Author(s): Venkata Triveni Devi Yerra, Stephanie Margaret Puvvada, Vinod Kumar Mugada, Srinivasa Rao Yarguntla


DOI: 10.52711/2231-5659.2023.00054   

Address: Venkata Triveni Devi Yerra1*, Stephanie Margaret Puvvada1, Vinod Kumar Mugada1, Srinivasa Rao Yarguntla2
1Department of Pharmacy Practice, Vignan Institute of Pharmaceutical Technology, Duvvada, AP, India.
2Department of Pharmaceutics, Vignan Institute of Pharmaceutical Technology, Duvvada, AP, India.
*Corresponding Author

Published In:   Volume - 13,      Issue - 4,     Year - 2023

IgG4-related disease (IgG4-RD) is an intricate autoimmune disorder that have an effect on multiple organs in the body, including the lachrymal and salivary glands, liver, biliary duct, thyroid, pancreas, gastrointestinal tract, kidney, aorta, prostate and retroperitoneum. Mikulicz disease, a subtype of IgG4-RD, specifically targets the lachrymal and salivary glands, leading to glandular swelling, xerostomia, and xerophthalmia. Diagnosis of Mikulicz disease is established on the presence of proportional and persistent inflammation of at least two pairs of lachrymal, submandibular, and parotid glands, along with exalted serum IgG4 levels and/or marked penetration of IgG4-positive plasmacytes into the affected glandular tissues. Treatment with glucocorticoids has shown to be beneficial in managing IgG4-related MD, but the efficacy of combination therapy in preventing relapse is still a topic of debate. The underlying causes of the disease can vary, but lymphoepithelial sialadenitis is the widespread cause. The disease typically presents in individuals with an average age ranging from 55 to 65 years, and the incidence among individuals under the age of 20 is exceedingly rare. Mikulicz disease is an enigmatic ailment that is still being researched. However, identifying its hallmark symptoms and distinguishing it from other similar conditions is essential for effective diagnosis and treatment. Further research is essential to better comprehend the fundamental causes of the disease and to promote more effective treatment options for patients with Mikulicz disease and other subtypes of IgG4-RD.

Cite this article:
Venkata Triveni Devi Yerra, Stephanie Margaret Puvvada, Vinod Kumar Mugada, Srinivasa Rao Yarguntla. Mikulicz Disease in Males: A Comprehensive Review. Asian Journal of Research in Pharmaceutical Sciences. 2023; 13(4):318-4. doi: 10.52711/2231-5659.2023.00054

Venkata Triveni Devi Yerra, Stephanie Margaret Puvvada, Vinod Kumar Mugada, Srinivasa Rao Yarguntla. Mikulicz Disease in Males: A Comprehensive Review. Asian Journal of Research in Pharmaceutical Sciences. 2023; 13(4):318-4. doi: 10.52711/2231-5659.2023.00054   Available on:

1.    Yao Q, Wu G, Hoschar A. IgG4-related Mikulicz’s disease is a multiorgan lymphoproliferative disease distinct from Sjögren’s syndrome: a Caucasian patient and literature review. Clinical and Experimental Rheumatology. 2013; 31(2):289–94.
2.    Doe K, Nozawa K, Okada T, Tada K, Yamaji K, Tamura N, et al. Usefulness of minor salivary gland biopsy in the diagnosis of IgG4-related disease: a case report. International Journal of Clinical and Experimental Pathology. 2014; 7(5):2673–7.
3.    Bhagwat NM, Tayde PS, Dalwadi PP, Sorabjee J, Varthakavi PK. Mikulicz’s Disease with hypophysitis — a new IgG4-mediated disorder. Endokrynologia Polska. 2016; 67(6):622–6.
4.    Narayan AK, Baer A, Fradin J. Sonographic findings of IgG4-related disease of the salivary glands: Case report and review of the literature. Journal of Clinical Ultrasound. 2017 ; 46(1):73–7.
5.    Masaki Y, Sugai S, Umehara H. IgG4-related Diseases Including Mikulicz’s Disease and Sclerosing Pancreatitis: Diagnostic Insights. The Journal of Rheumatology. 2010; 37(7):1380–5
6.    Uehara T, Ikeda S, Hamano H, Kawa S, Moteki H, Matsuda K, et al. A case of Mikulicz’s disease complicated by malignant lymphoma: a postmortem histopathological finding. Internal Medicine (Tokyo, Japan). 2012; 51(4):419–23.
7.    Vu DL, Thieu-Thi TM, Pham MT, Le-Thi M, Nguyen HA, Nguyen MD. A case report of Mikulicz’s disease. Pan African Medical Journal. 2020; 37(33598067).
8.    Yamamoto M, Harada S, Ohara M, Suzuki C, Naishiro Y, Yamamoto H, et al. Clinical and pathological differences between Mikulicz’s disease and Sjögren’s syndrome. Rheumatology (Oxford, England). 2005; 44(2):227–34.
9.    Hayashi Y, Moriyama M, Maehara T, Goto Y, Kawano S, Ohta M, et al. A case of mantle cell lymphoma presenting as IgG4-related dacryoadenitis and sialoadenitis, so-called Mikulicz’s disease. World Journal of Surgical Oncology. 2015 ; 13(1).
10.    Liu AC, Chen Y, Jia JS, Gao SY, Liu YY. [Non-Hodgkin’s lymphoma mimicking Mikulicz disease: a case report]. Beijing Da Xue Bao Yi Xue Ban = Journal of Peking University Health Sciences. 2016 ; 48(6):1074–6.
11.    Köhler S, Gargano M, Matentzoglu N, Carmody LC, Lewis-Smith D, Vasilevsky NA, et al. The Human Phenotype Ontology in 2021. Nucleic Acids Research. 2021; 49(D1):D1207–17.
12.    Yamamoto M, Takahashi H, Naishiro Y, Isshiki H, Ohara M, Suzuki C, et al. Mikulicz’s disease and systemic IgG4-related plasmacytic syndrome (SIPS). Japanese Journal of Clinical Immunology. 2008; 31(1):1–8.
13.    Kamiński B, Błochowiak K. Mikulicz’s disease and Küttner’s tumor as manifestations of IgG4-related diseases: a review of the literature. Reumatologia/Rheumatology. 2020 ; 58(4):243–50.
14.    Kubota K, Wada T, Kato S, Mozaki Y, Yoneda M, Fujita K, et al. Highly Active State of Autoimmune Pancreatitis With Mikulicz Disease. Pancreas. 2010 ; 39(1):e6.
15.    Piao Y, Wang C, Yu W, Mao M, Yue C, Liu H, et al. Concomitant occurrence of Mikulicz’s disease and immunoglobulin G4-related chronic rhinosinusitis: a clinicopathological study of 12 cases. Histopathology. 2015; 68(4):502–12.
16.    Coloma-González I, Ruíz-García L, Flores-Preciado J, Encampira-Luna EO, Ceriotto A, Salcedo-Casillas G. Enfermedad de Mikulicz. A propósito de un caso. Archivos de la Sociedad Española de Oftalmología. 2014; 89(8):332–5.
17.    Lee S, Tsirbas A, Mccann JD, Goldberg RA. Mikulicz’s Disease: A New Perspective and Literature Review. European Journal of Ophthalmology. 2006; 16(2):199–203.
18.    Yamamoto M, Takahashi H, Ishigami K, Yajima H, Shimizu Y, Tabeya T, et al. Evaluation and Clinical Validity of a New Questionnaire for Mikulicz’s Disease. International Journal of Rheumatology. 2012; 2012(283459):e283459
19.    Bhattacharjee A, Uddin S, Prakash R, Rathor A, Kalita S. Diagnostic and Management Challenges in Mikulicz Syndrome. International Journal of Head and Neck Surgery. 2015; 6(4):139–45.
20.    Rubin P, Besse BE. The Sialographic Differentiation of Mikulicz’s Disease and Mikulicz’s Syndrome. Radiology Society of North America. 1957; 68(4):477–87.
21.    Yamamoto M, Suzuki C, Naishiro Y, Takahashi H, Shinomura Y, Imai K. [The significance of disease-independence in Mikulicz’s disease--revival interests in Mikulicz’s disease]. Nihon Rinsho Men’eki Gakkai Kaishi = Japanese Journal of Clinical Immunology. 2006; 29(1):1–7
22.    Moriyama M, Tanaka A, Maehara T, Ohyama Y, Shimizu M, Nakashima H, et al. Clinical characteristics of Mikulicz’s disease as an IgG4-related disease. Clinical Oral Investigations. 2012; 17(9):1995–2002.
23.    Yamamoto M, Takahashi H, Shinomura Y. Mikuliczs Disease and its Extraglandular Lesions. Current Immunology Reviews (Discontinued). 2011; 7(2):162–71.
24.    Shimizu M, Moriyama M, Okamura K, Kawazu T, Chikui T, Goto TK, et al. Sonographic diagnosis for Mikulicz disease. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology and Endodontics. 2009; 108(1):105–13.
25.    Božić K, Glišić B, Radić-Tasić O, Knežević B. Case report of Mikulicz`s disease: A modern concept of an old entity. Vojnosanitetski pregled. 2023; 73(4):393–6.
26.    Karim F, Loeffen J, Bramer W, Westenberg L, Verdijk R, van Hagen M, et al. IgG4-related disease: a systematic review of this unrecognized disease in pediatrics. Pediatric Rheumatology. 2016; 14(1).
27.    Li S, Deng C, Chen J, Li F. Mikulicz's Disease With Immune Thrombocytopenia: A New Immunoglobulin G4-Mediated Disorder and Literature Review. Arch Rheumatol. 2018; 34(3): 343-347.
28.    Yamamoto M, Hashimoto M, Takahashi H, Shinomura Y. IgG4 Disease. Journal of Neuro-Ophthalmology. 2014; 34(4):393–9.
29.    Qu LM. IgG4-related autoimmune pancreatitis overlapping with Mikulicz’s disease and lymphadenitis: A case report. World Journal of Gastroenterology. 2013; 19(48):9490.
30.    Lin P, Medeiros LJ. Mikulicz Disease - an overview | Science Direct Topics. 2009.
31.    Zhang Y, Du Y, Li K, He J. IgG4-related Mikulicz’s disease associated with thyroiditis: a case report and review of the literature. Eye Science. 2014; 29(1):47–52.
32.    Takenaka K, Takada K, Kobayashi D, Moriguchi M, Harigai M, Miyasaka N. A case of IgG4-related disease with features of Mikulicz’s disease, and retroperitoneal fibrosis and lymphadenopathy mimicking Castleman’s disease. Modern Rheumatology. 2011; 21(4):410–4.
33.    Kim HJ, Kim JA, Min JK. Mikulicz’s Disease with Progressively Transformed Germinal Centers-type Immunoglobulin G4-related Lymphadenopathy Mimicking Sjögren’s Syndrome. Journal of Rheumatic Diseases. 2015; 22(6):395.
34.    Umehara H, Okazaki K, Kawa S, Takahashi H, Goto H, Matsui S, et al. The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD. Modern Rheumatology. 2021; 31(3):529–33.
35.    Wu Y, Xu ZR, Zhou WJ, Yang YM. Immunoglobulin G4-related Disease with Features of Mikulicz’s Disease and Autoimmune Pancreatitis Which Firstly Presented as Asymptomatic Lymphadenopathy: A Case Report. Chinese Medical Journal. 2015; 128(5):706.
36.    Yamamoto M, Takahashi H, Ohara M, Suzuki C, Naishiro Y, Yamamoto H, et al. A new conceptualization for Mikulicz’s disease as an IgG4-related plasmacytic disease. Modern Rheumatology. 2006; 16(6):335–40.
37.    Kudo-Tanaka E, Nakatsuka S, Hirano T, Kawai M, Katada Y, Matsushita M, et al. A case of Mikulicz’s disease with Th2-biased cytokine profile: possible feature discriminable from Sjögren’s syndrome. Modern Rheumatology. 2009 ; 19(6):691–5.
38.    Matsui S, Sugiyama E, Taki H, Kawagishi Y, Miwa T, Hayashi R, et al. [A case of bronchiolitis with IgG4-positive plasma cells penetration associated with Mikulicz’s disease and autoimmune pancreatitis]. Nihon Kokyuki Gakkai Zasshi = the Journal of the Japanese Respiratory Society. 2009; 47(2):139–44.
39.    Wu C, Zeng YP. Mikulicz Disease. JAMA Dermatology. 2020; 156(4):451.
40.    Miyake K, Moriyama M, Aizawa K, Nagano S, Inoue Y, Sadanaga A, et al. Peripheral CD4+ T cells showing a Th2 phenotype in a patient with Mikulicz’s disease associated with lymphadenopathy and pleural effusion. Modern Rheumatology. 2008; 18(1):86–90.
41.    An W, Wu Z, Li M, Yu H, Zhao X, Wang X, et al. Clinical characteristics and therapeutic response of immunoglobulin G4-related disease: a retrospective study of 127 Chinese patients. Orphanet Journal of Rare Diseases. 2022; 17(1).
42.    Takano K, Yamamoto M, Kondo A, Takahashi H, Himi T. A clinical study of olfactory dysfunction in patients with Mikulicz’s disease. Auris Nasus Larynx. 2011; 38(3):347–51.
43.    Mihas AA, Lawson PB, Dreiling BJ, Gurram VS, Heuman DM. Mikulicz Syndrome Associated With a Malignant Large Cell Gastric Lymphoma: A Case Report and Review of the Literature. International Journal of Gastrointestinal Cancer. 2003; 33(2-3): 123–8.
44.    Suga K, Kawakami Y, Hiyama A, Hori K, Takeuchi M. F-18 FDG PET-CT Findings in Mikulicz Disease and Systemic Involvement of IgG4-Related Lesions. Clinical Nuclear Medicine. 2009; 34(3): 164–7.
45.    Takagi T, Doi T, Sakamoto H, Tatsuta H, Kobayashi Y, Tsubota Y, et al. [A case report of autoimmune pancreatitis with Mikulicz’ s disease and diabetes mellitus]. Nihon Shokakibyo Gakkai Zasshi = The Japanese Journal of Gastro-Enterology. 2006; 103(2):180–8.
46.    Hiroto Nakata, Shushibasaki, Masato Nakajima, Hiroyoshi Yoshinami, Yasuhiro Kase. Three cases of IgG4-related Mikulicz disease. Bulletin of the Japanese Society of Otorhinolaryngology. 2010; 113(10):798–804.
47.    Zhou W, Li Y, Yan C, Zheng L, Liu F, Sun C. The prevalence of extra‐salivary gland complications in immunoglobulin G4‐related sialadenitis. Oral Diseases. 2021; 28(6):1468–83.
48.    Yamamoto M, Ohara M, Suzuki C, Naishiro Y, Yamamoto H, Takahashi H, et al. Elevated IgG4 concentrations in serum of patients with Mikulicz’s  disease. Scandinavian Journal of Rheumatology. 2004; 33(6):432–3.
49.    Kuruma S, Kamisawa T, Tabata T, Hara S, Fujiwara T, Kuwata G, et al. Clinical Characteristics of Patients with Autoimmune Pancreatitis with or without Mikulicz’s Disease and Mikulicz’s Disease Alone. 2013; 7(1):96–9.
50.    S S. Sjögren’s Syndrome. International Journal of Nursing Education and Research. 2020; 8(1):114–116.
51.    Babadjanova G. Peculiarities of Long-Term Glucocorticoid Therapy. Research Journal of Pharmacy and Technology. 2019; 12(9):4578–7582.
52.    Yadav R, Yadav N, Kharya MD. Steroid Chemistry and Steroid Hormone Action: A Review. Asian Journal of Research in Chemistry. 2014; 7(11):964–9.

Recomonded Articles:

Author(s): K.Vijaya Sri, Guda Santhoshini, D. Ravi Sankar, K.Niharika

DOI: 10.5958/2231-5659.2018.00005.X         Access: Open Access Read More

Author(s): Nikunja B. Pati, T. Swayam Jyothi, Gajender Singh Thakur, Sushma Reddy, G. Meghna

DOI: 10.5958/2231-5659.2017.00028.5         Access: Open Access Read More

Author(s): Sarika S. Lokhande

DOI: 10.5958/2231-5659.2019.00032.8         Access: Open Access Read More

Author(s): Dasari Nirmala, Enjamuri Aslesha , M. Sudhakar

DOI:         Access: Open Access Read More

Author(s): S.C. Shivhare, U.D.Shivhare, Preeti Srivastav, K.G. Malviya

DOI:         Access: Open Access Read More

Author(s): Dhanashri U. Gadhave, Puja S. Gaikwad, Nayana V. Pimpodkar, Swati B. Udugade

DOI: 10.5958/2231-5659.2015.00020.X         Access: Open Access Read More

Author(s): A.V.S. Hima Bindu, G. Bhavya, K. Padmalatha

DOI: 10.52711/2231-5659.2021.00046         Access: Open Access Read More

Author(s): A. A. Shinde, A. B. Velhal, P. D. Jadhav, V. K. Redasani

DOI: 10.52711/2231-5659.2022.00057         Access: Closed Access Read More

Author(s): Bhagyashri V. Aiwale, Bharatee P. Chaudhari, Atish B. Velhal, Vivekkumar K. Redasani

DOI: 10.52711/2231-5659.2022.00054         Access: Closed Access Read More

Author(s): Anamika Saxena, Gayatri Tiwari, Vikash Bhatt

DOI: 10.52711/2231-5659.2023.00026         Access: Closed Access Read More

Author(s): Harshada I Patil, Dhiraj Kamble

DOI: 10.52711/2231-5659.2022.00051         Access: Closed Access Read More

Author(s): Dhapake P. R, Baheti J. R, Suruse P.

DOI: 10.52711/2231-5659.2023.00025         Access: Closed Access Read More

Author(s): Venkata Triveni Devi Yerra, Stephanie Margaret Puvvada, Vinod Kumar Mugada, Srinivasa Rao Yarguntla

DOI: 10.52711/2231-5659.2023.00054         Access: Closed Access Read More

Author(s): Ghorpade Arti Changdev, S. S. Siddheshwar

DOI: 10.52711/2231-5659.2024.00009         Access: Closed Access Read More

Asian Journal of Research in Pharmaceutical Sciences (AJPSci) is an international, peer-reviewed journal, devoted to pharmaceutical sciences....... Read more >>>

RNI: Not Available                     
DOI: 10.52711/2231-5659 

Recent Articles