scholarly journals Immunoglobulin G4-related masses surrounding coronary arteries: a case report

2021 ◽  
Vol 5 (3) ◽  
Author(s):  
Takuya Nakamura ◽  
Yutaka Goryo ◽  
Takuya Isojima ◽  
Hiroyuki Kawata

Abstract Background Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition with high serum IgG4 levels affecting various organs, such as the pancreas, lacrimal and salivary glands, thyroid, kidney, and lung. Typical cardiovascular manifestations of IgG4-RD include periaortitis, coronary arteritis, and pericarditis. However, reports of IgG4-RD associated with coronary arteritis are rare. Here, we report a case of IgG4-related masses surrounding the coronary arteries. Case summary A 59-year-old man was referred to our hospital because of mediastinal masses detected by computed tomography (CT). Coronary CT angiography revealed masses surrounding the right coronary artery and the left anterior descending coronary artery. An elevated serum level of IgG4 and histological findings led to the diagnosis of IgG4-related coronary arteritis with mass formation. Coronary angiography showed numerous feeding arteries to the masses, which were demonstrated as multiple microchannels in the intravascular ultrasound (IVUS) images. Discussion IgG4-RD involving the cardiovascular system has been reported. However, coronary artery disease associated with IgG4-RD is very rare, and the mechanism of mass formation in IgG4-related coronary arteritis is unclear. In our case, within the cardiovascular system, IgG4-RD was limited to the coronary arteries, suggesting that the affected coronary arteries may provide the necessary blood supply to the mass, thus, aiding its growth. These findings were supported by the images from coronary angiography and IVUS.

2005 ◽  
Vol 8 (1) ◽  
pp. 42 ◽  
Author(s):  
C. Probst ◽  
A. Kovacs ◽  
C. Schmitz ◽  
W. Schiller ◽  
H. Schild ◽  
...  

Objective: Invasive, selective coronary angiography is the gold standard for evaluation of coronary artery disease (CAD) and degree of stenosis. The purpose of this study was to compare 3-dimensional (3D) reconstructed 16-slice multislice computed tomographic (MSCT) angiography and selective coronary angiography in patients before elective coronary artery bypass graft (CABG) procedure. Methods: Sixteen-slice MSCT scans (Philips Mx8000 IDT) were performed in 50 patients (42 male/8 female; mean age, 64.44 8.66 years) scheduled for elective CABG procedure. Scans were retrospectively electrocardiogram-gated 3D reconstructed. The images of the coronary arteries were evaluated for stenosis by 2 independent radiologists. The results were compared with the coronary angiography findings using the American Heart Association segmental classification for coronary arteries. Results: Four patients (8%) were excluded for technical reasons. Thirty-eight patients (82.6%) had 3-vessel disease, 4 (8.7 %) had 2-vessel disease, and 4 (8.7%) had an isolated left anterior descending artery stenosis. In the proximal segments all stenoses >50% (56/56) were detected by MSCT; medial segment sensitivity was 97% (73/75), specificity 90.3%; distal segment sensitivity was 90.7% (59/65), specificity 77%. Conclusion: Accurate quantification of coronary stenosis greater than 50% in the proximal and medial segments is possible with high sensitivity and specificity using the new generation of 16-slice MSCTs. There is still a tendency to overestimate stenosis in the distal segments. MSCT seems to be an excellent diagnostic tool for screening patients with possible CAD.


2020 ◽  
Vol 9 (8) ◽  
pp. 2425
Author(s):  
Wei-Hsin Yuan ◽  
Anna Fen-Yau Li ◽  
Shu-Yi Yu ◽  
Ying-Yuan Chen ◽  
Chia-Hung Wu ◽  
...  

Background: Benign immunoglobulin G4 (IgG4)-related orbital disease (IgG4-ROD)—characterized as tumors mimicking malignant orbital lymphoma (OL)—responds well to steroids, instead of chemotherapy, radiotherapy and/or surgery of OL. The objective of this study was to report the differences in computed tomography (CT) features and- serum IgG4 levels of IgG4-ROD and OL. Methods: This study retrieved records for patients with OL and IgG4-ROD from a pathology database during an eight-year-and-five-month period. We assessed the differences between 16 OL patients with 27 lesions and nine IgG4-ROD patients with 20 lesions according to prebiopsy CT features of lesions and prebiopsy serum IgG4 levels and immunoglobulin G (IgG) levels This study also established the receiver-operating curves (ROC) of precontrast and postcontrast CT Hounsfield unit scales (CTHU), serum IgG4 levels, serum IgG levels and their ratios. Results: Significantly related to IgG4-ROD (all p < 0.05) were the presence of lesions with regular borders, presence of multiple lesions—involving both lacrimal glands on CT scans—higher median values of postcontrast CTHU, postcontrast CTHU/precontrast CTHU ratios, serum IgG4 levels and serum IgG4/IgG level ratios. Compared to postcontrast CTHU, serum IgG4 levels had a larger area under the ROC curve (0.847 [95% confidence interval (CI): 0.674–1.000, p = 0.005] vs. 0.766 [95% CI: 0.615–0.917, p = 0.002]), higher sensitivity (0.889 [95% CI: 0.518–0.997] vs. 0.75 [95% CI: 0.509–0.913]), higher specificity (0.813 [95% CI: 0.544–0.960] vs. 0.778 [95% CI: 0.578–0.914]) and a higher cutoff value (≥132.5 mg/dL [milligrams per deciliter] vs. ≥89.5). Conclusions: IgG4-ROD showed distinct CT features and elevated serum IgG4 (≥132.5 mg/dL), which could help distinguish IgG4-ROD from OL.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yongcheol Kim ◽  
Jonathan James Hyett Bray ◽  
Benjamin Waterhouse ◽  
Alexander Gall ◽  
Georgia May Connolly ◽  
...  

AbstractNon-atherosclerotic abnormalities of vessel calibre, aneurysm and ectasia, are challenging to quantify and are often overlooked in qualitative reporting. Utilising a novel 3-dimensional (3D) quantitative coronary angiography (QCA) application, we have evaluated the characteristics of normal, diabetic and aneurysmal or ectatic coronary arteries. We selected 131 individuals under 50 years-of-age, who had undergone coronary angiography for suspected myocardial ischaemia between 1st January 2011 and 31st December 2015, at the Bristol Heart Institute, Bristol, UK. This included 42 patients with angiographically normal coronary arteries, 36 diabetic patients with unobstructed coronaries, and 53 patients with abnormal coronary dilatation (aneurysm and ectasia). A total of 1105 coronary segments were analysed using QAngio XA 3D (Research Edition, Medis medical imaging systems, Leiden, The Netherlands). The combined volume of the major coronary arteries was significantly different between each group (1240 ± 476 mm3 diabetic group, 1646 ± 391 mm3 normal group, and 2072 ± 687 mm3 abnormal group). Moreover, the combined coronary artery volumes correlated with patient body surface area (r = 0.483, p < 0.01). Inter-observer variability was assessed and intraclass correlation coefficient of the total coronary artery volume demonstrated a low variability of 3D QCA (r = 0.996, p < 0.001). Dedicated 3D QCA facilitates reproducible coronary artery volume estimation and allows discrimination of normal and diseased vessels.


2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Murat Yuksel ◽  
Abdulkadir Yildiz ◽  
Mustafa Oylumlu ◽  
Nihat Polat ◽  
Halit Acet ◽  
...  

Coronary cameral fistulas are abnormal communications between a coronary artery and a heart chamber or a great vessel which are reported in less than 0.1% of patients undergoing diagnostic coronary angiography. All three major coronary arteries are even less frequently involved in fistula formation as it is the case in our patient. A 68-year-old woman was admitted to cardiology clinic with complaints of exertional dyspnea and angina for two years and a new onset palpitation. Standard 12-lead electrocardiogram revealed atrial fibrillation (AF) with a ventricular rate of 114 beat/minute and accompanying T wave abnormalities and minimal ST-depression on lateral derivations. Transthoracic echocardiographic examination was normal except for diastolic dysfunction, minimally mitral regurgitation, and mild to moderate enlargement of the left atrium. Sinus rhythm was achieved by medical cardioversion with amiodarone infusion. Coronary angiography revealed diffuse and multiple coronary-left ventricle fistulas originating from the distal segments of both left and right coronary arterial systems without any stenosis in epicardial coronary arteries. The patient’s symptoms resolved almost completely with medical therapy. High volume shunts via coronary artery to left ventricular microfistulas may lead to increased volume overload and subsequent increase in end-diastolic pressure of the left ventricle and may cause left atrial enlargement.


Author(s):  
Jagadeesh Chandrasekaran ◽  
Phani Krishna Machiraju

Immunoglobulin G4 (IgG4)-related disease is a multi-organ, immune-mediated, fibro-inflammatory disorder characterized by tumefactive masses in the affected organs. Incidence and prevalence of IgG4-related disease (RD) are not clearly known and have slight male preponderance. It often involves multiple organs at the time of presentation or over the course of disease mimicking malignancy, Sjogren's syndrome, antineutrophil cytoplasmic antibodies associated vasculitis, infections. A thorough workup is needed to rule out these mimickers. A 33-year-old gentleman presented to us with history of progressive swelling in the right peri-orbital region for four years. On evaluation, abdominal imaging was notable for the sausage-shaped pancreas and hypoenchancing nodules in bilateral kidneys. Histological examination of right lacrimal gland revealed lymphoplasmacytic infiltrate and storiform fibrosis. Serum IgG4 levels were normal, and immunostaining was negative. A diagnosis of IgG4-RD was suggested because of multi-organ involvement, classical radiological and histopathological features. Awareness about IgG4-RD, an under-recognized entity is essential, as it is treatable, and early recognition may help in a favourable outcome. Appropriate use of clinicopathological, serological and imaging features in the right clinical context may help in accurate diagnosis. Elevated serum IgG4 levels and biopsy are not mandatory for the diagnosis.


2018 ◽  
Vol 46 (4) ◽  
pp. 1657-1665 ◽  
Author(s):  
Wen-Ling Hsu ◽  
Shu-Min Chang ◽  
Pei-Yin Wu ◽  
Chin-Chuan Chang

Autoimmune pancreatitis (AP) is a rare autoimmune pancreatic manifestation of systemic immunoglobulin G4 (IgG4)-related sclerosing disease. Distinguishing between AP and pancreatic cancer is crucial because the clinical courses, treatments, and prognoses of these two disease entities are quite different. We herein report a case involving a 52-year-old man with subacute epigastralgia who visited our hospital for evaluation of a suspicious pancreatic mass found during esophagogastroduodenoscopy. Enhanced computed tomography (CT) revealed an enlarged lesion in the pancreatic head with encasement of hepatic vessels. The lesion also exhibited increased 18F-fluorodeoxyglucose accumulation on positron emission tomography/CT imaging, which was highly suggestive of pancreatic cancer. After open biopsy, morphologic examination showed an inflammatory infiltrate in the pancreas, which was compatible with chronic sclerotic pancreatitis. Further laboratory tests revealed an elevated serum IgG4 level, and the diagnosis of sclerotic pancreatitis was then confirmed. After corticosteroid treatment, the pancreatic lesion showed shrinkage on follow-up CT, and the serum IgG4 titer decreased to the normal range. This case suggests that clinicians should be familiar with the clinical presentations and diagnostic criteria of AP versus pancreatic cancer. An awareness of the differences between these diseases may avoid misdiagnosis and unnecessary surgical intervention.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
Y Panahi ◽  
M S Ghahrodi ◽  
M S Jamshir ◽  
M A Safarpour ◽  
M Pirro ◽  
...  

Abstract Background Plasma PCSK9 levels, a novel and effective therapeutic target for CVD prevention, have been associated with CVD events irrespective of traditional risk factors. Whether PCSK9 levels predict coronary artery disease (CAD) burden and severity is a matter of dispute. Purpose To investigate the association between plasma PCSK9 levels and CAD characteristics, including number of major diseased vessels, severity of coronary stenosis, and the burden of coronary calcifications. Methods One hundred and one patients undergoing coronary angiography were recruited for this cross-sectional study. The number of major coronary diseased vessels was defined as the presence of ≥1 stenoses ≥50% in diameter of at least one major coronary artery. CAD severity was defined as either the absence of coronary stenosis (no-CAD), CAD<50% or CAD≥50% in one or more coronary arteries. The burden of coronary calcifications was estimated by angiography visual inspection and classified as absent, mild, moderate or severe. Results Coronary angiography showed single, double and triple vessel disease in 26 (25.7%), 23 (22.8%) and 21 (20.8%) patients, respectively; 20 (19.8%) and 11 (10.9%) pts had either minimal CAD (<50%) or normal angiographic findings. Also, calcifications were absent in 65 patients (64.4%), and mild, moderate and severe in 23 (22.8%), 11 (10.9%) and 2 (2%) patients, respectively. Plasma PCSK9 levels were significantly associated with age (rho=0.22, p=0.025) and SBP (rho=0.21, p=0.034), and were almost doubled in patients with chronic kidney disease (CKD) as compared to those without CKD [164.6 ng/mL (104.6–187.0) vs 94.8 ng/mL (86.8–114.9), p=0.006]. Among patients without CKD, those with CAD≥50% had higher plasma PCSK9 levels than those without [97.1 ng/mL (87.8–143.0) vs 83.2 ng/mL (73.4–102.6), p=0.04]. In the overall population, higher plasma PCSK9 levels were found in pts with triple vessel disease [165.7 ng/mL (121.3–180.5)] than in those with double/single vessel involvement [97.9 ng/mL (87.6–99.8) and 88.4 ng/mL (87.3–97.4), p<0.001 for both comparisons] or without CAD [87.5 ng/mL (74.3–114.9), p<0.001]. Also, a trend toward an increase of plasma PCSK9 levels was found with higher CAD severity [no-CAD: 87.5 ng/mL (74.3–114.9), CAD<50%: 89.1 ng/mL (78.9–105.3), CAD≥50%: 97.6 ng/mL (87.9–155.3), p=0.051], which turned significant after exclusion of CKD patients (p=0.042). Adjustment for age, sex, plasma LDL-cholesterol levels, statin use and CKD abolished the association between PCSK9 and CAD severity but not with the number of significantly diseased vessels and the burden of coronary calcifications. Conclusions Circulating PCSK9, whose plasma levels are significantly influenced by the presence of CKD, discriminates patients with significant coronary artery stenosis from those without CAD. In addition, both the number of diseased coronary vessels and total coronary calcifications are independently predicted by an elevated plasma PCSK9 level. Acknowledgement/Funding None


2019 ◽  
Vol 6 (4) ◽  
pp. e568 ◽  
Author(s):  
Michaël Levraut ◽  
Mikaël Cohen ◽  
Saskia Bresch ◽  
Caroline Giordana ◽  
Fanny Burel-Vandenbos ◽  
...  

ObjectiveMeningeal involvement in Immunoglobulin G (IgG)-4-related disease is rare and only described in case reports and series. Because a review into the disease is lacking, we present 2 cases followed by a literature review of IgG4-related hypertrophic pachymeningitis (IgG4-HP).MethodsTwo IgG4-HP cases were reported, one involving the spinal cord and responding to surgical management and a second involving the brain and responding to Rituximab therapy. We then review clinical cases and case-series of histologically proven IgG4-HP that were published in the PubMed-NCBI database.ResultsForty-two case reports and 5 case-series were studied (60 patients, 20 women). The median age was 53. Eighteen patients had systemic involvement and 24 had single-organ IgG4-HP. Fifty-five percent of patients had an elevated serum IgG4. Treatment was surgical in 20/53 cases. Steroid therapy and immunosuppressors were effective in 85% and more than 90% of the cases, respectively. The rate of disease relapse was 42.1% after steroid therapy was discontinued.Discussion/conclusionIgG4-HP is characterized by the lack of extra-neurologic organ-involvement and systemic signs. Histopathologic studies should be performed as it is crucial for diagnosis because serum markers are rarely informative. 18F-FDG positon tomography can be useful to characterize systemic forms. There is no specific CSF marker for IgG4-HP and the diagnostic value of CSF IgG4 levels needs to be studied with larger samples. We provide a treatment algorithm for IgG4-HP. Such treatment strategies rely on early surgery, steroids, and early immunosuppressive therapy to prevent neurologic complications.


2020 ◽  
Vol 48 (10) ◽  
pp. 030006052095921
Author(s):  
Cheng Xu ◽  
Yongmei Han

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a novel clinical disease that is characterized by elevated serum IgG4 concentrations and tumefaction or tissue infiltrated by IgG4+ plasma cells. The clinical manifestations of IgG4-RD depend on the type of tissues affected. IgG4-related sclerosing cholangitis is a type of IgG4-RD. We report a patient who initially visited a local hospital with a 5-month history of jaundice. He was found to have a mass in the upper part of the common bile duct that mimicked cholangiocarcinoma. He underwent surgery in our hospital and was later diagnosed with IgG4-related sclerosing cholangitis. We administered prednisolone 40 mg once a day for treatment. Taking into account the possible side effects of moderate-dose hormone therapy, we also administered teprenone, potassium chloride, and calcium carbonate. The patient did not have any recurrence of symptoms or adverse drug reactions during follow-up.


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