Efficacy, Safety and Immunological Evaluation of Upadacitinib for Relapsing Polychondritis

Inclusion Criteria:

• 1.Male or female ≥18 and ≤70 years

• 2.Meet the revised Michet criteria

• 3.Patients had an inadequate response to standard treatment for ≥ 4 weeks. Thebackground treatment included corticosteroids (≤0.5 mg/ kg), immunosuppressants (methotrexate, hydroxychloroquine, azathioprine, mycophenolate mofetil leflunomide,or cyclophosphamide)

4. Negative urine pregnancy test

5. Written informed consent form

Exclusion Criteria:

Any subject who meets any of the following criteria shall be excluded:

• 1.Use rituximab or other monoclonal antibodies within 2 months.

• 2.1 months after treatment with high dose glucocorticoid (> 1 mg/kg/d).

• 3.Serious complications: heart failure (≥ New York Heart Association（NYHA） IIIgrade), renal insufficiency (creatinine clearance rate ≤ 30 ml/min), liver functioninsufficiency (serum alanine transaminase or glutamic-pyruvic transaminaseT > 3times normal upper limit, or total bilirubin > normal upper limit)

• 4.Other serious, progressive or uncontrollable hematological, gastrointestinal,endocrine, lung, heart, nerve, or brain diseases (including demyelination diseases,such as multiple sclerosis).

• 5.Known allergies, hyperresponsiveness or IL-2 or its excipients are intolerant.

• 6.Severe infections (including, but not limited to, hepatitis, pneumonia,bacteremia, pyelonephritis, Epstein-Barr virus, tuberculosis infection),hospitalization for infection, or intravenous antibiotics 2 months before the firstdose of treatment.

• 7.Chest imaging showed abnormalities in malignant tumors or current activeinfections (including tuberculosis) within 3 months before the first use of thestudy.

• 8.Infected with HIV (HIV antibody positive serology) or hepatitis C (Hep C antibodypositive serology). If seropositive, consult a doctor with expertise in thetreatment of HIV or hepatitis C virus infection.

• 9.There has been any known malignant tumor or history of malignant tumor in the past 5 years (with the exception of non-melanoma skin cancer, non-melanoma skin cancerwith no sign of recurrence or surgically cured cervical tumor within 3 months of useof the first study preparation).

• 10.There are uncontrolled mental or emotional disorders, including a history of drugand alcohol abuse over the past three years, which may hinder the successfulcompletion of the study. 11.Within 3 months before the first injection of theresearch agent, during the study period or within 4 months after the last injectionof the research agent, any live virus or bacterial vaccine is received or expectedto be received. Bacillus Calmette-Guerin was vaccinated within 12 months afterscreening.

• 11.Pregnant and lactating women (WCBP) are reluctant to use medically approvedcontraceptives during and 12 months after treatment.

• 12.Men whose partners have fertility potential but do not want to use appropriatemedically approved contraceptives during and within 12 months of treatment.