IgG4関連疾患疑診群の1 例

the lesion.

Administration of steroids might be effective

for IgG4-RD depending on the patientʼs condition,

but some reports have described relapse during

steroid tapering24). Because IgG4-RD in the

breast does not cause serious organ damage, we

observed it and did not administer steroid

treatment. However, follow-up is necessary

because lesions in other organs can lead to serious

pathological conditions.

Conclusion

We reported an additional case of IgG4-related

mastitis. IgG4-RD should be considered the

primary diagnosis if the clinicopathological findings show fibrotic, mass-like, and hypertrophic

lesions in organs throughout the body with

lymphoproliferation. IgG4-RD may lead to serious

pathological conditions in other major organs,

such as pancreatitis and nephritis. Steroid therapy

is the first choice, but it is careful that some

reports have described relapse during steroid

tapering. Because the number of cases is small,

further studies are needed to clarify the clinicopathological features and the diagnostic criteria.

Ethics approval and consent to participate

Not applicable

Consent for publication

Written informed consent was obtained from

1)

Higashiyama T, Nishida Y, Ugi S, Ishida M,

Nishio Y and Ohji M : A case of extraocular

muscle swelling due to IgG4-related sclerosing

disease. Jpn J Ophthalmol 55 : 315-317, 2011.

2) Ishida M, Hotta M, Kushima R, Shibayama M,

Shimizu T and Okabe H : Multiple IgG4-related

sclerosing lesions in the maxillary sinus, parotid

gland and nasal septum. Pathol Int 59 : 670-675,

2009.

3) Wong S, Lam WY, Wong WK and Lee KC :

Hypophysitis presented as inflammatory

pseudotumor in immunoglobulin G4-related

systemic disease. Hum Pathol 38 : 1720-1723,

2007.

4) Cheuk W and Chan JK : Lymphadenopathy of

IgG4-related disease : an underdiagnosed and

overdiagnosed entity. Semin Diagn Pathol 29 :

226-234, 2012.

5) Watanabe T, Maruyama M, Ito T, Fujinaga Y,

Ozaki Y, Maruyama M, Kodama R, Muraki T,

Hamano H, Arakura N, Kadoya M, Suzuki S,

Komatsu M, Shimojo H, Notohara K, Uchida M

and Kawa S : Clinical features of a new disease

concept, IgG4-related thyroiditis. Scand J

Rheumatol 42 : 325-330, 2013.

6) Zen Y, Inoue D, Kitao A, Onodera M, Abo H,

Miyayama S, Toshifumi G, Matsui O and

Nakanuma Y : IgG4-related lung and pleural

disease : A clinicopathologic study of 21 cases.

Am J Surg Pathol 33 : 1886-1893, 2009.

7) Kasashima S, Zen Y, Kawashima A, Endo M,

Matsumoto Y and Kasashima F : A new

clinicopathological entity of IgG4-related inflammatory abdominal aortic aneurysm. J Vasc

Surg 49 : 1264-1271, 2009.

Possible case of IgG-related mastitis

8)

9)

10)

11)

12)

13)

14)

15)

16)

17)

18)

Ishida M, Hotta M, Kushima R, Asai T and

Okabe H : IgG4-related inflammatory aneurysm

of the aortic arch. Pathol Int 59 : 269-273, 2009.

Kawano M, Saeki T and Nakashima H : IgG4-related kidney disease and retroperitoneal fibrosis : An update. Mod Rheumatol 29 : 231-239,

2019.

Nakanuma Y, Ishizu Y, Zen Y, Harada K and

Umemura T : Histopathology of IgG4-related

autoimmune hepatitis and IgG4-related hepatopathy in IgG4-related disease. Semin Liver Dis

36 : 229-241, 2016.

Tokura Y, Yagi H, Yanaguchi H, Majima Y,

Kasuya A, Ito T, Maekawa M and Hashizume

H : IgG4-related skin disease. Br J Dermatol

171 : 959-967, 2014.

Cheuk W, Chan AC, Lam WL, Chow SM,

Crowley P, Lloydd R, Campbell L, Thorburn M

and Chan JK : IgG4-related sclerosing mastitis :

description of a new member of the IgG4-related sclerosing diseases. Am J Surg Pathol 33 :

1058-1064, 2009.

Zen Y, Kasahara Y, Horita K, Miyayama S,

Miura S, Kitagawa S and Nakanuma Y :

Inflammatory pseudotumor of the breast in a

patient with a high serum IgG4 level : histologic

similarity to sclerosing pancreatitis. Am J Surg

Pathol. 29 : 275-278, 2005.

Dítě P, Trna J, Kinkor Z, Novotný I, Lata J,

Kianička B and Hermanová M : Unusual multiorgan immunoglobulin G4 (IgG4) inflammation :

autoimmune pancreatitis, Mikulicz Syndrome,

and IgG4 Mastitis. Gut Liver 7 : 621-624, 2013.

Ogiya A, Tanaka K, Tadokoro Y, Kikutani M,

Uematsu T, Kashiwagi H, Kasami M and

Takahashi K : IgG4-related sclerosing disease of

the breast successfully treated by steroid

therapy. Breast Cancer 21 : 231-235, 2014.

Chougule A, Bal A, Das A and Singh G : IgG4

related sclerosing mastitis : expanding the

morphological spectrum of IgG4 related diseases. Pathology 47 : 27-33, 2015.

Moriya T, Hirakawa H, Nagashima M, Yasuda

M and Kimijima I : IgG4-related disease of the

breast : a systemic disease whose mammary

manifestations mimic breast cancer. Int Cancer

Conf J 4 : 67-72, 2015.

Yamada R, Horiguchi S, Yamashita T and

57

Kamisawa T : IgG4-related mastitis, a rare

disease, can radiologically and histologically

mimic malignancy. BMJ Case Rep 2016 :

bcr2016214870,2016.

19) Tan ES, Friesen B, Loh SF and Fox J :

Immunoglobulin-G4 related mastitis : A case

report. Int J Surg Case Rep 37 : 169-172, 2017.

20) Vitkovski T, Marder GS, Filardi DA, Gupta E and

Breuer F : IgG4-related sclerosing disease of the

breast in a male patient. Int J Surg Pathol 25 :

711-715, 2017.

21) Kriegsmann M, Gomez C, Heil J, Schäfgen B,

Gutjahr E, Kommoss FKF, Kriegsmann K,

Flechtenmacher C, Goeppert B and Sinn HP :

IgG4-related sclerosing mastitis in a

49-year-old patient with multiple, tumor-like

nodules-Diagnostic accuracy of core needle

biopsy. Breast J 25 : 1251-1253, 2019.

22) Yokoe T, Hayashida T, Kikuchi M, Watanuki R,

Nakashoji A, Maeda H, Toyota T, Seki T,

Takahashi M, Iwasaki E, Mikami S, Kameyama

K and Kitagawa Y : IgG4-related mastopathy :

A case report and literature review. Clin Case

Rep 6 : 1549-1553, 2018.

23) Tsuda B, Kumaki N, Ishida R, Mizuno M,

Yokoyama K, Oshitanai R, Terao M, Morioka T,

Okamura T, Saito Y, Suzuki Y and Niikura N :

Distinction of IgG4-related mastitis from breast

cancer : a case report. Surg Case Rep 5 : 123,

2019.

24) Umehara H, Okazaki K, Masaki Y, Kawano M,

Yamamoto M, Saeki T, Matsui S, Yoshino T,

Nakamura S, Kawa S, Hamano H, Kamisawa T,

Shimosegawa T, Shimatsu A, Nakamura S, Ito

T, Notohara K, Sumida T, Tanaka Y, Mimori T,

Chiba T, Mishina M, Hibi T, Tsubouchi H, Inui K

and Ohara H : Comprehensive diagnostic

criteria for IgG4-related disease (IgG4-RD),

2011. Mod Rheumatol 22 : 21-30, 2012.

25) Sato Y, Kojima M, Takata K, Morito T,

Mizobuchi K, Tanaka T, Inoue D, Shiomi H,

Iwao H and Yoshino T : Multicentric

Castlemanʼs disease with abundant IgG4-positive cells : a clinical and pathological analysis of

six cases. J Clin Pathol 63 1084-1089, 2010.

(Received for publication October 1, 2020)

58

K. Endo et al.

（和文抄録）

IgG4 関連疾患疑診群の 1 例

1)

関西医科大学附属病院 乳腺外科

関西医科大学附属病院 病理診断科

3)

関西医科大学附属病院 放射線科

2)

遠藤香代子1)，石 田 光 明2)，木川雄一郎1)，平 井 千 惠1)，多田真奈美1)，

直 木3)，関 本 貢 嗣1)，杉 江 知 治1)

矢 内 洋 次1)，菅

IgG4 関連疾患（IgG4-RD）は，一疾患として認識されており，乳腺における IgG4-RD は極めて珍し

い．乳房における IgG4 関連疾患疑診群と診断した症例について報告する．症例は，55 歳の日本人女

性で，左乳房腫瘤を自覚した．超音波検査では，左 C 区域の低エコーと両側の腋窩リンパ節腫大を示

した．その後左乳房腫瘤の増大と右乳房腫瘍が指摘されたため，左乳房腫瘤に対して腫瘤摘出術が行

われた．術後，耳下腺腫瘍と複数の肺結節が出現し，血清 IgG4 レベルが上昇していた（144mg/dL）．

手術標本の病理結果は，リンパ濾胞周囲を主体に多数形質細胞が浸潤し，一部で線維化・硝子化を伴

い静脈炎も観察された．免疫染色では，IgG4 陽性の形質細胞浸潤を認めたが，IgG4/IgG 陽性形質細

胞比は 23.6％であったため，IgG4 関連疾患疑診群の診断となった．

この疾患の乳腺における診断基準がまだ確立されていないため，IgG4 関連乳腺炎の臨床病理学的

特徴を明らかにするために症例の蓄積が必要である．

キーワード：IgG4 関連疾患，IgG4 陽性形質細胞，乳腺炎

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