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IgG4-related aortitis/periaortitis and periarteritis: a distinct spectrum of IgG4-related disease
Linyi Peng1, Panpan Zhang1, Jieqiong Li1
1Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.
Insights
Immunoglobulin (Ig) G4-related disease (IgG4-RD) can affect large vessels, particularly the abdominal aorta. Treatment with glucocorticoids and immunosuppressants effectively manages IgG4-RD with aortitis/periaortitis and periarteritis (PAO/PA).
Area of Science:
- Vascular Medicine
- Rheumatology
- Immunology
Background:
- Immunoglobulin (Ig) G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition.
- Large vessel involvement, specifically aortitis/periaortitis and periarteritis (PAO/PA), is a recognized but less studied manifestation of IgG4-RD.
Purpose of the Study:
- To elucidate the clinical characteristics of IgG4-RD patients with PAO/PA.
- To assess the therapeutic efficacy of standard treatments for IgG4-RD involving large vessels.
Main Methods:
- Prospective enrollment of 587 IgG4-RD patients with over six months of follow-up.
- Classification of IgG4-related PAO/PA into four distribution types.
- Analysis of demographic data, clinical features, laboratory parameters, and treatment outcomes.
Main Results:
- 15.2% (89/587) of IgG4-RD patients presented with PAO/PA, predominantly affecting the abdominal aorta (83.1%).
- Common complications included hydronephrosis (61.8%) and renal insufficiency (48.3%), often requiring ureteral stenting.
- Treatment with glucocorticoids and immunosuppressants led to remission in 82% of patients, with significant reduction in perivascular mass.
Conclusions:
- IgG4-RD with PAO/PA exhibits distinct demographic and clinical features compared to non-PAO/PA IgG4-RD.
- The abdominal aorta is the most frequently affected large vessel in this cohort.
- Effective management of IgG4-RD with PAO/PA is achievable with current therapeutic strategies.
Background:
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).
Methods:
This 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.
Results:
Of 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%.
Conclusions:
IgG4-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.
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