Background: Idiopathic granulomatous mastitis (IGM) is a rare, benign inflammatory breast condition often mistaken for inflammatory breast cancer and, therefore, requires a biopsy for accurate diagnosis. Although not cancerous, IGM can cause emotional distress because of severe pain and ensuing breast deformity. Differentiating IGM from other breast inflammations caused by infections is essential. IGM mostly affects premenopausal women and is potentially associated with recent pregnancies and breastfeeding. The risk factors, including smoking and contraceptive use, have inconsistent associations. Steroid responses suggest an autoimmune component, though specific markers are lacking. Methods: We performed a narrative review on potential risk factors, diagnostics, and therapy of IGM. Results: Diagnostics and clinical management of IGM are challenging. The treatment options include NSAIDs, steroids, surgery, antibiotics, immunosuppressants, prolactin suppressants, and observation, each with varying effectiveness and side effects. Conclusions: Current IGM treatment evidence is limited, based on case reports and small series. There is no consensus on the optimal management strategy for this disease. The GRAMAREG study by the EUBREAST Study Group aims to collect comprehensive data on IGM to improve diagnostic and treatment guidelines. By enrolling patients with confirmed IGM, the study seeks to develop evidence-based recommendations, enhancing patient care and understanding of this condition.
背景:特发性肉芽肿性乳腺炎(IGM)是一种罕见的良性乳腺炎症性疾病,常被误诊为炎性乳腺癌,因此需要通过活检进行准确诊断。尽管IGM并非恶性肿瘤,但其引起的剧烈疼痛及继发的乳房畸形可导致患者情绪困扰。将IGM与感染引起的其他乳腺炎症相鉴别至关重要。该病好发于绝经前女性,可能与近期妊娠及哺乳相关。吸烟、避孕药使用等风险因素与IGM的关联性尚未明确。类固醇治疗有效提示其存在自身免疫成分,但尚缺乏特异性标志物。 方法:本研究对IGM的潜在风险因素、诊断及治疗方法进行叙述性综述。 结果:IGM的诊断与临床管理具有挑战性。治疗方案包括非甾体抗炎药、类固醇、手术、抗生素、免疫抑制剂、催乳素抑制剂及观察随访等,各类方法的疗效与副作用存在差异。 结论:当前IGM的治疗证据主要基于病例报告和小样本研究,证据质量有限。目前尚未就该疾病的最佳管理策略达成共识。EUBREAST研究组开展的GRAMAREG研究旨在收集IGM的全面数据以完善诊疗指南。通过纳入确诊的IGM患者,该研究致力于制定循证医学建议,从而提升临床诊疗水平并深化对该疾病的认识。