Serum Immunoglobulin G4 Level in 32 Patients with Active-phase Pemphigus Vulgaris_West China Medical Journal

Authors：

 QingYong ^1,2 , WangLian ¹ , XiaDengmei ¹ , ZhangRan ¹ , XuFei ¹ , YanWei ¹ ,  LiWei ¹

1. Department of Dermatology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;
2. Department of Anorectal Dermatology, Chengdu Anorectal Hospital, Chengdu, Sichuan 610017, P. R. China;

Corresponding author：

LiWei, Email: 724564806@qq.com

Keywords：

Pemphigus vulgaris; Immunoglobulin G4; Immuno-scatter turbidmetry

DOI：

10.7507/1002-0179.201600500

Video：

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Objective To learn the serum immunoglobulin G4 (IgG4) level in patients with pemphigus vulgaris (PV) in the active phase by detecting the IgG4 concentration in the patients with PV. Methods We collected the serum samples from patients with active-phase PV (including acute-onset and chronic active phases) between April and December 2014. The serum IgG4 level was detected by immuno-scatter turbidmetry, and the difference of serum IgG4 level between PV patients and normal subjects was assessed by Student's t-test. Results Thirty-two patients with pemphigus vulgaris in the active phase were enrolled for this study, among whom 22 (including 8 males and 14 females) had normal serum IgG4 level, 8 (including 3 males and 5 females) higher and 2 (including 1 male and 1 female) lower. Compared with normal subjects, the average level of serum IgG4 in PV patients was not significantly different (P>0.05). The serum IgG4 level of acute-onset and chronic active PV patients was (1.015±0.825) g/L and (1.058±1.133) g/L, respectively, with no significant difference (P>0.05). Conclusion The serum IgG4 level of patients with pemphigus vulgaris in the active phase can be normal, higher or lower. And there is no obvious difference in the serum IgG4 level between acute-onset and chronic active PV patients.

Citation： QingYong, WangLian, XiaDengmei, ZhangRan, XuFei, YanWei, LiWei. Serum Immunoglobulin G4 Level in 32 Patients with Active-phase Pemphigus Vulgaris. West China Medical Journal, 2016, 31(11): 1822-1825. doi: 10.7507/1002-0179.201600500 Copy

1.	Ruocco V, Ruocco E, Lo Schiavo A, et al. Pemphigus:etiology, pathogenesis, and inducing or triggering factors:facts and controversies[J].Clin Dermatol, 2013, 31(4):374-381.
2.	Spaeth S, Riechers R, Borradori L, et al. IgG, IgA and IgE autoantibodies against the ectodomain of desmoglein 3 in active pemphigus vulgaris[J].Br J Dermatol, 2001, 144(6):1183-1188.
3.	Stone JH, Zen Y, Deshpande V. IgG4-related disease[J].N Engl J Med, 2012, 366(6):539-551.
4.	Ayatollahi M, Joubeh S, Mortazavi H, et al. IgG(4) as the predominant autoantibody in sera from patients with active state of pemphigus vulgaris[J].J Eur Acad Dermatol and Venereol, 2004, 18(2):241-242.
5.	林玮, 张文. IgG4相关性疾病[J].中华临床免疫和变态反应杂志, 2010, 4(4):307-311.
6.	Khosroshahi A, Bloch DB, Deshpande V, et al. Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related systemic disease[J].Arthritis Rheum, 2010, 62(6):1755-1762.
7.	黄晓春. 美罗华的临床应用进展[J].中国临床新医学, 2012, 5(7):659-663.
8.	Vidarsson G, Dekkers G, Rispens T. IgG subclasses and allotypes:from structure to effector functions[J].Front Immunol, 2014, 5(520):1-17.
9.	Nirula A, Glaser SM, Kalled SL, et al. What is IgG4? A review of the biology of a unique immunoglobulin subtype[J].Curr Opin Rheumatol, 2011, 23(1):119-124.
10.	Funakoshi T, Lunardon L, Ellebrecht CT, et al. Enrichment of total serum IgG4 in patients with pemphigus[J].Br J Dermatol, 2012, 167(6):1245-1253.
11.	Abé T, Maruyama S, Babkair H, et al. Simultaneous immunolocalization of desmoglein 3 and IgG4 in oral pemphigus vulgaris:IgG4predominant autoantibodies in its pathogenesis[J].J Oral Pathol Med, 2015, 44(10):850-856.
12.	Van DW, Stanic B, Yaman G, et al. IgG4 production is confined to human IL-10-producing regulatory B cells that suppress antigen-specific immune responses[J].J Allergy Clin Immunol, 2013, 131(4):1204-1212.
13.	Giordano CN, Sinha AA. Cytokine networks in pemphigus vulgaris:an integrated viewpoint[J].Autoimmunity, 2012, 45(6):427-439.
14.	万芙容, 程丽英, 索翠萍. IgG亚型检测与疾病的相关研究[J].医学检验与临床, 2011, 26(6):55-56.
15.	Futei Y, Amagai M, Ishii K, et al. Predominant IgG4 subclass in autoantibodies of pemphigus vulgaris and foliaceus[J].J Dermatol Sci, 2001, 26(1):55-61.
16.	Green MG, Bystryn JC. Effect of intravenous immunoglobulin therapy on serum levels of IgG1 and IgG4 antidesmoglein 1 and antidesmoglein 3 antibodies in pemphigus vulgaris[J].Arch Dermatol, 2008, 144(12):1621-1624.

1. Ruocco V, Ruocco E, Lo Schiavo A, et al. Pemphigus:etiology, pathogenesis, and inducing or triggering factors:facts and controversies[J].Clin Dermatol, 2013, 31(4):374-381.
2. Spaeth S, Riechers R, Borradori L, et al. IgG, IgA and IgE autoantibodies against the ectodomain of desmoglein 3 in active pemphigus vulgaris[J].Br J Dermatol, 2001, 144(6):1183-1188.
3. Stone JH, Zen Y, Deshpande V. IgG4-related disease[J].N Engl J Med, 2012, 366(6):539-551.
4. Ayatollahi M, Joubeh S, Mortazavi H, et al. IgG(4) as the predominant autoantibody in sera from patients with active state of pemphigus vulgaris[J].J Eur Acad Dermatol and Venereol, 2004, 18(2):241-242.
5. 林玮, 张文. IgG4相关性疾病[J].中华临床免疫和变态反应杂志, 2010, 4(4):307-311.
6. Khosroshahi A, Bloch DB, Deshpande V, et al. Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related systemic disease[J].Arthritis Rheum, 2010, 62(6):1755-1762.
7. 黄晓春. 美罗华的临床应用进展[J].中国临床新医学, 2012, 5(7):659-663.
8. Vidarsson G, Dekkers G, Rispens T. IgG subclasses and allotypes:from structure to effector functions[J].Front Immunol, 2014, 5(520):1-17.
9. Nirula A, Glaser SM, Kalled SL, et al. What is IgG4? A review of the biology of a unique immunoglobulin subtype[J].Curr Opin Rheumatol, 2011, 23(1):119-124.
10. Funakoshi T, Lunardon L, Ellebrecht CT, et al. Enrichment of total serum IgG4 in patients with pemphigus[J].Br J Dermatol, 2012, 167(6):1245-1253.
11. Abé T, Maruyama S, Babkair H, et al. Simultaneous immunolocalization of desmoglein 3 and IgG4 in oral pemphigus vulgaris:IgG4predominant autoantibodies in its pathogenesis[J].J Oral Pathol Med, 2015, 44(10):850-856.
12. Van DW, Stanic B, Yaman G, et al. IgG4 production is confined to human IL-10-producing regulatory B cells that suppress antigen-specific immune responses[J].J Allergy Clin Immunol, 2013, 131(4):1204-1212.
13. Giordano CN, Sinha AA. Cytokine networks in pemphigus vulgaris:an integrated viewpoint[J].Autoimmunity, 2012, 45(6):427-439.
14. 万芙容, 程丽英, 索翠萍. IgG亚型检测与疾病的相关研究[J].医学检验与临床, 2011, 26(6):55-56.
15. Futei Y, Amagai M, Ishii K, et al. Predominant IgG4 subclass in autoantibodies of pemphigus vulgaris and foliaceus[J].J Dermatol Sci, 2001, 26(1):55-61.
16. Green MG, Bystryn JC. Effect of intravenous immunoglobulin therapy on serum levels of IgG1 and IgG4 antidesmoglein 1 and antidesmoglein 3 antibodies in pemphigus vulgaris[J].Arch Dermatol, 2008, 144(12):1621-1624.

West China Medical Journal

Serum Immunoglobulin G4 Level in 32 Patients with Active-phase Pemphigus Vulgaris

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