Mastitis in Autoimmune Diseases: Review of the Literature, Diagnostic Pathway, and Pathophysiological Key Players

Abstract

Mastitis frequently affects women of childbearing age. Of all the pathological breast conditions requiring specific management, autoimmune mastitis is in the third position after infection and breast cancer. The aim of this literature review was to make a comprehensive description of autoimmune diseases targeting the mammary gland. Four main histological patterns of autoimmune mastitis are described: (i) lymphocytic infiltrates; (ii) ductal ectasia; (iii) granulomatous mastitis; and (iv) vasculitis. Our literature search found that all types of autoimmune disease may target the mammary gland: organ-specific diseases (diabetes, thyroiditis); connective tissue diseases (such as systemic erythematosus lupus or Sjögren’s syndrome); vasculitides (granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, giant cell arteritis, polyarteritis nodosa, Behçet’s disease); granulomatous diseases (sarcoidosis, Crohn’s disease); and IgG4-related disease. Cases of breast-specific autoimmune diseases have also been reported, including idiopathic granulomatous mastitis. These breast-limited inflammatory diseases are sometimes the first symptom of a systemic autoimmune disease. Although autoimmune mastitis is rare, it is probably underdiagnosed or misdiagnosed. Early diagnosis may allow us to detect systemic diseases at an earlier stage, which could help to initiate a prompt, appropriate therapeutic strategy. In case of suspected autoimmune mastitis, we hereby propose a diagnostic pathway and discuss the potential pathophysiological pathways leading to autoimmune breast damage.

Granulomatosis with polyangiitis: a life‐threatening cause of granulomatous mastitis

“…The histologic pattern of lobulocentric granulomatous inflammation can also be observed in malignancies, infections and autoimmune conditions. Thus, idiopathic granulomatous mastitis must remain a diagnosis of exclusion, especially in women who are nulliparous or post‐menopausal 5 …”

“…Thus, idiopathic granulomatous mastitis must remain a diagnosis of exclusion, especially in women who are nulliparous or post-menopausal. 5 In addition to standard triple assessment of the breast, work-up for granulomatous mastitis includes submission of tissue samples for bacterial and mycobacterial culture, together with Quantiferon-Gold blood test, to exclude tuberculosis and alternative infective aetiologies. 5 Autoimmune granulomatous disorders including sarcoidosis and vasculitis should be considered through chest X-ray, serum angiotensin converting enzyme and anti-neutrophil cytoplasmic antibodies.…”

“…5 Autoimmune granulomatous disorders including sarcoidosis and vasculitis should be considered through chest X-ray, serum angiotensin converting enzyme and anti-neutrophil cytoplasmic antibodies. 5 Prolactin should also be measured as hyperprolactinaemia may play an aetologic role. 5 GPA is a similarly rare condition characterized by systemic vasculitis which classically involves the upper and lower respiratory tracts, and kidneys, often in association with non-specific constitutional symptoms.…”

“…5 Prolactin should also be measured as hyperprolactinaemia may play an aetologic role. 5 GPA is a similarly rare condition characterized by systemic vasculitis which classically involves the upper and lower respiratory tracts, and kidneys, often in association with non-specific constitutional symptoms. 6 The presence of c-ANCA/proteinase-3 antibody is highly specific.…”

Effectiveness of Comprehensive Traditional Chinese Therapy in Treating Granulomatous Lobular Mastitis: a Systematic Review and a Meta-analysis

“…Granulomatous lobular mastitis (GLM) is an Idiopathic Granulomatous Mastitis (IGM) that mainly encroaches on the breast lobule. And it is a rare benign inflammatory breast disease to which women of childbearing age are susceptible [1] [2] . It is often considered as an autoimmune disease which was first proposed by Kessler et al [3] in 1972.…”