Leflunomide for Maintenance of Remission in IgG4 Related Disease

• Register: ClinicalTrials.gov
• Last refreshed on: 12 December 2020
• Main ID: NCT02703194
• Date of registration: 27/02/2016
• Prospective Registration: Yes
• Primary sponsor: Chinese PLA General Hospital
• Public title: Leflunomide for Maintenance of Remission in IgG4 Related Disease
• Scientific title: A Study of Safety and Efficacy of Leflunomide for Maintenance of Remission in IgG4 Related Disease
• Date of first enrolment: March 2016
• Target sample size: 68
• Recruitment status: Completed
• URL: https://clinicaltrials.gov/show/NCT02703194
• Study type: Interventional
• Study design: Allocation: Randomized. Intervention model: Parallel Assignment. Primary purpose: Treatment. Masking: None (Open Label).
• Phase: Phase 4

Countries of recruitment

China

Contacts

• Name: Jian Zhu
• Affiliation: Chinese PLA General Hospital

Key inclusion & exclusion criteria

Inclusion Criteria:

1. Age between 18 and 80 years.

2. Diagnosis of IgG4-RD according to either Consensus statement on the pathology of
IgG4-related disease (for those who have undergone biopsies) or 2011 Comprehensive
diagnostic criteria for IgG4-related disease. Both of the two criteria for diagnosis
are specified below.

(1)Consensus statement on the pathology of IgG4-related disease

1. Histopathologic features consisting of dense lymphoplasmacytic infiltrate,
fibrosis(usually storiform in character)and/or obliterative phlebitis within involved
organs.

2. Either an elevated IgG4+/IgG+cell ratio of >40% within the affected organs or elevated
IgG4-bearing plasma cells per high-power field is necessary. The cut-off number of
IgG4-bearing plasma cells per high-power field is different depending upon the types
of affected organs and specimens(through surgery or needle puncture biopsy).

(2)2011 Comprehensive diagnostic criteria for IgG4-related disease

1. Clinical examination showing characteristic diffuse/localized swelling or masses in
single or multiple organs.

2. Hematological examination shows elevated serum IgG4 concentrations(135 mg/dl).

3. Histopathologic examination shows marked lymphocyte and plasmacyte infiltration and
fibrosis or Infiltration of IgG4+ plasma cells(ratio of IgG4+/IgG+ cells > 40% and >10
IgG4+ plasma cells/HPF).

Definite: a + b + c,Probable: a + c,Possible: a + b

4. Excluded from malignant tumors of each organ (e.g. cancer, lymphoma) and similar
diseases (e.g. Sjögren's syndrome, primary sclerosing cholangitis, Castleman's
disease, secondary retroperitoneal fibrosis, Wegener's granulomatosis, sarcoidosis,
Churg-Strauss syndrome) by additional histopathological examination.

5. Even when patients cannot be diagnosed using the Comprehensive diagnostic criteria,
they may be diagnosed using organ-specific diagnostic criteria for IgG4-RD, such as
Diagnostic criteria for IgG4-Mikulicz's disease.

Exclusion Criteria:

1. Subjects having received steroids or immunosuppressants in recent 3 months will be
excluded.

2. Subjects who were hypersensitive to leflunomide will be excluded.

3. ALT and/or AST is more than two folds of the upper limit of relevant reference value
at baseline.

4. WBC is less than 3×10*9/L at baseline.

5. Female patients who are pregnant or breastfeeding.

6. Known significant concurrent medical disease.



Age minimum: 18 Years
Age maximum: 80 Years
Gender: All

Health Condition(s) or Problem(s) studied

Immunoglobulin G4 Related Sclerosing Disease

Intervention(s)

Drug: Prednisone

Drug: Leflunomide

Primary Outcome(s)

Relapse rate at 12 months. [Time Frame: 12 months]

Secondary Outcome(s)

Relapse rate at 6 months. [Time Frame: 6 months]

Complete response assessed by IgG4-RD Responder Index (IgG4-RD RI) at 1, 3, 6 and 12 months. [Time Frame: Up to 12 months]

Partial response assessed by IgG4-RD RI at 1, 3, 6 and 12 months. [Time Frame: Up to 12 months]

Serum IgG4 concentrations (mg/dL) measured by immunonephelometry at 1, 3, 6 and 12 months. [Time Frame: Up to 12 months]

Adverse effect events [Time Frame: Up to 12 months]

Number of circulating plasmablasts (cell number/mL) assessed by flow cytometry by gating peripheral blood at 1, 3, 6 and 12 months. [Time Frame: Up to 12 months]

Secondary ID(s)

ChinaPLAGH-IgG4

Source(s) of Monetary Support

Please refer to primary and secondary sponsors