The First Department of Internal Medicine, University of Occupational and Environmental Health Japan, Kitakyushu, Japan.
Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
Mod Rheumatol. 2023 Mar 2;33(2):229-236. doi: 10.1093/mr/roac141.
Understanding of the pathophysiology of immunoglobulin G4-related disease (IgG4-RD) over the last dozen years has opened the door to a variety of targeted treatment approaches. Glucocorticoids are an effective treatment for IgG4-RD if used at a sufficiently high dose, but disease flares are common during or after glucocorticoid tapers and these medications seldom lead to long-term, treatment-free remissions. Moreover, their long-term use in a disease that frequently affects middle-aged to elderly individuals and often causes major pancreatic damage leads to a narrow therapeutic index. Biological therapies offer the possibility of effective disease control with fewer treatment-associated side effects. Promising avenues of investigation include B-cell depletion, immunomodulation of B-cell subsets, interference with co-stimulation, Bruton's tyrosine kinase inhibition, and Signaling lymphocytic activation molecule F7-directed treatment.
在过去的十几年中，对免疫球蛋白 G4 相关疾病 (IgG4-RD) 病理生理学的认识为各种靶向治疗方法开辟了道路。如果使用足够高的剂量，糖皮质激素是 IgG4-RD 的有效治疗方法，但在糖皮质激素递减期间或之后疾病常会复发，而且这些药物很少能导致长期、无需治疗的缓解。此外，在一种经常影响中年到老年人群且常导致主要胰腺损伤的疾病中，长期使用这些药物会导致治疗指数较窄。生物疗法提供了以较少治疗相关副作用有效控制疾病的可能性。有前途的研究途径包括 B 细胞耗竭、B 细胞亚群的免疫调节、共刺激干扰、布鲁顿酪氨酸激酶抑制和信号淋巴细胞激活分子 F7 靶向治疗。
