IgG4 Coronary Arteritis Presenting as a Cardiac Pseudo-Tumour

We report on an unusual case presenting with a cardiac pseudo-tumour on echocardiogram, which corresponded to a large soft tissue mural thickening around the mid-right coronary artery. There were similar but not as thick mural lesions around other parts of the coronary arteries. The so-called “pigs-in-a-blanket” sign on computed tomography (CT) scan was pathognomonic of IgG4 coronary arteritis. The IgG4 level was grossly elevated at more than 10 times the upper limit of normal. Positron emission tomography (PET)-CT scans with 18F-fluoro-deoxy-glucose (FDG) and 68Ga-Fibroblast Activation Protein Inhibitor (FAPI) were performed to assess the extent of organ involvement of the IgG4-related disease. The patient was treated with 8 injections of rituximab with good serological response. However, the coronary arteritis findings on CT scan remained unchanged.

Pneumonia and ocular disease as the primary presentations of Takayasu arteritis:a case report

Dear Editor,Takayasu arteritis(TA)is a rare,idiopathic,granulomatous,large-vessel vasculitis,with preferential involvement of the aorta,its main branches,and pulmonary arteries[1].It has been reported that the ocular involvements were found in 8.1%–68%[2],non-specific arthralgia or myalgia in 13%–41%,aortic regurgitation in 5%–55%.

“Guidezilla”extension catheter combined with balloon technique for treating pulmonary artery stenosis caused by Takayasu arteritis

Takayasu arteritis(TA)is a rare systemic vasculitis of the aorta and its primary branches,which usually occurs in young women.Due to its insidious onset and lack of specific symptoms,this disease can be easily misdiagnosed or missed.Approximately 50%of the patients having TA with pulmonary artery involvement develop pulmonary hypertension(PH).The 3-year survival rate among patients with TA-related PH is lower than that among patients with TA alone.Early balloon pulmonary angioplasty(BPA)can improve the clinical symptoms and survival of patients with stable TA.To the best of our knowledge,this is the first case reported in the English literature in which a“Guidezilla”catheter was used during BPA to treat stenosis and occlusion of the pulmonary artery caused by Takayasu arteritis(TA).

Imaging diagnosis and interventional radiology in systemic vasculitis with particular emphasis on Takayasu arteritis: Part 2

In part 1 of this article,clinical features and imaging findings of Takayasu arteritis(also known as aortitis syndrome)were described in detail.In part 2,treatment of Takayasu arteritis will be first described and discussed.This will be followed by description of clinical features and imaging findings of other systemic vasculitis.Comments on interventional radiology for systemic vasculitis will also be made.

Unilateral pleural effusion secondary to Takayasu arteritis: a case report and literature review

Takayasu arteritis (TA) is a chronic, nonspecific inflammatory disease of large and medium-sized vessels that primarily involves the aorta and its branches. TA involving the pulmonary arteries has a prevalence ranging from 14% to 86%, which can lead to pulmonary hypertension, a progressive increase in pulmonary artery pressure, and eventually death from right heart failure. The presentation of pulmonary arteritis (PA) is very nonspecific, with a reported misdiagnosis rate of up to 60% and a diagnosis time ranging from 1 month to more than 10 years. The clinical manifestation of pleural effusion is very rare in both TA and PA cases. Based on our literature review, this is the 6th reported case of TA with pleural effusion, and the specific mechanism of TA with pleural effusion is still unclear. The characteristics of this case and the previously reported cases are summarized in this article to improve the understanding of TA and PA and reduce the misdiagnosis rate.

A Rare Association of Giant Cell Arteritis with Recurrent Corneal Ulcer

We report a rare case of association of temporal arteritis with recurrent central corneal ulcer. A 91-year-old male with a remote history of clinically diagnosed giant cell arteritis (GCA) and penetrating keratoplasty (PK) for corneal edema in 1990’s in the left eye presented with irritation and severe dry eye. He progressively developed central corneal ulcer regardless of treatment with aggressive lubrication and patching. The clinical course, blood tests and temporal artery biopsy results yielded the diagnosis of recurrent GCA. The clinical course improved after starting oral steroids. The case report illustrates the importance of ruling out GCA in patients with recurrent corneal ulcers and history of GCA. It also highlights the significance of shared understanding between primary care physicians and specialists to handle common and chronic diseases to make efficient diagnoses and plan treatment regimens. This is the first plausible case of reported GCA association with central corneal ulcer in the United States.

Large Vessel Vasculitis in a Patient with Systemic Lupus Erythematosus Presenting as Takayasu’s Pulseless Arteritis

Systemic lupus erythematosus (SLE) with an associated aortoarteritis presenting as an ischemic stroke is a rarity in the medical literature. We report the case of an 11-year-old male presenting with an acute ischemic stroke meeting the criteria for the diagnosis of SLE and findings consistent with an aortitis on imaging but mimicking the diagnosis of Takayasu’s pulseless arteritis. Blood and imaging investigations revealed the finding of SLE aortitis following an acute stroke presentation. Thus, it is imperative to note that even though it is infrequent, SLE can be associated with a large vessel vasculitis.

Tuberculosis in Takayasu arteritis: a retrospective study in 1105 Chinese patients

Background Tuberculosis (TB) infection has been reported to have a possible relationship with the occurrence and clinical course of Takayasu arteritis (TA). We aimed to describe the characteristics of TB in a large population of TA patients. Methods We included a total of 1105 patients with TA, who were hospitalized between January 1992 and December 2017. Comparisons of clinical features were made according to the presence of TB. Results Among the 1105 patients, 109 (9.9%) had TB, including 53 patients (48.6%) diagnosed with TB before the onset of TA, 23 (21.1%) with a concurrent diagnosis of TB and TA, and 24 patients (22.0%) who developed TB after TA. Pulmonary TB was the most frequently identified (97 patients, 89.0%). Patients with TB had more frequent involvement of the pulmonary artery and experienced more chest discomfort and constitutional symptoms but had less interventional treatment. Demographic characteristics, comorbid diseases, and use of steroids were similar between patients with and without TB. Conclusions The proportion of Chinese TA patients with TB was not low, and about half of the patients had TB before TA. Pulmonary TB was the most common. Pulmonary artery involvement and pulmonary hypertension was more frequent in TA patients with TB.

Crohn's disease and Takayasu's arteritis: An uncommon association

Takayasu’s arteritis(TA)and Crohn’s disease(CD)are two rare autoimmune disorders;however some reports describe the presence of both diseases in the same patient.This finding has suggested the possibility that both diseases could share some common etiologic origin.We describe a case of a 13-year-old male affected by CD characterized by fever,diarrhea,weight loss,abdominal pain and elevation of inflammatory markers.Clinical and histological features from colonic specimens were consistent with CD.Treatment with steroids and azathioprine was started,however disease flared every time steroids were tapered.One year later,while still on treatment,he came back to our attention for dyspnea at rest and at night,tiredness and weakness.At physical examination a diastolic heart murmur was found as well as a left carotid artery bruit.A transthoracic echocardiography showed mild aortic valve insufficiency,left ventricular hypertrophy and a dilated ascending aorta with same findings at the aortic arch.A computed tomography scan showed abdominal aortathickening,dilated thoracic aorta and the presence of a thoracic aortic aneurysm.TA associated with CD was diagnosed and medical treatment with cyclophosphamide,steroids and aminosalicylic acid was started,with good clinical response at 6 mo follow-up.We discuss the presence of possible common causes for the two diseases and the importance of differential diagnosis in those patients characterized for intractable disease.

Anemia in patients with Takayasu arteritis: prevalence, clinical features, and treatment

Background Anemia is a common comorbidity of patients with Takayasu arteritis(TA). This study evaluated the prevalence, clinical characteristics, and treatment in Chinese TA patients with anemia. Methods This retrospective study included 533 consecutive patients hospitalized for TA from January 2009 to April 2018. Anemia was diagnosed on the basis of hemoglobin level, according to World Health Organization criteria. Results A total of 194 patients(36.4%) were diagnosed with anemia. Most had mild anemia(177, 91.2%). Female patients were predominant(92.8% of anemic patients). Normocytic anemia(62.9%) was the most common pattern. Anemic patients were more likely than non-anemic patients to have dizziness(29.4% vs. 21.2%), low body mass index(22.0 ± 3.6 vs. 22.9 ± 3.4 kg/m2), and active disease stage(64.9% vs. 50.1%);pulmonary involvement(12.4% vs. 26.8%), pulmonary hypertension(12.9% vs. 20.1%) and pulmonary hypertensive-target drugs(2.8% vs. 11.6%) were less common among anemic than non-anemic patients(all P < 0.05). Larger left ventricular end-diastolic diameter and lower left ventricular ejection fraction were observed in anemic patients. Over a median follow-up of four months, the increase of hemoglobin in anemic patients was associated with the use of iron supplementation. Conclusions Anemia is a very common concurrent condition in TA, especially in young, female patients. Patients with anemia are more likely to be in the active disease stage. Iron supplementation helps increase hemoglobin.

Drug coated balloon angioplasty for renal artery stenosis due to Takayasu arteritis: Report of five cases

BACKGROUND Takayasu arteritis is a rare but intractable chronic disease in young female patients. Percutaneous transluminal angioplasty of the involved renal arteries has been reported;however, few studies have reported the use of drug coated balloon angioplasty in the treatment of Takayasu arteritis. We aimed to demonstrate five young female patients who presented with a history of hypertension due to Takayasu arteritis. CASE SUMMARY From April 2017 to October 2018, five female patients were diagnosed with hypertension due to Takayasu arteritis by computed tomography angiography (CTA) and laboratory tests. Four patients had a complaint of headache with or without dizziness, and one patient showed no symptom. There was no significant family or past history of hypertension or kidney disease, and the physical examinations were almost normal on admission. We performed a treatment by drug coated balloon angioplasty. Blood pressure decreased dramatically in all patients after balloon angioplasty, and the patency of treated renal artery was demonstrated with CTA over 5 months after the angioplasty procedure. CONCLUSION Drug coated balloon angioplasty is safe and effective for renal artery stenosis due to Takayasu arteritis. A prospective study with a larger sample size is necessary to further demonstrate the effectiveness of the treatment.

Glomerular Disease Associated with Takayasu Arteritis:6 Cases Analysis and Review of the Literature

Objective To evaluate the clinical features,renal histopathology and therapeutic response to glucocorticoid and immunosuppressive agents in patients with glomerular disease associated with Takayasu arteritis(TA).Methods Patients with TA and renal biopsy-confirmed glomerular disease were investigated retrospectively.None of them had renal artery stenosis or occlusive changes.Results Six patients with glomerulopathy,accounting for 3.75% of the 160 TA patients admitted to our hospital at the same period,were analyzed.All of them were females with a mean age of 35.5 ± 10.0 years.Four cases presented with lower extremity edema.Laboratory tests showed that one was nephrotic syndrome,three were nephrotic range proteinuria,and two of them had mild renal dysfunction.The other two patients were asymptomatic microscopic hematuria and proteinuria.Renal pathology revealed mild immunoglobulin A nephropathy in two cases,mild mesangial proliferative glomerulonephritis(GN),membranoproliferative GN,minimal change disease,and fibrillary GN in one case respectively.Five cases received glucocorticoids and cyclophosphamide therapy.Proteinuria and microscopic hematuria disappeared in 2 to 4 weeks after the initiation of therapy in three cases.The patient with membranoproliferative GN also reached complete remission of proteinuria and recovered renal function 6 months after the treatment.Conclusions TA may induce glomerular disease as a part of its histological spectrum.Apart from ischemic glomerular disease,glomerular disease should be suspected when TA patients have microscopic hematuria or proteinuria,that may be therapeutically responsive to glucocorticoids and immunosuppressive agent in relative early phase.

Neurological Manifestations of Takayasu Arteritis

Objective To investigate the clinical neurological manifestations of Takayasu arteritis (TA). Methods A retrospective study was conducted with 63 consecutive TA cases admitted to Peking Union Medical College Hospital from January 2009 to May 2010. All the patients fulfilled the diagnostic criteria of TA by the American College of Rheumatology. Among the 63 TA patients, 27 with neurological manifestations were included in the present study. All the patients were evaluated using standardized neurological examination, sonography, computed tomography (CT) angiography, and cerebral CT or magnetic resonance imaging. Results Dizziness and visual disturbance were the most common symptoms, which occurred in 20 (74.1%) and 16 (59.3%) patients respectively. Another common symptom was headache, observed in 15 (55.6%) patients. Six (22.2%) patients had suffered from ischemic stroke; 7 (25.9%) patients had epileptic seizures. Two (7.4%) patients were diagnosed as reversible posterior encephalopathy syndrome (RPES) based on typical clinical and imaging manifestations. Conclusions Neurological manifestations are common symptoms in TA patients in the chronic phase, including dizziness, visual disturbance, headache, ischemic stroke, seizures, and some unusual ones such as RPES. We suggested RPES be included into the differential diagnosis of acute neurological changes in TA.

Clinical features of aortic dissection associated with Takayasu＇s arteritis

Takayasu＇s arteritis （TA） is a chronic and nonspecific in- flammatory vessel disease that involves the aorta and its major branches. TA results in stenosis, occlusion or aneu- rysmal degeneration of large arteries pathologically. Al- though TA is an unusual disease, it is more commonly ob- served to occur in Asian females compared to the general population worldwide. Aortic dissection is a very rare com- plication of TA, and only a limited number of TA cases presenting with aortic dissection have been published so far.

Giant cell arteritis: Current treatment and management

Glucocorticoids remain the cornerstone of medicaltherapy in giant cell arteritis(GCA) and should be started immediately to prevent severe consequences of the disease, such as blindness. However, glucocorticoid therapy leads to significant toxicity in over 80% of the patients. Various steroid-sparing agents have been tried, but robust scientific evidence of their efficacy and safety is still lacking. Tocilizumab, a monoclonal IL-6 receptor blocker, has shown promising results in a number of case series and is now being tested in a multi-centre randomized controlled trial. Other targeted treatments, such as the use of abatacept, are also now under investigation in GCA. The need for surgical treatment is rare and should ideally be performed in a quiescent phase of the disease. Not all patients follow the same course, but there are no valid biomarkers to assess therapy response. Monitoring of disease progress still relies on assessing clinical features and measuring inflammatory markers(C-reactive protein and erythrocyte sedimentation rate). Imaging techniques(e.g., ultrasound) are clearly important screening tools for aortic aneurysms and assessing patients with largevessel involvement, but may also have an important role as biomarkers of disease activity over time or in response to therapy. Although GCA is the most common form of primary vasculitis, the optimal strategies for treatment and monitoring remain uncertain.

A complicated case of innominate and right common arterial aneurysms due to Takayasu’s arteritis

BACKGROUND Innominate artery aneurysms (IAAs) are relatively rare. Endovascular therapy has been an alternative to open surgery in some IAA cases, but open repair is still necessary in complicated cases. CASE SUMMARY We report a 35-year-old female who suffered from Takayasu’s arteritis. The patient did not get regular treatment, and IAA and right common carotid artery aneurysm developed, which complicated with occlusion of the left carotid artery, subclavian artery, and the initial part of the left vertebral artery. The patient also had moderate aortic valve insufficiency. With inflammation being controlled well, the patient received the surgery for arterial aneurysms of innominate and right common carotid arteries and aortic valve insufficiency. The shunts for cerebral blood supply were designed to protect the brain and the surgery was conducted successfully under extracorporeal circulation. CONCLUSION The case illustrates that open surgery may be appropriate for some complicated IAAs, and brain protection is important.

Prevalence and long-term prognosis in Takayasu arteritis patients with left ventricular dilatation and a decreased ejection fraction:data from 1,105 Chinese cases

Background Left ventricular dilatation and a decreased ejection fraction is one of the most serious complications in patients with Takayasu arteritis(TA).We aim to describe the prevalence,clinical features,treatment,and long-term prognosis of TA patients with this complication.

Malignancy in Chinese patients with Takayasu arteritis

Objective Takayasu arteritis(TA)is a kind of large vessel vasculitis of unknown reason.Autoimmune disorder was perhaps one of the risk factors.Cancer was related to autoimmune disease.This study aimed to describe the type and incidence of malignancy in 1,105 TA patients.

Anemia in Chinese patients with Takayasu arteritis

Objective Anemia as a concomitant disease of patients with Takayasu arteritis (TA) has been reported in several case reports, but epidemiological studies of large populations are lacking. This study evaluated the prevalence, clinical characteristics, and medical treatment in Chinese TA population with anemia.

Transthoracic arterial bypass for the treatment of severe cerebral ischemia induced by brachiocephalic arteritis: A report of 15 cases

BACKGROUND： Carotid arterial reconstruction is hard to cure the extensive lesions of carotid arteries and subclavian arteries in patients with brachiocephalic arteritis; however, transthoracic arterial bypass surgery provides an opportunity for the treatment of brachiocephalic arteritis. OBJECTIVE： To report the improving effects of transthoracic arterial bypass surgery on the clinical symptoms of severe cerebral ischemia induced by brachiocephalic arteritis and observe the occurrence of complications after the intervention. DESIGN： Case observation. SETTING： Department of Vascular Surgery, Union Hospital Affiliated to Tongji Medical College, Huazhong University of Science and Technology. PARTICIPANTS： Fifteen patients with Takayasu arteritis, including 1 male and 14 females, were selected from Department of Vascular Surgery, Wuhan Union Hospital from June 2003 to June 2007. Their ages ranged from 16 to 37 years. All patients were finally diagnosed by clinical histories, health examinations and color Doppler detection. All patients provided the confirmed consent. METHODS： All patients had received transthoracic artificial vascular bypass surgery. Among them, 6 patients had the bridge from ascending aorta to bilateral axillary arteries to lateral internal carotid artery; 3 from ascending aorta to bilateral axillary arteries; 5 from ascending aorta to lateral axillary artery to lateral internal carotid artery; In particular, one patient combining with abdominal aorta occlusion had received two step surgical interventions. In the first step, bridging surgery was performed from ascending aorta to bilateral axillary arteries to lateral internal carotid artery; in the second step, patients received left axillofemoral bypass. MAIN OUTCOME MEASURES：① Detecting blood velocity in variously intracranial-arterial systoles by using transcranial Doppler postoperatively; ② following-up the improve of clinical symptoms at 40 months after surgery; ③ observing postoperative complications. RESULTS： All 15 patients were involved in the final analysis. ① Intracranial-arterial blood velocity： Average intracranial-arterial blood velocity was significantly increased postoperatively （P 〈 0.01）. ② Following-up results of clinical symptoms： All patients did not have death and blindness; while, transient cerebral ischemia, dizzy, photophobia, giddiness, hemoptysis and other symptoms disappeared gradually; therefore, patients were able to live by themselves. ③ Postoperative complications： Among them, 3 patients had injury of hypoglossal nerve and 3 patients had injury of recurrent laryngeal nerve. All recovered after 1 - 3 months conservative treatment. One patient with injury of brachial plexus nerve on one side relieved gradually after a half-year treatment. One patient had occlusion in subclavian arterial bridge on the third day after surgery and once more embolism after arterial embolectomy on the next day; however, the limb did not have obvious ischemic symptoms but low skin temperature as compared with the contralateral side. CONCLUSION： Transthoracic arterial bypass surgery can relieve clinical symptoms of brachiocephalic arteritis postoperatively, complications are mild, and the effects are confirmed.