IgG4-related periaortitis/periarteritis: An under-recognized condition that is potentially life-threatening

Mizushima, Ichiro; Kasashima, Satomi; Fujinaga, Yasunari; Kawano, Mitsuhiro; Ishizaka, Nobukazu

doi:10.1080/14397595.2018.1546367

Cited by 38 publications

(52 citation statements)

References 115 publications

(117 reference statements)

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“…The onset symptoms of PAO/PA patients were non-specific, including pain (often back or abdominal pain, as chest or groin pain was relatively rare seen), edema of the lower limbs, and dyspnea [27]. In our cohort, the most common onset symptoms were back pain and abdominal pain, followed by lymph node swelling, submandibular gland enlargement, and lower limb edema in most patients with multiorgan involvement.…”

Section: Discussionmentioning

confidence: 66%

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

Zhang

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.

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Section: Discussionmentioning

confidence: 66%

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

Zhang

Liu

et al. 2020

Preprint

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“…The pancreas, and the salivary and lacrimal glands are the main involved organs 2 . However, many other organ systems may be synchronously or metachronously involved including the cardiovascular system 3 . Herein, we describe a case of IgG4‐RD with biopsy‐proven cardiac and retroperitoneal involvement with possible involvement of the pituitary gland.…”

Section: Introductionmentioning

confidence: 95%

A challenging case report of IgG4‐related systemic disease involving the heart and retroperitoneum with a literature review of similar heart lesions

et al. 2020

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The IgG4‐related disease is a distinct, steroid‐responsive fibro‐inflammatory disorder of unknown etiology. This multiorgan disease is characterized by tumefactive lesions that contain rich infiltrations of IgG4‐positive plasma cells, with the pancreas, and the salivary and lacrimal glands being the main involved. The more common cardiovascular involvements include inflammatory peri‐aortitis, coronary arteritis, and pericarditis. Intra‐cardiac tumefactive lesions are rarely reported. Herein, we describe a challenging case of IgG4‐related disease with a long‐time lag between initiation of symptoms to proper diagnosis with biopsy‐proven cardiac and retroperitoneal and possible pituitary gland involvement. Concerning the rarity of the cardiac lesion in our case, we conducted a literature review of similar case reports.

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“…The onset symptoms of PAO/PA patients were non-specific, including pain (often back or abdominal pain, chest or groin pain was relatively rare seen), edema of lower limbs, and dyspnea [26]. In our cohort, the most common onset symptoms were back pain and abdominal pain, followed by lymph node swelling, submandibular gland enlargement, and lower limb edema in most patients with multiorgan involvement.…”

Section: Discussionmentioning

confidence: 64%

“…Aortitis needs to be treated urgently because inflammatory aortic aneurysms may have a large diameter or high enlargement rate and are at a high risk of rupture. Patients with chronic periaortitis were often treated with medium to high dose GCs [14,26]. Immunosuppressants are steroid-sparing treatments for PAO/PA patients.…”

Section: Discussionmentioning

confidence: 99%

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

Zhang

Liu

et al. 2020

Preprint

View full text Add to dashboard 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.

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IgG4-related periaortitis/periarteritis: An under-recognized condition that is potentially life-threatening

Cited by 38 publications

References 115 publications

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

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

A challenging case report of IgG4‐related systemic disease involving the heart and retroperitoneum with a literature review of similar heart lesions

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

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