Pulmonary and coronary arterial abnormalities in patients with IgG4-related disease

Cochran, Rory L.; Brideau, Holly R.; Wu, Markus; Stone, John H.; Wallace, Zachary S; Little, Brent P.

doi:10.1016/j.radcr.2022.09.070

Cited by 4 publications

(4 citation statements)

References 19 publications

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“…Glucocorticoids are considered first-line-therapy that is also used for remission maintenance though the majority of patients experience disease relapse during or after glucocorticoid tapers. Therefore, B cell-depleting therapy such as rituximab or adalimumab may also be required [2] , [3] , [4] .…”

Section: Discussionmentioning

confidence: 99%

“…Its common manifestations include autoimmune pancreatitis (AIP), sclerosing cholangitis, sialadenitis, orbital region disease, retroperitoneal fibrosis, and chronic periaortitis. It most commonly affects middle-aged and older adults; however, it has also been reported in women and children [1] , [2] , [3] .…”

Section: Introductionmentioning

confidence: 99%

“…Cardiovascular system involvement is less common in these patients and is often revealed as an incidental finding on extensive diagnostic imaging [3] . In cases of involvement, it tends to affect medium- to large-sized arteries.…”

Section: Introductionmentioning

confidence: 99%

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Coronary artery involvement in a patient with IgG4-related disease

Mohammadzadeh

Houshmand

Pouraliakbar

et al. 2023

Radiology Case Reports

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Coronary artery involvement in a patient with IgG4-related disease

Mohammadzadeh

Houshmand

Pouraliakbar

et al. 2023

Radiology Case Reports

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“…The most common presentations of IgG4-RD include autoimmune pancreatitis, sclerosing cholangitis, sialadenitis, orbital region disease, retroperitoneal fibrosis, and chronic periaortitis [2]. Thoracic manifestations of IgG4-RD are less common and can present in the form of pulmonary involvement or involvement of mediastinal vasculature [3][4][5]. While pulmonary lesions can present as lung masses, groundglass opacities, interstitial involvement and peribronchial lesions, mediastinal vascular involvement can be in the form of arteritis or perivascular soft tissue, occasionally leading to vessel stenosis or aneurysm.…”

Section: Introductionmentioning

confidence: 99%

Imaging Findings in IGG4 Disease with Isolated Thoracic Involvement- A Case Report

Senapathy,

Gella,

Beevi

2023

CCR

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Immunoglobulin G4 -related disease (IgG4-RD) is a multisystemic fibro inflammatory disorder that can involve multiple organs and organ systems of the body. While autoimmune pancreatitis and retroperitoneal fibrosis are the most described forms of this entity, a few patients may present with thoracic involvement, either in isolation or more commonly, in association with abdominal and other systemic manifestations. Thoracic presentation of the disease can be in the form of lung parenchymal, interstitial, mediastinal vasculature and pleural involvement. In the mediastinal vasculature, thoracic aorta is the most commonly involved vessel; involvement of the pulmonary artery is less frequently described in literature. We present the imaging findings of an unusual case of IgG4-RD with isolated thoracic involvement in a middle-aged male patient who presented with shortness of breath.Contrast enhanced CT of the chest revealed a poorly marginated mediastinal mass causing encasement and narrowing of the superior vena cava, encasement and cut-off of the right pulmonary artery, with extension of the soft tissue along the right peribronchial interstitium.A solid nodule and mild interstitial changes were also seen in the right lung as part of the disease spectrum.

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Solitary recurrence of IgG4-related giant coronary aneurysm: Case report and review of the literature focusing on treatment strategies and complications

Kawahara,

Mizushima,

Matsumoto

et al. 2023

Modern Rheumatology Case Reports

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Coronary periarteritis is a dangerous manifestation of IgG4-related disease, because it forms coronary artery aneurysms, which may cause sudden cardiac death. We report the case of a 78-year-old woman with IgG4-related coronary periarteritis and a coronary aneurysm, which showed progressive enlargement despite maintenance therapy for Type 1 autoimmune pancreatitis. This case was unique, in that coronary periarteritis was the only active lesion that recurred. Low-dose glucocorticoids suppressed the progression of periarterial lesions but led to rapid thinning of the aneurysmal wall and an increase in the size of mural thrombi, which pose a risk of myocardial infarction. Our systematic literature review including 98 cases of 86 articles was performed to examine its treatment strategies and complications. Among the cases in which the effect of immunosuppressive therapy could be followed radiologically, 33 of 37 (89.1%) cases showed improvement in wall thickening/periarterial soft tissue, while 6 of 13 (46.2%) showed worsening increase in the outer diameter of the coronary aneurysms. We propose a draft treatment algorithm and suggest that immunosuppressive therapy for IgG4-related coronary periarteritis with coronary aneurysms should be conducted only after the therapeutic benefit has been determined to outweigh the risks. Because coronary periarteritis can occur without other organ involvement, as in our case, all cases of IgG4-related disease require careful monitoring of coronary artery lesions.

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Pulmonary and coronary arterial abnormalities in patients with IgG4-related disease

Cited by 4 publications

References 19 publications

Coronary artery involvement in a patient with IgG4-related disease

Coronary artery involvement in a patient with IgG4-related disease

Imaging Findings in IGG4 Disease with Isolated Thoracic Involvement- A Case Report

Solitary recurrence of IgG4-related giant coronary aneurysm: Case report and review of the literature focusing on treatment strategies and complications

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