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Cohort Study of Patients With IgG4-Related Disease

Phase 0
18 Years
75 Years
Open (Enrolling)
IgG4-related Disease

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Trial Information

Cohort Study of Patients With IgG4-Related Disease

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.

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

Type of Study:


Study Design:

Observational Model: Cohort, Time Perspective: Prospective

Outcome Measure:

Clinical Response

Outcome Description:

Clinical Response is measured by IgG4-RD Responder Index, including organ/site score and serum IgG4 level.

Outcome Time Frame:

6 months

Safety Issue:


Principal Investigator

Wen Zhang, MD

Investigator Role:

Principal Investigator

Investigator Affiliation:

Deptment of Rheumatology, Peking Union Medical College Hospital


China: National Natural Science Foundation

Study ID:




Start Date:

August 2012

Completion Date:

March 2014

Related Keywords:

  • IgG4-related Disease
  • IgG4-related disease
  • IgG4-RD