A Prospective Cohort Study of IgG4RD in China

Chinese Academy of Medical Sciences & Peking Union Medical College

Status: Enrolling

Conditions: IgG4-related Disease

Study type: Observational

Funder types: Other

Identifiers

NCT01670695

IgG4RD-Cohort

Details and patient eligibility

About

This is an cohort study to investigate the disease course and treatment response of patients with IgG4-related disease.

Full description

Immunoglobulin G4-related disease (IgG4-RD) is a recently defined emerging clinical entity characterized by tissue infiltration by IgG4-positive plasma cells, tissue fibrosclerosis and elevated serum IgG4 concentration.

In this study, patients with IgG4-RD will be investigated, including clinical presentation, laboratory findings and imaging study. All patients will be treated by standard care and follow-uped for at least 6 months.

Enrollment: 1,000 estimated patients

Sex: All

Ages: 18 to 75 years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• Males and females

• Age 18-75 years old with informed consent

• Patients with IgG4-RD:

  1. swelling, sclerosing and inflammatory involvement of one or more organ, including sclerosing pancreatitis, sclerosing cholangitis, inflammatory pseudotumors, retroperitoneal or mediastinal fibrosis, interstitial nephritis, hypophysitis, sclerosing dacryoadenitis, sialadenitis, inflammatory aortic aneurysm, lymphadenopathy, or other inflammatory conditions;
  2. elevated serum IgG4 (>1.35 g/L)
  3. histopathologic features of fibrosis and/or lymphocytic and polyclonal plasma cell infiltration (and IgG4+ plasma cells on immunohistology when performed);
  4. exclusion of other diseases.

Exclusion criteria

• Females planning to bear a child recently or with childbearing potential
• Concurrent severe and/or uncontrolled and/or unstable diseases
• Patient with malignancy

Trial design

1,000 participants in 1 patient group

IgG4-RD

Description:

Patients with IgG4-RD, including sclerosing pancreatitis, sclerosing cholangitis, inflammatory pseudotumors, retroperitoneal or mediastinal fibrosis, interstitial nephritis, hypophysitis, sclerosing dacryoadenitis, sialadenitis (Mikulicz disease and Küttner's tumor), inflammatory aortic aneurysm, lymphadenopathy, or other inflammatory conditions.