Phase II prospective treatment study for IgG4+MOLPS
- Conditions
- IgG4+MOLPS (IgG4+Multiorgan lymphoproliferative syndrome)
- Registration Number
- JPRN-UMIN000002311
- Lead Sponsor
- Department of Hematology and Immunology, Kanazawa Medical University
- Brief Summary
RESULTS: This study enrolled 61 patients. After clinicopathological review, three patients were excluded, and one, 13, and 44 patients were diagnosed with probable, possible, and definite IgG4-related disease, respectively. Of the 44 patients with definite IgG4-RD, 29 (65.9%) achieved CR, and the ORR was 93.2%. No patient was refractory to primary treatment. The most frequent adverse events were glucose intolerance. Six patients relapsed. CONCLUSIONS: Glucocorticoid treatment is usually effective for patients with IgG4-RD, and we should examine the possibility of other disorders when a patient is glucocorticoid refractory. Some patients are misdiagnosed, making central clinicopathological review of diagnosis very important in conducting clinical studies.
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- Complete: follow-up complete
- Sex
- All
- Target Recruitment
- 57
Not provided
1, Castleman disease, Wegener granulomatosis, Sarcoidosis, cancer lymphoma, and other distinct disorders. 2, severe infectious disease 3, pre-treatment of steroid or immuno-suppressive drugs 4, physician's decision
Study & Design
- Study Type
- Interventional
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method CR rate
- Secondary Outcome Measures
Name Time Method Maintenance dose of steroid, relapse rate, adverse effects