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IgGn 4相关性疾病（IgGn 4-RD）是一种较罕见的由免疫介导的慢性炎症伴纤维化疾病，可累及全身多个器官和系统，临床表现复杂多样。由于对该病认识时间较短，我国IgGn 4-RD的整体诊治水平参差不齐，国内亦无相关专家共识或诊治指南。为进一步提高各专业医生对IgGn 4-RD的认识和规范诊治水平，由中国罕见病联盟与中华医学会风湿病学分会联合组织专家组，在总结国内外经验和研究结果的基础上，制定了本共识，旨在统一我国临床医生对该病的诊治认识，减少漏诊和误诊，改善患者预后。n “,”IgGn 4 related disease (IgGn 4-RD) is an immune medicated rare disease, characterized with chronic inflammation and fibrosis in the involved organs, it is a systemic disease affected nearly every anatomic site of the body, usually involvement of multiple organs, and with diverse clinical manifestations. Due to the the relative novelty of the disease and under-recognition, the overall level of diagnosis and treatment in China is uneven. Till now, there is no relevant expert consensus or guidance of IgGn 4-RD in China. In order to further improve the understanding and standardize the management of IgGn 4-RD, on the basis of summarizing domestic and international experience, the China Alliance For Rare Diseases, together with the Chinese Rheumatology Association, organized an expert group and established the Chinese expert consensus on the diagnosis and treatment of IgGn 4 related diseases.n