Jeries Helana, Braun-Moscovici Yolanda, Balbir-Gurman Alexandra
Rheumatology Unit, Galilee Medical Center, Nahariya, Israel.
The Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel.
Rambam Maimonides Med J. 2024 Oct 28;15(4):e0018. doi: 10.5041/RMMJ.10532.
IgG4-related disease (IgG4-RD) is a rare illness with inflammatory and fibrotic changes in affected organs such as pancreas, thyroid, salivary or lacrimal glands, and retroperitoneal space; rarely other organs may be involved. IgG4-related breast disease (IgG4-BD) is very rare and generally presents as a lump or mastitis. IgG4-BD as a presenting feature of IgG4-RD is extremely rare. Hence, this paper reviews the known (n=48) IgG-BD cases reported in the literature to date. The majority of cases were diagnosed on routine mammography or during assessment for other clinically significant features. The absence of a lump border, and especially the absence of calcifications on ultrasonography, mammography, or computed tomography, is typical for IgG4-BD. Characteristic IgG4-BD pathological findings were dense lymphoplasmacytic infiltration with stromal fibrosis, and more than 10% IgG4 plasma cells/high-power field (HPF); the mean percentage of IgG4/IgG plasma cells was 54.2%, and only one-third of the patients had all "classical" signs of IgG4-BD including storiform fibrosis and obliterative phlebitis. Most of the cases had a benign course and responded to surgical excision with or without steroid therapy.
IgG4相关性疾病（IgG4-RD）是一种罕见疾病，在胰腺、甲状腺、唾液腺或泪腺以及腹膜后间隙等受累器官中出现炎症和纤维化改变；很少累及其他器官。IgG4相关性乳腺疾病（IgG4-BD）非常罕见，通常表现为肿块或乳腺炎。IgG4-BD作为IgG4-RD的首发特征极为罕见。因此，本文回顾了迄今为止文献中报道的已知（n = 48）例IgG4-BD病例。大多数病例是在常规乳腺钼靶检查或评估其他临床显著特征时被诊断出来的。IgG4-BD的典型表现是肿块边界不清，尤其是在超声、乳腺钼靶或计算机断层扫描上没有钙化。IgG4-BD的特征性病理表现为密集的淋巴浆细胞浸润伴间质纤维化，每高倍视野（HPF）有超过10%的IgG4浆细胞；IgG4/IgG浆细胞的平均百分比为54.2%，只有三分之一的患者具有IgG4-BD的所有“经典”体征，包括席纹状纤维化和闭塞性静脉炎。大多数病例病程良性，手术切除联合或不联合类固醇治疗有效。
