Diagnostic and Prognostic Biomarkers for IgG4-related Disease.

NCT05901207 | Unknown DMC

• 1 other identifier: IIBSP-EIG-2022-92
• Study Type: observational
• Target: 50
• Locations: 1 country
• Sites: 1

Brief 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. 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:

1. 1.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.
2. 2.To evaluate the correlation of these biomarkers with inflammatory activity, clinical manifestation and diagnostic certainty (possible, probable or definite) of IgG4-Related Disease.
3. 3.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.

Conditions

Immunoglobulin G4-Related Disease

Outcome Measures

Primary Outcomes (6)

• 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).

  Change from Baseline at one year after the study began

• Change in IL-4

  pg/mL

  Change from Baseline at one year after the study began

• Change in IL-5

  pg/mL

  Change from Baseline at one year after the study began

• Change in IL-10

  pg/mL

  Change from Baseline at one year after the study began

• Change in IL-13

  pg/mL

  Change from Baseline at one year after the study began

• Change in plasmablast

  SFU/million

  Change from Baseline at one year after the study began

Study Arms (1)

Case

Diagnostic Test: blood test

Interventions

blood test DIAGNOSTIC_TEST

blood test

Case

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

In the monographic consultations of systemic autoimmune disease of a tertiary university, Hospital Universitari Sant Pau

You may qualify if:

• Patients over 18 years of age
• Diagnosed of IgG4-related disease (possible, probable or definite) according to the revised Umehara diagnostic criteria

You may not qualify if:

• 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.

Sponsors & Collaborators

• Fundació Institut de Recerca de l'Hospital de la Santa Creu i Sant Pau: lead

Study Sites (1)

Hospital de la Santa Creu i Sant Pau

Barcelona, 08025, Spain

RECRUITING

Related Links

• 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.
• 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, primary
• 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.
• 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.

MeSH Terms

Conditions

Immunoglobulin G4-Related Disease

Interventions

Hematologic Tests

Condition Hierarchy (Ancestors)

Autoimmune Diseases; Immune System Diseases

Intervention Hierarchy (Ancestors)

Clinical Laboratory Techniques; Diagnostic Techniques and Procedures; Diagnosis; Investigative Techniques

Study Officials

• Patricia Moya Alvarado

  Sant Pau

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Patricia Moya Alvarado, Doctor

CONTACT

639196767; pmoyaa@santpau.cat

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: CROSS SECTIONAL
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: January 11, 2023
• First Posted: June 13, 2023
• Study Start: May 2, 2024
• Primary Completion: December 1, 2024
• Study Completion: December 1, 2024
• Last Updated: May 6, 2024

Record last verified: 2023-06

Data Sharing

• IPD Sharing: Will not share

Locations

ES (1)