Immunoglobulin G4-Related Lesions in Autoimmune Diseases: Unusual Presentations at Atypical Sites-A Tale of 2 Cases with Literature Review

被引:1
作者
Baisya, Ritasman [1 ]
Yerram, Keerthi Vardhan [1 ]
Baby, Arun [1 ]
Devarasetti, Phani Kumar [1 ]
Rajasekhar, Liza [1 ]
机构
[1] Nizams Inst Med Sci, Clin Immunol & Rheumatol, Hyderabad, India
关键词
IgG4-RD; cardiac IgG4-RD; esophageal IgG4-RD; RA; primary APS; IGG4-RELATED DISEASE; IGG4; AUTOANTIBODIES; ESOPHAGITIS; CELLS; MASS;
D O I
10.5152/eurjrheum.2023.23052
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Immunoglobulin G4-related disease (IgG4-RD) coexisting with clinically apparent autoimmune diseases, such as rheumatoid arthritis (RA) or antiphospholipid syndrome (APS), is a rarely documented combination in the scientific literature. In this case-based review, we present 2 intriguing cases with preexisting autoimmune diseases, namely, RA and primary APS, who exhibited coexistent IgG4related lesions at unusual sites. The first case pertains to a patient with known RA who presented with an encasing mass in the esophagus leading to stricture, with histopathological diagnosis of IgG4-RD.The second patient, diagnosed with primary APS, experienced breathlessness, and imaging revealed a right atrial mass. Histopathological examination of the mass confirmed IgG4-RD. Notably, both patients demonstrated significant clinical improvement upon initiation of steroid therapy. Rheumatoid arthritis patients commonly exhibit elevated levels of IgG4 in their sera; however, RA with coexisting IgG4-RD is rarely reported in the literature. Similarly, APS with IgG4-related lesions is exceedingly rare. Although there are few case reports and series on esophageal and cardiac IgG4-RD, the occurrence of such unusual location of IgG4-related lesions in the context of known autoimmunity is presented here for the first time.
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页数:64
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