Mikulicz’s syndrome: one of immunoglobulin G4–related diseases
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Abstract
Abstract
Mikulicz’s syndrome is clinically characterized by the bilateral symmetrical enlargement of the lacrimal, parotid and submandibular salivary glands without tenderness. So far it is considered the very rare entity. Herein we additionally reported one case of Mikulicz’s syndrome that was clinically diagnosed in a Thai woman. She was a 42-year-old Thai patient who developed the slowly progressive symmetrical enlargement of parotid glands without pain for 6 months. She had no
fever or weight loss. The physical examination revealed the symmetrical enlargement of bilateral lacrimal, parotid and submandibular salivary glands with firm consistency and normal covering skin but no tenderness and no lymphadenopathy. The chest film showed unremarkable study. Her blood tests showed: Hb 13.2 g%, WBC 6,800/mm3, platelet 299,000/mm3, ESR 60 mm/hr, anti-SSA (Ro)-negative, anti-SSB (La)-negative, IgG1 905.0 mg/dl, IgG2 475.0 mg/dl, IgG3 279.9 mg/dl, IgG4
1,141.9 mg/dl, ANA-negative, normal thyroid function, amylase 55.0 U/l, albumin 3.8 g%, globulin 4.3 g%, creatinine 0.67 mg%, Hb A1c 5.1%, HIV antigen / antibody and VDRL-negative. She refused the gland biopsy. She was clinically diagnosed as having Mikulicz’s syndrome and treated with oral prednisolone 10 mg a day. All tumor masses gradually decreased in size every month. The serum IgG4 was lowered to be 637.2 mg/dl and ESR to 25 mm/hr in one month. This case seemingly
supported Mikulicz’s syndrome was one of IgG4 related-diseases because her serum IgG4 was strikingly rising at the initial presentation and lowered after treatment in accordance to decreased size of all tumor masses, not related to Sjogren’s syndrome because the ANA and anti-SSA tests
which were frequently positive in Sjogren’s syndrome were not found.
Article Details
ต้นฉบับที่ส่งมาพิจารณายังวารสารหู คอ จมูก และใบหน้า จะต้องไม่อยู่ในการพิจารณาของวารสารอื่น ในขณะเดียวกันต้นฉบับที่จะส่งมาจะผ่านการอ่านโดยผู้ทรงคุณวุฒิ หากมีการวิจารณ์หรือแก้ไขจะส่งกลับไปให้ผู้เขียนตรวจสอบแก้ไขอีกครั้ง ต้นฉบับที่ผ่านการพิจารณาให้ลงตีพิมพ์ถือเป็นสมบัติของวารสารหู คอ จมูกและใบหน้า ไม่อาจนำไปลงตีพิมพ์ที่อื่นโดยไม่ได้รับอนุญาต
ตารางแผนภูมิ รูปภาพ หรือข้อความเกิน 100 คำที่คัดลอกมาจากบทความของผู้อื่น จะต้องมีใบยินยอมจากผู้เขียนหรือผู้ทรงลิขสิทธิ์นั้นๆ และใหร้ะบุกำกับไว้ในเนื้อเรื่องด้วย
References
2. Yamamoto M, Harada S, Ohara M, et al. Clinical and pathological differences between Mikulicz’s disease and Sjogren’s syndrome. Rheumatol 2005; 44: 227-34.
3. Yamamoto M, Takahashi H, Ohara M, et al. A new conceptualization for Mikulicz’s disease as an IgG4-related plasmacytic disease. Mod Rheumatol 2006; 16: 335-40.
4. Bhattacharjee A, Uddin S, Prakash R, Rathor A, Kalita S. Diagnostic and management challenges in Mikulicz syndrome. Int J Head Neck Surg 2015; 6: 139-45.
5. Wang L, Niu W. IgG4-related Mikulicz disease in a 76-year-old Chinese male: a case report. J Ocul Immunol Inflam 2015; 23: 173-5.
6. Božić K, Glišić B, Radić-Tasić O, Knežević B. Case report of Mikulicz`s disease – a modern concept of an old entity. Vojnosanit Pregl 2016; 73: 393-6.
7. Rao D, Natter P, Fernandes R, Wang ZB, Sandhu SJS. A case report of Mikulicz Syndrome. J Radiol Case Rep 2017; 11: 1–7.
8. Masaki Y, Dong L, Kurose N, et al. Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis 2009; 68: 1310-5.
9. Uchida K, Masamune A, Shimosegawa T, Okazaki K. Prevalence of IgG4-related disease in Japan based on nationwide survey in 2009. Int J Rheumatol 2012: ID 358371, 5 pages. doi:10.1155/2012/358371.
10. Angsupankosol A, Sukto C, Insiripong S. Mikulicz’ syndrome: a case report. Nakhon Racth Med Bull 2018; 40: 187-91.
11. Takahashi H, Yamamoto M, Tabeya T, et al. The immunobiology and clinical characteristics of IgG4 related diseases. J Autoimmun 2012; 39: 93-6.
12. Yamamoto M, Ohara M, Suzuki C, et al. Elevated IgG4 concentrations in serum of patients with Mikulicz's disease. Scand J Rheumatol 2004; 33: 432-3.
13. Masaki Y, Sugai S, Umehara H. IgG4-related diseases including Mikulicz's disease and sclerosing pancreatitis: diagnostic insights. J Rheumatol 2010; 37: 1380-5.
14. Toyoda K, Oba H, Kutomi K, et al. MR imaging of IgG4-related disease in the head and neck and brain. AJNR Am J Neuroradiol 2012; 33: 2136–9.
15. Fayyaz A, Kurien BT, Scofield H. Autoantibodies in Sjogren’s syndrome. Rheum Dis Clin North Am 2016: 42: 419-34.
16. Yamamoto M, Takahashi H, Sugai S, Imai K. Clinical and pathological characteristics of Mikulicz's disease (IgG4-related plasmacytic exocrinopathy). Autoimmun Rev 2005; 4: 195-200. .
17. Himi T, Takano K, Yamamoto M, Naishiro Y, Takahashi H. A novel concept of Mikulicz's disease as IgG4-related disease. Auris Nasus Larynx 2012; 39: 9-17.