legal requirement.
- Limitations Because it is a rare disease, the sample size is the main limitation of the study and will limit the number of variables that can be included in the analysis. In order to address this limitation, the investigators will disseminate the present study in the rest of the services and multidisciplinary meetings of our center, making an active search for possible candidates to be included in the study. Another limitation is that the diagnosis of the cases is defined by clinical criteria, not always confirmed histopathologically. This is an unavoidable limitation due to the clinical nature of the study. For this reason it was decided to opt for a prospective design compared to many studies of histopathologically confirmed cases that are usually retrospective. Finally, the results will not be validated in an independent sample so the results must be interpreted in context.
- CHRONOGRAM The total duration of the project is 2 years; 18 months for field work with recruitment and inclusion of patients and processing of biological samples. The last 6 months are reserved for the analysis, interpretation and writing up of results.
- Conditions
- Immunoglobulin G4-Related Disease
- Being tested
- blood test
- Medical subject headings
- Immunoglobulin G4-Related Disease
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
blood testDiagnostic test
blood test
| Study group | Role | What they receive |
|---|---|---|
| Case | Not described Receives: Diagnostic Test: blood test |
What is being measured
The primary outcome is the main question the study is designed to answer.
Change in IgG4 in peripheral blood
g/L Independent variables: plasmablast count, differentiation stages of B lymphocytes and Thf lymphocytes in peripheral blood and serum concentration of interleukins (IL-4, IL-5, IL-10 and IL-13).
and serum concentration of interleukins (IL-4, IL-5, IL-10 and IL-13).
Measured: Change from Baseline at one year after the study began
Change in IL-4
pg/mL
Measured: Change from Baseline at one year after the study began
Change in IL-5
pg/mL
Measured: Change from Baseline at one year after the study began
Change in IL-10
pg/mL
Measured: Change from Baseline at one year after the study began
Change in IL-13
pg/mL
Measured: Change from Baseline at one year after the study began
Change in plasmablast
SFU/million
Measured: Change from Baseline at one year after the study began
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted13 Jun 2023
- Study start2 May 2024 (estimated)
- Primary completion1 Dec 2024 (estimated)
- Study completion1 Dec 2024 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Immunoglobulin G4-Related DiseaseStudies of the same condition, open and under way.
- More trials run by Fundació Institut de Recerca de l'Hospital de la Santa Creu i Sant PauThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Case-control
- Time perspective
- Cross-sectional
- Enrolment
- 50 participants (estimated)
Sponsor and investigators
- Sponsor type
- University, hospital or other
- Responsible party
- Sponsor
- Investigators
- Patricia Moya Alvarado, Principal investigator, Sant Pau
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT05901207
- Sponsor's protocol ID
- IIBSP-EIG-2022-92
- Status last verified
- Jun 2023
- First submitted
- 11 Jan 2023
- Last updated
- 6 May 2024
- Results posted
- No
- Data monitoring committee
- Yes
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No
Study at a glance
From the registry record, last updated by the sponsor on 6 May 2024. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Diagnostic and Prognostic Biomarkers for IgG4-related Disease.
- ClinicalTrials.gov ID
- NCT05901207
- Acronym / sponsor ID
- IIBSP-EIG-2022-92
- Conditions
- Immunoglobulin G4-Related Disease
- Study type
- Observational
- Recruitment status
- Unknown status
- Enrolment
- 50 participants (estimated)
- Lead sponsor
- Fundació Institut de Recerca de l'Hospital de la Santa Creu i Sant PauUniversity, hospital or other
- Study start
- 2 May 2024 (estimated)
- Primary completion
- 1 Dec 2024 (estimated)
- Study completion
- 1 Dec 2024 (estimated)
- First posted
- 13 Jun 2023 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
IgG4-immunoglobulin-related disease (IgG4-IRD) is a relatively new pathology, characterized by intense inflammation, fibrosis, infiltration and elevated IgG4 levels in peripheral blood.
Despite the interest in the disease, these diagnostic criteria are not without discrepancies and false negatives. In fact, despite the fact that elevated serum IgG4 concentrations can provide an important clue for the diagnosis of the disease,described that the specificity and positive predictive value of elevated serum IgG4 concentrations are 60 % and 34 % respectively. And, when they increased the cut-off values to double to improve specificity, the sensitivity of IgG4 levels drops to 35 %.
In 2014, was described the presence of elevated concentrations of plasmablasts (CD19 low, CD20 -, CD38+ and CD27+) in the serum of patients with active ER-IgG4, even in patients with normal IgG4 levels, compared with healthy patients and with other autoimmune pathologies.
Furthermore, several studies have shown that follicular T helper (Thf) cells are increased in both peripheral blood and affected tissue of patients with ER-IgG4. These cells appear responsible for the development of germinal centers in lymph nodes and for the production of interleukins that drive IgG4 class switching, and creation of IgG4-secreting plasmablasts and plasma cells. This suggests that interleukins IL4, IL5, IL 10, IL13 might also be relevant in discerning ER-IgG4 from other immune-mediated processes with similar symptoms.
ACTION GOALS:
- To evaluate the diagnostic validity of plasmablast count and other immunological markers (B lymphocyte differentiation stage, follicular T helper lymphocytes (Thf) and IL-4, IL5, IL-10 and IL-13) in peripheral blood for IgG4-Related Disease.
- To evaluate the correlation of these biomarkers with inflammatory activity, clinical manifestation and diagnostic certainty (possible, probable or definite) of IgG4-Related Disease.
- To evaluate whether high counts or concentrations of these biomarkers at diagnosis are prognostic factors for relapse during the first 12 months of follow-up in patients with IgG4-Related Disease.
Detailed description
- Project design The project will consist of a first diagnostic study (objectives 1-2) of cross-sectional analytical design to assess the validity of the biomarkers. Subsequently, a second prospective cohort study (objective 3) will be carried out to evaluate whether these biomarkers are prognostic factors for relapse in patients with IgG4-related disease after treatment.
- Scope of the project The project will be carried out at the Hospital Sant Pau which is a tertiary referral center of the AIS Dreta (Área Integral de Salud) of Barcelona. The hospital covers a population of 407,550 inhabitants. IgG4-Related Disease is a rare disease that requires diagnostic tests and treatments for hospital use, so almost all cases are referred and followed up at the hospital. At the Functional Autoimmune Unit of the Hospital de Sant Pau the investigators perform monographic consultations on systemic vasculitis, the investigators are an active part of the Aortic Pathology Unit (source of patients with aortitis and retroperitoneal fibrosis), and the investigators maintain a close relationship with the other services involved in this pathology (otorhinolaryngology, digestive and nephrology).
- Period of performance and recruitment procedure Patients will be selected by sequential sampling between April 2023 and December 2023 in the Systemic Autoimmune Diseases monographic consultations.
- Sample size IgG4-related disease is a rare disease and has therefore been defined under a pragmatic and realistic approach. A total of 50 participants will be recruited at Hospital Sant Pau in Barcelona. It is planned to recruit 50 patients with IgG4 disease which would be the totality of newly diagnosed patients during 12 months in our center and 50 patients with osteoarthritis or soft tissue pathology. In a previous study (doi: 10.1097/MD.00000000000000003785) the correlation of serum IgG4 concentration with active disease was r=0.17 while the correlation of IgG4+ plasmablasts (10.1136/annrheumdis-2014-205233) with active disease was r=0.47. The expected size to detect a difference (r1-r) of 0.28 is 84 patients with a beta of 20% and alpha of 5%.
- Data collection Data will be collected from the electronic medical record of the hospital database.
- Demographic and disease assessment: Collection of demographic (age, sex) and disease information (onset, duration of symptoms, treatments). Disease onset will be defined as the time at which ER- IGg4 diagnostic counseling is performed according to the Umehara criteria. The status of the outcome event (disease relapse) and the date on which it occurs will be collected. The start date of follow-up, date of outcome event, last follow-up visit or last contact with the patient in the case of lost subjects will be collected.
- Clinical evaluation: The clinical evaluation will be carried out according to medical criteria, taking into account the combination of the patient's clinical status and the complementary radiological and serological examinations, classifying the patient as active or inactive.
- Radiological studies: Data from radiological examinations in the form of CT and/or FDG18F-PET/CT performed in the diagnosis and/or evolutionary control of the patients will be collected. The organs affected and the increase of 18F FDG in case of PET-CT will be taken into account. In those patients who have scans at diagnosis and control visits, a qualitative comparative study of the images will be performed (no change, improvement, worsening).
- Laboratory studies: Plasmablasts are one of the differentiation stages of B lymphocytes. At the peripheral blood level, the investigators can identify them by labeling against molecules on their surface. Plasmablasts are defined as CD45+ CD19+ IgM- IgD- CD27+ CD38high cells. The use of these markers also allows us to identify other differentiation stages such as naïve B lymphocytes (CD45+ CD19+ IgD+ CD27-), marginal zone (CD45+ CD19+ IgD+ CD27+), class non-switched (CD45+ CD19+ IgM+ IgD+ IgD+ CD27+ CD38-), class switched memory (CD45+ CD19+ IgM- IgD- CD27+ CD38-), transitional (CD45+ CD19+ IgM+ IgD+ CD27- CD38high CD24high), CD19low (CD45+ CD19low CD27+) or CD21low (CD45+ CD19+ CD21low CD38-). To analyze the differentiation stages of B lymphocytes, the investigators will perform a flow cytometry analysis. Briefly, from peripheral blood in EDTA, the investigators will perform a wash and subsequent staining with the different monoclonal antibodies directed against the surface molecules of interest labeled with fluorochromes. After a 15-minute incubation, the red blood cells are lysed, washed again and analyzed in the BD FACSLyric cytometer. For the determination of follicular T helper (Thf) lymphocytes and Thf1 and Thf2 subpopulations the investigators will also perform cytometry analysis with monoclonal antibody staining, defining Tfh1 as CD4+ CD45RA- CXCR5+ CCR6- CXCR3+ T lymphocytes and Tfh2 as CD4+ CD45RA- CXCR5+ CCR6- CXCR3- T lymphocytes. The analysis of interleukins IL-4, IL-5, IL-10 and IL-13 will be performed, from our serum, by individualized ELISAs for each of them. The analysis of the biosamples and images will be performed at the research center (Hospital Sant Pau de Barcelona).
- Description of variables 6.1. Baseline study
- Dependent or response variables: Disease activity, prevalence of IgG4-related disease according to different degrees of diagnostic certainty, type of clinical manifestation, number of organs affected, IGg4 concentration and acute phase reactants.
- Independent variables: plasmablast count, differentiation stages of B lymphocytes and Thf lymphocytes in peripheral blood and serum concentration of interleukins (IL-4, IL-5, IL-10 and IL-13). 6.2. Longitudinal study
- Response-dependent variables: Disease relapse and persistence of disease activity will be studied according to medical criteria.
- Independent variables: plasmablast count, differentiation stages of B lymphocytes and Thf lymphocytes in peripheral blood and serum concentration of interleukins (IL-4, IL-5, IL-10 and IL-13).
- Potentially confounding variables: Demographics (age, sex, race), IgG4-related disease subtype according to organ specificity, number of organs affected, time from onset of symptoms and time to initiation of treatment, accumulated corticosteroid dose, immunomodulatory or biological treatment.
- Follow-up At the baseline visit, demographic and disease information will be collected in a standardized manner. Blood sampling is performed within 30 days as per standard clinical practice. Imaging tests such as CT or PET-CT are usually performed 1 month before or after the baseline visit. Follow-up visits will be performed every 4 months per usual clinical practice without requiring additional visits except for those presenting complications or adverse effects to the treatments.
- Statistical analysis Logarithmic transformation of biomarker levels will be performed if they do not follow a normal distribution.
- Objectives 1 and 2: Sensitivity, specificity and area under ROC curve will be calculated. Pearson's or Spearman's correlation coefficient will be used according to applicability conditions.
- Objective 3: Univariate Cox regression will be performed to evaluate the prognostic value of biomarkers for predicting relapse. Survival will be defined as the time between the baseline visit at diagnosis to the date of the outcome event (relapse or persistence of inflammatory activity). Median follow-up will be calculated according to the inverse Kaplan Meier method. Other factors that may be related to survival (biological treatment, cumulative corticosteroid dose, age, acute phase reactants) will be added. Binary logistic analysis will also be performed to assess the association between biomarkers and dependent variables. The significance level will be set at 5% bilaterally. The statistical program Stata 14.0 for Windows will be used.
- Ethics The study will be conducted in strict accordance with international ethical recommendations for medical research in humans. The investigator will be responsible for ensuring that the study is conducted in accordance with the Bioethical Research Law 14/2007. Before starting the study, the Ethics Committee of the investigating Hospital must approve the study protocol, the information to be given to the subject and the informed consent form to be used. The CEIC will be informed of any subsequent amendments to the protocol and must be asked for its opinion in the event that a new evaluation of the ethical aspects of the study is necessary. Regarding the confidentiality of the study data, the provisions of the Organic Law 3/2018 of December 5, "Protection of Personal Data" will be followed. Your name will not appear in any of the study reports and your identity will not be disclosed to any person except to fulfill the purposes of the study, and in the case of medical emergency or legal requirement.
- Limitations Because it is a rare disease, the sample size is the main limitation of the study and will limit the number of variables that can be included in the analysis. In order to address this limitation, the investigators will disseminate the present study in the rest of the services and multidisciplinary meetings of our center, making an active search for possible candidates to be included in the study. Another limitation is that the diagnosis of the cases is defined by clinical criteria, not always confirmed histopathologically. This is an unavoidable limitation due to the clinical nature of the study. For this reason it was decided to opt for a prospective design compared to many studies of histopathologically confirmed cases that are usually retrospective. Finally, the results will not be validated in an independent sample so the results must be interpreted in context.
- CHRONOGRAM The total duration of the project is 2 years; 18 months for field work with recruitment and inclusion of patients and processing of biological samples. The last 6 months are reserved for the analysis, interpretation and writing up of results.
Study design
- Observational model
- Case-control
- Time perspective
- Cross-sectional
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| Case | – | Diagnostic Test: blood test |
Who may qualify
- Ages
- 18 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
- Study population
- In the monographic consultations of systemic autoimmune disease of a tertiary university, Hospital Universitari Sant Pau
Key inclusion criteria (2 listed)
- Patients over 18 years of age
- Diagnosed of IgG4-related disease (possible, probable or definite) according to the revised Umehara diagnostic criteria
Key exclusion criteria (7 listed)
- Patients with cancer
- secondary causes of fibrosis
- Granulomatosis
- Castleman's disease
- seropositive Sjögren's syndrome
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| blood testblood test | Diagnostic test | Case |
Primary outcome
Change in IgG4 in peripheral blood
Time frame: Change from Baseline at one year after the study began
g/L Independent variables: plasmablast count, differentiation stages of B lymphocytes and Thf lymphocytes in peripheral blood and serum concentration of interleukins (IL-4, IL-5, IL-10 and IL-13). and serum concentration of interleukins (IL-4, IL-5, IL-10 and IL-13).
Change in IL-4
Time frame: Change from Baseline at one year after the study began
pg/mL
Change in IL-5
Time frame: Change from Baseline at one year after the study began
pg/mL
Change in IL-10
Time frame: Change from Baseline at one year after the study began
pg/mL
Change in IL-13
Time frame: Change from Baseline at one year after the study began
pg/mL
Change in plasmablast
Time frame: Change from Baseline at one year after the study began
SFU/million
Results and safety
Results are not available in the source record.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 6 May 2024.
- Carruthers, M. N., Khosroshahi, A., Augustin, T., Deshpande, V., \& Stone, J. H. (2015). The diagnostic utility of serum IgG4 concentrations in IgG4-related disease. Annals of the Rheumatic Diseases, 74(1), 14-18.Link provided by the sponsor.
- Hong, X., Li, W., Xie, X.-Y., Zhang, Z.-Y., Chen, Y., Gao, Y., Peng, X., Su, J.-Z., Zhang, Y.-Y., Wang, Z., Cai, Z.-G., Zhang, L., Liu, Y.-Y., He, J., Ren, L.-M., Li, Z.-G., \& Yu, G.-Y. (2017). Differential diagnosis of IgG4-related sialadenitis, primaryLink provided by the sponsor.
- Maehara, T., Pillai, S., Stone, J. H., \& Nakamura, S. (2019). Clinical features and mechanistic insights regarding IgG4-related dacryoadenitis and sialoadenitis: a review. International Journal of Oral and Maxillofacial Surgery, 48(7), 908-916.Link provided by the sponsor.
- Okazaki, K., Uchida, K., Koyabu, M., Miyoshi, H., \& Takaoka, M. (2011). Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related disease. Journal of Gastroenterology, 46(3), 277-288.Link provided by the sponsor.
- Individual participant dataSharing planned: No
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Patricia Moya Alvarado principal investigator
Sant Pau
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Immunoglobulin G4-Related Disease, run by Fundació Institut de Recerca de l'Hospital de la Santa Creu i Sant Pau at 1 site in Spain.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 2 inclusion and 7 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: status not confirmed, as of the sponsor's last update on 6 May 2024. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in Spain. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
Results are not available in the source record.
How can a doctor refer a patient or contact the research team?
Contact Patricia Moya Alvarado, Doctor (639196767, pmoyaa@santpau.cat), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Change in IgG4 in peripheral blood (time frame: Change from Baseline at one year after the study began). The study lists 6 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 6 May 2024.