Differences and similarities between IgG4-related disease with and without dacryoadenitis and sialoadenitis: clinical manifestations and treatment efficacy

Liu

et al. 2020

Preprint

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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; 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.

Section: Discussionsupporting

confidence: 91%

Section: Introductionmentioning

confidence: 99%

IgG4-related aortitis/periaortitis and periarteritis: a distinct spectrum of IgG4-related disease

Liu

et al. 2020

Preprint

Self Cite

“…Of PAO/PA patients, the most prevalent type of vessel distribution was type 2, consistent with Ozawa et al study [5,25]. However, PAO/PA patients had much lower rate of DS and paranasal sinusitis compared with non-PAO/PA patients, which is consistent with our previous study that patients without DS had a higher percentage of aorta or larger blood vessel involvement [7].…”

Section: Discussionsupporting

confidence: 91%

“…According to allergy, a substantial percentage of IgG4-RD patients had histories of allergy [8], the percentage of allergy was higher in patients with IgG4-related dacryoadenitis/sialadenitis (DS) than those with non-IgG4 DS [7], and non-PAO/PA patients also had higher percentage of allergy than PAO/PA patients [5]. The demographic features of our PAO/PA patients were consistent with those in other studies [5,24,25].…”

Section: Discussionsupporting

confidence: 86%

“…IgG4-related disease (IgG4-RD) is a fibro-inflammatory condition characterized by tumor-like 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 which 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]. Evidence suggested that IgG4-RD could affect various organs including the vascular system as aortitis/periaortitis/periarteritis (PAO/PA) [9][10][11][12], and the most frequently involved blood vessel is the aorta, and medium-sized artery, such as the iliac artery and carotid artery, may be a potential target [5].…”

Section: Introductionmentioning

confidence: 99%

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IgG4-related aortitis/periaortitis and periarteritis: a distinct spectrum of IgG4-related disease

Liu

et al. 2020

Preprint

Self Cite

Background: Large vessels could be involved in IgG4-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 enrolled 587 IgG4-RD patients in a prospective cohort with a follow-up time for 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; 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. 55 (61.8%) PAO/PA patients had hydronephrosis, with renal insufficiency occurred in 43 (48.3%), and 31 (34.8%) PAO/PA patients had D-J stent drainage due to severe ureteral obstruction. After treatment with glucocorticoid and immunosuppressants, 82% patients achieved a remission with shrinking of perivascular mass. Conclusions: IgG4-RD with PAO/PA was distinct from non-PAO/PA in demographic features, organs involvement distribution, inflammatory markers, serum IgG4 and IgE. The most common affected vessel was abdominal aorta, and most patients responded well with treatment.

Lifetime Allergy Symptoms in IgG4‐Related Disease: A Case–Control Study

Sanders

Arthritis Care & Research

et al. 2022

Objective. The etiology of IgG4-related disease (IgG4-RD) is unknown, and there has been controversy over the significance of allergic conditions in IgG4-RD. We examined the prevalence of lifetime allergy symptoms in IgG4-RD and the association between these and IgG4-RD.Methods. We identified IgG4-RD patients and non-IgG4-RD controls without autoimmune conditions seen at a single center. IgG4-RD patients were classified using the American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria. Allergy symptoms were ascertained by questionnaire. We assessed the association of IgG4-RD features with allergy symptoms. We compared the proportion of cases and controls with allergy symptoms using conditional logistic regression to estimate odds ratios (ORs) and 95% confidence intervals (95% CIs) after matching cases and controls 1:1 by age and sex.Results. Lifetime allergy symptoms were reported by 165 (71%) of 231 IgG4-RD patients. Aeroallergen symptoms were most commonly reported (n = 135, 58%), followed by skin allergy symptoms (n = 97, 42%) and food allergy symptoms (n = 47, 20%). IgG4-RD cases with a history of allergy symptoms were more likely to have head and neck involvement (OR 2.0 [95% CI 1.1-3.6]) and peripheral eosinophilia (OR 3.3 [95% CI 1.2-9.0]) than those without allergy symptoms. The prevalence of any allergy symptoms was similar between cases and controls (OR 0.7 [95% CI 0.4-1.1]); this remained consistent after stratifying by head and neck involvement.Conclusion. Lifetime allergy symptoms are common in IgG4-RD but are not reported more often in IgG4-RD compared to non-IgG4-RD patients without autoimmune conditions. These findings suggest that allergies are not uniquely associated with the pathogenesis or presentation of IgG4-RD.