Soft Tissue Mass as Primary Manifestation of IgG4 Related Disease
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Abstract
IgG4- related disease is a systemic fibroinflammatory syndrome characterized by tumefactive enlargement of affected organ , diffuse lymphoplasma cells infiltration with a large number of IgG4 producing plasma cells and sclerotic changes. The most commonly involved organs are pancreas, lacrimal and salivary glands. Patients show good response to glucocorticoid therapy and despite its relapsing and remitting course, patients have good prognosis. We report an unusual presentation of IgG4- related disease manifested as isolated soft tissue mass of hand which is an extremely rare manifestation of this disorder.
1. Kim JH, Byun JH, Lee SS, Kim HJ, Lee MG. Atypical manifestations of IgG4- related sclerosing disease in the abdomen: imaging findings and pathologic correlations. Am J Roentgenol. 2013;200(1):102-112
2. Wang W, Kang X, Ding Y, Mao L, Dilinuer A, Li W. IgG4- related disease manifested as: cutaneous plasmacytosis: a case report. Clin Cosmet Investig Dermatol .2023;16:1997-2004.
3. Nasser R, Gilshtein H, Mansour S, Yasin K, Borzellina G, Khari S. Isolasted type immunoglobulin G4 sclerosing cholanigitis: the misdiagnosed cholangiocarcinoma. J Clin Med Res . 2021; 13(2): 75-81.
4. Brody G, Nguyen MO, Rojek NW, Lee BA. A unique case of IgG4- related skin disease and sclerosing cholangitis in a patient with previous hepatitis exposure . JAAD Case Rep. 2021; 15: 52-55.
5. Shenoy A, Mohandas N, Gottlieb A. Cutaneous and systemic IgG4- related disease in review for dermatologists. Dermatol Online J. 2019;25(6): 1-12.
6. Miyabe K, Zen Y, Cornell LD, Rajagopalan G, Chowdhary VR, Roberts LR, Chari ST. Reviews in basic and clinical gastroenterology and hepatology. Gastroenterology. 2018; 155: 990-1003.
7. Creze M, Boussebaa S, Lazure T, Biand S, Court C. IgG4- related disease: rare presentation as a soft- tissue mass in the thigh of an adolescent . Skeletal Radiol. 2020; 49:155-160.
8. Priori R, Lucchino B, Cerbelli B, Alessandri C, Battaro V, Zodda A, et al. An unusual manifestation of IgG4- related disease. Rheumatology. 2018;57:1305-1307.
9. Martinez DS, Oliva FL, Acenero MJF, Moyer EF, Cozar JLA. IgG 4- related disease presenting as a soft tissue tumor affecting skeletal muscle: a case report. lnt J Clin Med . 2013;4:1-4.
10. Katerji K, Smoller BR. Immunoglobulin- G4-related skin disease. Clin Dermatol. 2021;39(2):283-290.
11. Stone JH, Zen Y, Despende V. IgG4- related disease. N Engl J Med. 2012;366(6)539-551.
12. Chandraskharar R, Mathew V, Ashish G, Tyagi Ak, Jab AK. Isolated IgG4- related disease of sphenoid. sinus manifesting as blindness. Otorhinolaryngology clinics: Int J Otorhinolaryngol Clin. 2014;3:19-22.
13 Yousefi R, Amani D. IgG4 Characteristics and IgG4- related disease. Research in medicine .2018; 41(4):294-302.
14. Haider M, Haji F, Alalwan O, Aljufairi E, Shah TS. IgG4- related disease , the malignancy mimicker: case series from Bahrain. Case Rep Rheumatol. 2018;2018:1-9.
15. Abraham M, Khosroshahi A. Diagnostic and treatment workup for IgG4-related disease. Expert Rev clin immunol. 2017;13(9):867-875.
16. Katz G, Stone JH. Clinical perspective on IgG4-related disease and its classification . Annu Rev. Med 2022; 73:545-562.
17. Desponde V, Zen Y, Chan JKC. Consensus statement on the pathology of IgG4-related disease, Med Pathol. 2012;25:1181-1192.
2. Wang W, Kang X, Ding Y, Mao L, Dilinuer A, Li W. IgG4- related disease manifested as: cutaneous plasmacytosis: a case report. Clin Cosmet Investig Dermatol .2023;16:1997-2004.
3. Nasser R, Gilshtein H, Mansour S, Yasin K, Borzellina G, Khari S. Isolasted type immunoglobulin G4 sclerosing cholanigitis: the misdiagnosed cholangiocarcinoma. J Clin Med Res . 2021; 13(2): 75-81.
4. Brody G, Nguyen MO, Rojek NW, Lee BA. A unique case of IgG4- related skin disease and sclerosing cholangitis in a patient with previous hepatitis exposure . JAAD Case Rep. 2021; 15: 52-55.
5. Shenoy A, Mohandas N, Gottlieb A. Cutaneous and systemic IgG4- related disease in review for dermatologists. Dermatol Online J. 2019;25(6): 1-12.
6. Miyabe K, Zen Y, Cornell LD, Rajagopalan G, Chowdhary VR, Roberts LR, Chari ST. Reviews in basic and clinical gastroenterology and hepatology. Gastroenterology. 2018; 155: 990-1003.
7. Creze M, Boussebaa S, Lazure T, Biand S, Court C. IgG4- related disease: rare presentation as a soft- tissue mass in the thigh of an adolescent . Skeletal Radiol. 2020; 49:155-160.
8. Priori R, Lucchino B, Cerbelli B, Alessandri C, Battaro V, Zodda A, et al. An unusual manifestation of IgG4- related disease. Rheumatology. 2018;57:1305-1307.
9. Martinez DS, Oliva FL, Acenero MJF, Moyer EF, Cozar JLA. IgG 4- related disease presenting as a soft tissue tumor affecting skeletal muscle: a case report. lnt J Clin Med . 2013;4:1-4.
10. Katerji K, Smoller BR. Immunoglobulin- G4-related skin disease. Clin Dermatol. 2021;39(2):283-290.
11. Stone JH, Zen Y, Despende V. IgG4- related disease. N Engl J Med. 2012;366(6)539-551.
12. Chandraskharar R, Mathew V, Ashish G, Tyagi Ak, Jab AK. Isolated IgG4- related disease of sphenoid. sinus manifesting as blindness. Otorhinolaryngology clinics: Int J Otorhinolaryngol Clin. 2014;3:19-22.
13 Yousefi R, Amani D. IgG4 Characteristics and IgG4- related disease. Research in medicine .2018; 41(4):294-302.
14. Haider M, Haji F, Alalwan O, Aljufairi E, Shah TS. IgG4- related disease , the malignancy mimicker: case series from Bahrain. Case Rep Rheumatol. 2018;2018:1-9.
15. Abraham M, Khosroshahi A. Diagnostic and treatment workup for IgG4-related disease. Expert Rev clin immunol. 2017;13(9):867-875.
16. Katz G, Stone JH. Clinical perspective on IgG4-related disease and its classification . Annu Rev. Med 2022; 73:545-562.
17. Desponde V, Zen Y, Chan JKC. Consensus statement on the pathology of IgG4-related disease, Med Pathol. 2012;25:1181-1192.
| Files | ||
| Issue | Articles in Press: Vol 64 No 03 (2026) | |
| Section | Case Report(s) | |
| Keywords | ||
| IgG4-related disease Soft tissue Mass Unusual presentation Single organ | ||
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How to Cite
1.
Abbasi F, Abbasi A, Pashaei MR, Azimi K, Saedifar P, Nezafat A. Soft Tissue Mass as Primary Manifestation of IgG4 Related Disease. Acta Med Iran. 2026;.
