Lupus mastitis heralding macrophage activation syndrome: Case report and systematic review of the literature

ObjectiveThe purpose of this systematic review study was to showcase diagnostic challenges posed by lupus mastitis (LM).MethodsHere we report a case of a 47-year- old Caucasian female with LM heralding systemic lupus erythematosus (SLE) complicated by macrophage activation syndrome (MAS), in the light of a systematic review of the literature on LM.ResultsIncluding our patient, we identified 32 case reports of patients with LM in the course of SLE. Only in 6 cases, including ours, LM preceded SLE diagnosis. Ours is the first ever published case report of LM heralding MAS.ConclusionLM in the course of SLE is rare and published data is very limited. Diagnosis should be made combining physical exam, laboratory tests, imaging and histology results, and include differentiation from other autoimmune, malignant and infectious causes. Based on the reviewed literature, it is advised to consider minimally invasive core biopsies in lupus patients over open biopsies as the associated trauma may exacerbate local inflammation. Conservative treatment with immunosupressive and anti-inflammatory drugs allows for control of breast symptoms in most cases.