Abstract
BackgroundLarge vessels could be involved in immunoglobulin (Ig)-G4-related disease (IgG4-RD). This study aimed to clarify the clinical features and evaluate the treatment efficacy for IgG4-RD with aortitis/periaortitis and periarteritis (PAO/PA).MethodsThis study prospectively enrolled 587 patients with IgG4-RD with a follow-up time of more than 6 months. The distribution of IgG4-related PAO/PA was classified into four types: type 1, thoracic aorta; type 2a, abdominal aorta; type 2b, abdominal aorta and iliac artery; type 2c, iliac artery; type 3, thoracic and abdominal aorta; and type 4, other arteries. Patient’s demographic data, clinical characteristics, laboratory parameters, and treatment efficacy were analyzed.ResultsOf 587 IgG4-RD patients, 89 (15.2%) had PAO/PA. The average age was 58.3 ± 11.1 years, with male predominance (85.4%). Vessels affected were as follows: abdominal aorta (83.1%), iliac artery (70.8%), thoracic aorta (13.5%), and other vessels (13.5%). The most prevalent distribution type of IgG4-related PAO/PA was type 2b, with 74 (83.1%) patients, followed by type 2a, type 2c, type 3, and type 1. Fifty-five (61.8%) PAO/PA patients had hydronephrosis, with renal insufficiency occurring in 43 (48.3%), and 31 (34.8%) PAO/PA patients had D-J stent drainage due to severe ureteral obstruction. After treatment with a glucocorticoid and immunosuppressants, 82% patients achieved remission with shrinking of the perivascular mass by more than 30%.ConclusionsIgG4-RD with PAO/PA was distinct from non-PAO/PA in demographic features, organ involvement distribution, inflammatory markers, and serum IgG4 and IgE. The most common affected vessel was the abdominal aorta, and most patients responded well with treatment.
Highlights
Large vessels could be involved in immunoglobulin (Ig)-G4-related disease (IgG4-RD)
Evidence suggested that Immunoglobulin G4-related disease (IgG4-RD) could affect various organs including the vascular system as aortitis/periaortitis/periarteritis (PAO/Aortitis/periaortitis and periarteritis (PA)) [9,10,11,12]; the most frequently involved blood vessel is the aorta, and a medium-sized artery, such as the iliac artery and carotid artery, may be a potential target [5]
Demographic characteristics of IgG4-RD with PAO/PA Among 587 IgG4-RD patients, 89 patients (76 men and 13 women) were included in the IgG4-related PAO/PA group and 498 patients were included in the non-PAO/ PA group
Summary
Large vessels could be involved in immunoglobulin (Ig)-G4-related disease (IgG4-RD). This study aimed to clarify the clinical features and evaluate the treatment efficacy for IgG4-RD with aortitis/periaortitis and periarteritis (PAO/PA). Immunoglobulin (Ig)-G4-related disease (IgG4-RD) is a fibro-inflammatory condition characterized by tumorlike swelling of affected organs, with elevated serum IgG4 and massive infiltration of lymphocytes and plasma cells in involved organs [1,2,3,4,5]. It is a highly heterogeneous disease entity that could affect nearly any organ and often present with multiorgan involvement [6], and the most affected tissues are the lacrimal gland, submandibular gland, lymph node, and pancreas [6,7,8]. To further understand and achieve better management of IgG4-related PAO/ PA, we aimed to analyze the clinical patterns of IgG4related PAO/PA, distribution of affected vessels, and treatment efficacy
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