Nebulized UC-EVs for Recurrent or Refractory IMIDs With Lung Involvement

This trial is an investigator initiated, multicenter, open-label, single-arm, dose-escalation trial. The main goal is to learn whether nebulized UC-EVs are safe and tolerable in adults with recurrent or refractory IMIDs with lung involvement.\]. Researchers will also look for early signs of whether UC-EVs may help improve lung function, lung imaging findings, symptoms, and disease-related laboratory tests. The study will include patients aged 18 and 80 years with IMID-related lung involvement. Eligible participants will have a diagnosis of IgG4-RD or SSc for at least 6 months. They must be receiving stable standard-of-care (SOC) treatment for the required period, as described in the inclusion criteria. They must also have adequate organ function and no history of severe allergy, as described in the eligibility criteria. Eligible participants will: * Inhale nebulized UC-EVs once daily for 7 days, followed by 7 days without treatment * Repeat this 2-week treatment cycle 6 times, for a total treatment period of about 12 weeks * Have study visits during treatment and follow-up visits through week 52 * Have safety checks, lung function tests, chest HRCT scans, blood tests, and symptom assessments * Record symptoms, adverse events, and any rescue treatment in a study diary