IgG4-related sclerosing mastitis (IgG4-RM) is a rare, benign, mass-forming fibroinflammatory disease of the breast that can mimic a neoplastic process and pose a diagnostic challenge to clinicians and pathologists. We present a case of IgG4-RM in an 84-year-old woman and highlight the characteristic morphologic findings that should allow the pathologist to raise the possibility of this entity. We also briefly discuss pathophysiology and differential diagnosis. Awareness of clinical and morpholog...
Cystic neutrophilic granulomatous mastitis (CNGM) is a rare subtype of granulomatous mastitis with a highly distinct histological pattern often associated with Corynebacterium species. CNGM is characterised by suppurative lipogranulomas that are composed of central lipid vacuoles rimmed by neutrophils and an outer cuff of epithelioid histiocytes. Some of the lipid vacuoles may contain sparse, rod-shaped, gram-positive bacilli that can be easily missed or dismissed. The surrounding mixed inflamma...
OBJECTIVE: IgG4-related disease (IgG4-RD) is a unique inflammatory disorder in which Th2 cytokines promote IgG4 production. In addition, recent studies have implicated the Toll-like receptor (TLR) pathway. This study was undertaken to examine the expression of TLRs in salivary glands (SGs) from patients with IgG4-RD. METHODS: SGs from 15 patients with IgG4-RD, 15 patients with Sjogren's syndrome (SS), 10 patients with chronic sialadenitis, and 10 healthy controls were examined histologically. TL...
: Rosai-Dorfman disease (RDD) is an uncommon disorder, characterized by an atypical expansion of histiocytes which classically shows emperipolesis and immunoreactivity with S-100 protein. RDD affects the lymph nodes as well as extranodal sites; however, RDD of the breast is exceptionally rare. Herein, we describe the histopathologic features of 22 cases of RDD occurring in the breast, with an emphasis on the differential diagnosis. All cases were notable for an exuberant lymphocytic infiltrate w...
Last. Hans-Peter Sinn(University Hospital Heidelberg)H-Index: 39
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: Recently, it has been reported that IgG4-related disease may occur in the breast manifesting as nodular sclerosing interstitial mastitis. Here we report a case with multiple tumor-like nodules in one breast. The histologic diagnosis was established on core needle biopsies, and treatment was initiated without open biopsy. Diagnosis of IgG4-related sclerosing mastitis should be suspected in cases of tumor-like lesions on imaging with an interstitial plasma cell-rich sclerosing inflammation on hi...
Background Immunoglobulin (Ig) G4-related sclerosing disease is a pathological concept proposed in Japan during the early 2000s. This lesion-forming disease may exhibit characteristics of a systemic disease but often affects a single organ. To date, IgG4-related sclerosing disease in the mammary gland, or IgG4-related mastitis (IgG4-RM), has rarely been reported.
#2Panpan Zhang(Peking Union Medical College Hospital)H-Index: 7
Last. Wen Zhang(Peking Union Medical College Hospital)H-Index: 26
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OBJECTIVES: To study the impact of sex on the clinical presentation of IgG4-related disease (IgG4-RD). METHODS: We prospectively enrolled 403 newly diagnosed IgG4-RD patients. We compared the demographic features, clinical manifestations, organ involvement, laboratory tests and treatment outcomes between female and male patients. The organs involved were divided into superficial organs (salivary glands, lacrimal glands, orbit, sinus and skin) and internal organs (all the other organs). The patie...
Objective IgG4-related disease (IgG4-RD) is a heterogeneous, multiorgan condition of unclear aetiology that can cause organ failure. Difficulty recognising IgG4-RD contributes to diagnostic delays. We sought to identify key IgG4-RD phenotypes. Methods We used two cross-sectional studies assembled by an international, multispecialty network of IgG4-RD specialists who submitted 765 cases to derive and replicate phenotypic groups. Phenotype groups of disease manifestations and key covariate distrib...