Withdraw Drug in Stable IgG4-Related Disease

Chinese Academy of Medical Sciences & Peking Union Medical College

Status: Unknown

Conditions: Autoimmune Diseases

Treatments: Drug: Drug free, IS monotherapy and GC combined with IS

Study type: Interventional

Funder types: Other

Identifiers

NCT04124861

Withdraw drug in IgG4-RD

Details and patient eligibility

About

Evaluation and prediction of relapse risk after glucocorticoid or immunosuppressant withdrawal in patients with stable IgG4 related disease: a prospective cohort study from china.

Full description

IgG4-related disease (IgG4-RD) is a chronic autoimmune disease with a relapsing-remitting course. For patients in remission, glucocorticoid (GC) and immunosuppressant are used to be maintained for a long time in fear of disease flare. Long-term GC and immunosuppressant could bring a lot of side-effects and economic burden. Whether and when patients with stable disease should withdraw GC or immunosuppressant? How about the relapse risk respectively? What are the risk factors for disease flare? All the above remain unclear. The aim of this study is to explore the relapse risk after GC or immunosuppressant withdrawal in IgG4-RD patients with stable disease and to establish a predictive model for risk stratification. Meanwhile the investigators aim to testify the effects of immunosuppressant in preventing IgG4-RD relapse. This study is a prospective cohort clinical trial.

Enrollment: 138 estimated patients

Sex: All

Ages: 18 to 70 years old

Volunteers: No Healthy Volunteers

Inclusion criteria

1. All patients must meet the following diagnostic criteria of IgG4RD (2011): 1)Swelling, sclerosing and inflammatory involvement of one or more organ, including sclerosing pancreatitis, sialadenitis (Mikulicz disease), sclerosing cholangitis, inflammatory pseudotumors, retroperitoneal or mediastinal fibrosis, interstitial nephritis, hypophysitis, sclerosing dacryoadenitis, 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). Patients fulfill 1)+2)+3) are diagnosed as definite IgD4RD, 1)+2): possible IgG4RD; 1)+3): probable IgG4RD. exclusion of other diseases.
2. Disease stabilized more than one year (Responder Index < 2 points)
3. Dose of Glucocorticoid(GC): prednisone (or equivalent) ≤ 7.5mg/d for more than 6 months
4. Immunosuppressant: one of the following drugs, the same dose maintain at least 3 months (Mycophenolate mate <= 1g/d or Leflunomide <=20mg/d or Methotrexate <=12.5mg/w or Azathioprine <=100mg/d).

Exclusion criteria

1. Patient was diagnosed other connective tissue diseases
2. Patient with tumor
3. Women during pregnancy or planning pregnancy
4. Patient with active infections, including HIV, HCV, HBV, TB, etc.
5. Patient with severe irreversible organ damage
6. Active IgG4-RD, responder index >= 2 points
7. Stable condition less than one year.
8. Patient with two or more immunosuppressive agents.
9. Biological agents (such as CD20 monoclonal antibody and TNF-a inhibitor) have been used for half a year before admission.
10. Patient with IgG4-RD recurrence during hormone reduction in the past.

Trial design

Primary purpose: Treatment

Allocation: Randomized

Interventional model: Parallel Assignment

Masking: None (Open label)

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138 participants in 3 patient groups

Drug free

Experimental group

Description:

Arm A: Drug free Glucocorticoid（GC）is tapered and stopped in 8 weeks(GCs at a dose of ≤ 2.5 mg of prednisone or equivalent for treatment of adrenal insufficiency) . Immunosuppressant is also tapered and discontinues in 8 weeks.

Treatment:

Drug: Drug free, IS monotherapy and GC combined with IS

IS monotherapy

Experimental group

Description:

Arm B: Immunosuppressant only Glucocorticoid（GC）is tapered and stopped in 8 weeks. The same type and dosage of immunosuppressive agent before admission, including Mycophenolate mate(\<= 1g/d) or Leflunomide (\<=20mg/d) or Methotrexate (\<=15mg/w) or Azathioprine (\<=100mg/d)

Treatment:

Drug: Drug free, IS monotherapy and GC combined with IS

GC combined with IS

Experimental group

Description:

Arm C: GC+Immunosuppressant Both Glucocorticoid(GC) (no more than 7.5mg/d) and immunosuppressant are kept as maintaining dose.

Treatment:

Drug: Drug free, IS monotherapy and GC combined with IS