The goal of this clinical trial is to explore the effectiveness of sequential use of rituximab and belimumab in the treatment of resistant primary juvenile Sjogren's syndrome. Does sequential use of rituximab and belimumab reduce the activity of SS in resistant patients Researchers will compare the disease activity before and after the treatment of sequential use of rituximab and belimumab to see if the therapy works to treat SS. Participants will: Recieve Rituximab each week for 2-4 times until B%\<0.5% or B#\<20×10\^6/L Recieve Belimumab 4 weeks after the last use of Rituximab, and then every 4 weeks until week 28
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
15
Rituximab weekly until B%\<0.5% or B#\<20×10\^6/L
Belimumab every four weeks
Peking Union Medical College Hospital
Beijing, Beijing Municipality, China
RECRUITINGRespondence rate
In the context of the aforementioned criteria, a response is defined as the alteration of more than three of the following five indicators: a) Improvement of physician global assessment score by ≥30% b) Decrease in erythrocyte sedimentation rate (ESR) by ≥30% or normalization c) Improvement of B cell activation markers (IgG, RF) by ≥25% d) Improvement of lacrimal gland function: Improvement of Schirmer test by ≥5mm e) Improvement of salivary gland function: Increase in salivary flow rate by 25% or decrease in ultrasound score by ≥25%.
Time frame: Week 28
Change From Baseline in European League Against Rheumatism (EULAR) Sjogren's Syndrome Disease Activity Index (ESSDAI) at Week 28
The ESSDAI is a physician-administered tool designed to measure disease activity. It consists of 12 organ-specific 'domains' contributing to disease activity associated with the participant's Sjogren's Syndrome only (constitutional, lymphadenopathy, articular, muscular, cutaneous, glandular, pulmonary, renal, peripheral nervous system, central nervous system, hematological, biological). Each domain is assessed for activity level (i.e., no, low, moderate, high) and assigned a numerical score based on pre-determined weighting of each individual domain. Overall score (ranges from 0 (no activity) to 123 (worst activity)) is calculated as sum of all individual weighted domain scores. A negative change from baseline value indicates improvement.
Time frame: week 0 and 28
Change From Baseline in EULAR Sjogren's Syndrome Patient Reported Index (ESSPRI) at Week 28
The ESSPRI is a participant-reported questionnaire to assess subjective participant symptoms and includes 3 domains (dryness, pain, and fatigue). Each domain is scored on scale of 0-10 (0 = no symptom at all and 10 = worst symptom imaginable), and an overall score is calculated as the mean of the three individual domains where all domains carry the same weight. Minimum score can be 0 and maximum score can be 10. A negative change from baseline value indicates improvement.
Time frame: week 0 and 28
the Sjögren's Tool for Assessing Response (STAR)
The STAR is a composite responder index that includes 5 items, and the score is from 0 to 9, while more than 5 points means improvement.
Time frame: week 28
Schirmer test
Schirmer test is a assay to detect the production of tears and less than 5mm means abnormal.
Time frame: Week 0 and 28
salivary flow rate
Salivary flow rate is measured by unstimulated whole saliva. A higher salivary flow rate represents lower disease activity.
Time frame: week 0 and 28
Score of salivary ultrasounds
The Hocevar scoring system was used investigating (1) parenchymal echogenicity compared with the thyroid gland, graded 0-1; (2) homogeneity, graded 0-3; (3) presence of hypoechogenicareas, graded 0-3; (4) hyperechogenic reflections, graded 0-3 in parotid glands and 0-1 in submandibular glands; and (5) clearness of the salivary gland border, graded 0-3, in both parotid and submandibular salivary glands. Total ultrasound score was the sum of these five domains and can range from 0 to 48. A higher score usually represents more severe disease.
Time frame: week 0 and 28
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