IgG4-related Sclerosing Mastitis: Description of a New Member of the IgG4-related Sclerosing Diseases

Abstract

Immunoglobulin G (IgG)4-related sclerosing disease is a recently described syndrome characterized by mass-forming lesions in various organs due to dense lymphoplasmacytic infiltrates and stromal sclerosis, elevated serum IgG4 titer, increased tissue IgG4 plasma cells, and favorable clinical outcome. We describe 4 patients with IgG4-related sclerosing mastitis, which represents a new member of this family of diseases. All patients were female with a mean age of 47.5 years, presenting with painless masses in 1 or both breasts. One patient had concurrent IgG4-related lymphadenopathy, and another had eyelid swelling of undetermined cause. The serum IgG4 titer was elevated in 1 tested patient, and circulating autoantibodies were found in 3 tested patients. All patients were well with no recurrence after excision or biopsy of the mass. Histologically, the breast masses featured dense lymphoplasmacytic infiltrates, prominent stromal sclerosis and loss of breast lobules. Phlebitis was present in 1 case. IgG4 cells ranged from 272 to 495 per high-power field, constituting 49% to 85% of all IgG cells. IgG4 cells were scarce in 9 of 9 cases of lymphocytic mastitis and 6 of 7 cases of granulomatous mastitis studied as controls. In summary, IgG4-related sclerosing mastitis appears to be a distinctive form of mastitis, sometimes accompanied by other components of IgG4-related sclerosing disease, and shows a favorable clinical outcome.

Immunoglobulin G4–related disease in Hong Kong: clinical features, treatment practices, and its association with multisystem disease

“…[8][9][10][11][12][13][14][15][16][17][18] Only those reporting pre-treatment serum IgG4 and IgG levels were included in our study. Treatment modalities were not reported in four patients.…”

“…A summary of patient demographics, clinical features, and treatment modalities is shown in Table 2. [8][9][10][11][12][13][14][15][16][17][18] As a result, a total of 104 patients were identified from Queen Mary Hospital and literature review. For patients with pre-treatment results available, 68/72 (94.4%) patients had a serum IgG4 level of >135 mg/dL, and 58/63 (92.1%) patients had an IgG4:IgG ratio of >8%.…”

Rare Non-Tumor Diseases of the Pharynx and Larynx

“…Симптомы зависят от ор-гана-мишени. Наиболее часто поражаются под-желудочная железа (с развитием аутоиммунного панкреатита) [1], билиарная система (склерози-рующий холангит) [2], слезные и слюнные желе-зы (болезнь Микулича) [3], элементы орбиты [4], щитовидная железа (тиреоидит Риделя) [5], поч-ки (тубулоинтерстициальный нефрит) [6], забрю-шинное пространство (ретроперитонеальный фиброз) [7], аорта (аортит) [8], кишечник (ме-зентерит), предстательная и молочная железы, легкие, перикард, мозговые оболочки, гипофиз, лимфатические узлы и кожа. Обширная инфиль-трация IgG4 клетками плазмы и Т-лимфоцитами пораженного органа или органов нарушает фор-му и функции тканей, а также инициирует ин-тенсивную воспалительную реакцию [9].…”

IgG4-Related Cardiovascular Disorders

“…5) In 2001, Type 1 AIP with elevated serum IgG4 level and abundant IgG4-positive lymphoplasmacyte infiltration in the pancreas was reported and the concept of IgG4-related disease was proposed. 6) Then, it was gradually discovered that IgG4-related disease involves inflammation and sclerosing in many other organs, 7) especially exocrine organs: 8) bile ducts, 9) lacrimal and salivary glands, 10) retroperitoneum, 11) urinary organs, [12][13][14] pituitary gland, 15) prostate, 16) arteries, [17][18][19][20] veins, [21][22][23] heart, [24][25][26] lung, 25,27,28) eye, 24,29) mesenterium, 30) mammary gland, 31) central nervous system, 32) esophagus, 33) stomach, 34) liver, 35) gastrointestinal tract, 36) thyroid, 37,38) nose, 39) blood, 40,41) skin, 42) and pseudotumors in various organs 9,14,25,[43][44]…”