Age: 18+
Healthy Volunteers: Not accepted
Inclusion Criteria:
A. ≥18 years of age
B. Must fulfill McAdam's or Damiani's or Michet's Criteria Diagnostic Criteria for Relapsing Polychondritis McAdam's Criteria (1976)
≥ 3 criteria out of 6 of the following:
1. Bilateral auricular chondritis
2. Non-erosive seronegative polyarthritis
3. Nasal chondritis
4. Ocular inflammation
5. Respiratory tract chondritis
6. Cochlear and/or vestibular dysfunction
Damiani's Criteria (1979)
1. ≥3 of McAdam's Criteria as above
2. ≥1 of McAdam's Criteria with histological confirmation of chondritis
3. ≥2 of McAdam's Criteria with positive response to glucocorticoids or dapsone
Michet's Criteria (1986)
Presence of ≥2 of the following criteria:
1. Auricular chondritis
2. Nasal chondritis
3. Laryngotracheal chondritis
Or presence of ≥1 of the above criteria and ≥2 of the following criteria
1. Seronegative inflammatory arthritis
2. Ocular inflammation
3. Hearing loss
4. Vestibular dysfunction
C. Mild to moderately active disease within 60 days prior to screening where the symptoms cannot be attributed to any cause other than RP and which, in the investigator's opinion, requires addition/ increase in prednisone dose between 15-60 mg/ day.
At the time of enrollment and during the trial, the following symptoms of active disease which will be evaluated:
1. Auricular inflammation: defined as increase/ new onset pain/ swelling/redness of external ear(s), ear canal
2. Nasal inflammation: defined as increase/ new onset pain/ swelling/redness of external nose
3. Ocular inflammation: defined as new onset/ worsening unilateral/ bilateral episcleritis/scleritis/ uveitis.
4. Inflammatory arthritis: defined as new onset/ worsening morning stiffness≥30 minutes, physician diagnosed tenderness/swelling in ≥1 joint; new onset/ worsening costochondritis.
5. Mild to moderate airway inflammation: defined as new onset/ worsening mild to moderate inflammation of upper airway diagnosed by direct laryngoscopy and attributed to RP; abnormal CT airway/ bronchoscopy showing wall thickening of airway (larynx, trachea, bronchi) and absence of severe manifestations such as new onset SGS/tracheomalacia/ bronchomalacia.
6. Sinonasal disease: defined as new onset/ increase in nasal crusting, discharge bleeding
7. Constitutional symptoms: defined as new onset/ worsening fever, unintentional weight loss of ≥ 5% of body weight, night sweats Patients must have at least 1 of the first 5 criteria within the past 60 days prior to the enrollment.
D. Willing and able to comply with treatment and follow-up procedures.
E. Both men and women of childbearing potential must be willing to use an effective means of birth control while receiving treatment throughout the study. Effective contraception methods include abstinence, oral contraceptives (birth control pills), intra-uterine-device, diaphragm, approved hormone injections, condoms, or medical sterilization.
F. Willing and able to provide written informed consent.
Exclusion Criteria:
A. Severe disease manifestations within the past 14 days requiring hospitalization, and/or intravenous pulsed dose glucocorticoid treatment for disease control.
B. Prior exposure of 4 weeks or longer to adalimumab, infliximab, and/or tocilizumab.
C. Prior exposure to >2 biologics and/or JAKi.
D. Evidence of active infection.
E. Known infection with human immunodeficiency virus (HIV), untreated hepatitis C infection, or a positive hepatitis B virus surface antigen.
F. Patients at risk for tuberculosis (TB) defined as follows:
1. Current clinical, radiographic or laboratory evidence of active TB, even if currently being treated. Chest x-rays (posterior/anterior and lateral) obtained within the 6 months prior to screening and TB testing (IFN gamma release assay or PPD) performed in the past month prior to screening will be accepted; however, a copy of the reports must be placed in the participant binder.
2. A history of active TB unless there is documentation that the patient had received prior anti-TB treatment that was appropriate in duration and type according to local health authority guidelines.
3. Patients with a positive TB screening test indicative of latent TB will not be eligible for the study unless they: i. Have no evidence of current TB based on chest x-ray performed during the screening period and by history and physical exam, and ii. They are currently being treated for latent TB or the site has documentation of successful prior treatment of latent TB. Treatment regimens should be dictated by local guidelines as long as the treatment dose and duration meet or exceed local health authority guidelines. Patients with latent TB may be eligible for the trial prior to completion of treatment as long as they have completed at least 4 weeks of treatment and they have no evidence of current TB on chest x-ray at screening.
G. Inability to comply with study guidelines.
H. Cytopenia: platelet count <80,000/mm3, absolute neutrophil count <1500/mm3, hematocrit < 20%.
I. Other uncontrolled disease (co-morbidity) that could prevent a subject from fulfilling the study requirements or that would increase the risk of study procedures.
J. Patients who have a present malignancy or previous malignancy within the last 5 years prior to screening (except documented history of cured non-metastatic squamous or basal cell skin carcinoma or cervical carcinoma in situ). Patients who had a screening procedure that is suspicious for malignancy, and in whom the possibility of malignancy cannot be reasonably excluded following additional clinical, laboratory or other diagnostic evaluations.
K. Receipt of an investigational agent or device within 30 days prior to enrollment
L. A live vaccination < 4 weeks before enrollment
M. Presence of any of the following diseases:
1. ANCA-associated vasculitis
2. Polyarteritis nodosa
3. Giant cell arteritis
4. Takayasu's arteritis
5. Cogan's syndrome
6. Tuberculosis or atypical mycobacterial infections
7. Deep fungal infections
8. Lymphoma, lymphomatoid granulomatosis, or other type of malignancy that mimics vasculitis
9. Overlap with other systemic autoimmune diseases which would impair assessment of response
10. Diagnosis of VEXAS syndrome