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RMD Open Aug 2023To determine the impact of ultrasound (US) intrinsic limitation to assess aortitis versus FDG-PET/CT in patients with US-proven giant cell arteritis (GCA) and to... (Observational Study)
Observational Study
OBJECTIVE
To determine the impact of ultrasound (US) intrinsic limitation to assess aortitis versus FDG-PET/CT in patients with US-proven giant cell arteritis (GCA) and to identify factors associated with aortic involvement.
METHODS
Retrospective observational study of patients referred to US fast-track clinics at two academic centres over a 4-year period. Only patients with GCA confirmed by US were included. Temporal arteries (TA) and extracranial arteries US were performed at baseline. FDG-PET/CT was performed according to clinician's criteria. An FDG artery uptake at the aorta higher than liver uptake was considered positive for aortitis.
RESULTS
Seventy-two of 186 patients with US-proven GCA underwent an FDG-PET/CT; 29 (40.3%) had a positive FDG-PET/CT and 24 (33.3%) presented aortitis. Only 6 (20.7%) patients with positive FDG-PET/CT had negative US findings of large vessel (LV)-GCA. Among patients with aortitis in FDG-PET/CT, only two (8.3%) had negative US findings of LV-GCA. Patients with aortitis were younger (68.9 vs 81;p<0.001), more frequently females (79.2% vs 39.6%;p=0.002) and had higher platelets count (413.4 vs 311.1;p=0014). Patients with aortitis presented positive TA US less frequently (41.7% vs 83.3%;p<0.001), but more LV US involvement (91.7% vs 41.7%; p<0.001) versus patients without aortitis. None of the patients with aortitis exhibited visual symptoms (0% vs 31.2%;p=0.001).
CONCLUSIONS
FDG-PET/CT can detect aortitis in one out of every three patients with US-proven GCA. However, a negative US examination for LV-GCA suggests a low risk of aortitis. Younger and female GCA patients with thrombocytosis, absence of visual manifestations and LV-GCA on US may more frequently present aortitis by FDG-PET/CT.
Topics: Humans; Female; Giant Cell Arteritis; Aortitis; Fluorodeoxyglucose F18; Positron Emission Tomography Computed Tomography; Ultrasonography
PubMed: 37597848
DOI: 10.1136/rmdopen-2023-003329 -
International Journal of Hematology Dec 2023A 45-year-old man who was a sibling donor for allogeneic peripheral blood stem cell transplantation (allo-PBSCT) was administered 7.2 mg of pegfilgrastim for stem cell...
A 45-year-old man who was a sibling donor for allogeneic peripheral blood stem cell transplantation (allo-PBSCT) was administered 7.2 mg of pegfilgrastim for stem cell collection. Peripheral blood stem cells were collected 4 days after administration of pegfilgrastim (Day 4) and 4.32 × 10 /kg of CD34-positive cells per recipient body weight were obtained. Fever of 38 ℃ or higher and left submandibular pain appeared on Day 6. Ultrasonography and contrast-enhanced computed tomography (CT) showed wall thickening of the carotid artery and the abdominal aorta. We carefully excluded the possibilities of cardiovascular and autoimmune diseases by thorough examination, and ultimately diagnosed pegfilgrastim-induced aortitis. The patient's fever resolved rapidly after treatment with prednisolone (PSL) 1 mg/kg. We began to taper PSL after eight days. Sixty-one days after starting PSL, we confirmed that abdominal aortic wall thickening had improved by contrast-enhanced CT. We continued to taper off PSL and stopped 141 days later with no relapse thereafter. This is the first case report of pegfilgrastim-induced aortitis in an allo-PBSCT donor. Careful monitoring is warranted when administering pegfilgrastim to donors even without past medical history.
Topics: Male; Humans; Middle Aged; Granulocyte Colony-Stimulating Factor; Aortitis; Peripheral Blood Stem Cells; Filgrastim; Hematopoietic Stem Cell Transplantation
PubMed: 37578593
DOI: 10.1007/s12185-023-03649-0 -
Cerebellum (London, England) Jun 2024We describe a male patient presenting with cerebellar ataxia and behavioural frontotemporal dementia in whom imaging showed cerebellar atrophy. He had significantly low...
We describe a male patient presenting with cerebellar ataxia and behavioural frontotemporal dementia in whom imaging showed cerebellar atrophy. He had significantly low N-acetyl aspartate to creatine (NAA/Cr) area ratio on MR spectroscopy of the cerebellum, primarily affecting the vermis. CT body scan showed extensive abnormal tissue within the mesentery, the retroperitoneum and perinephric areas. PET-CT showed increased tracer uptake within the wall of the aorta suggestive of an aortitis and within the perinephric tissue bilaterally. Biopsy of the perinephric tissue confirmed IgG4 disease. Treatment with steroids and mycophenolate improved his clinical state, but he developed symptoms attributed to pericardiac effusion that necessitated treatment initially with drainage and subsequently with pericardial window. After a course of rituximab, he had an episode of sepsis that did not respond to appropriate treatment and died as a result. Both the imaging findings and neurological presentation with cerebellar ataxia and behavioural frontotemporal dementia are novel in the context of IgG4 disease.
Topics: Humans; Male; Cerebellar Ataxia; Immunoglobulin G4-Related Disease; Middle Aged; Frontotemporal Dementia
PubMed: 37558930
DOI: 10.1007/s12311-023-01592-8 -
The Israel Medical Association Journal... Jul 2023Perivascular cuffing as the sole imaging manifestation of pancreatic ductal adenocarcinoma (PDAC) is an under-recognized entity.
BACKGROUND
Perivascular cuffing as the sole imaging manifestation of pancreatic ductal adenocarcinoma (PDAC) is an under-recognized entity.
OBJECTIVES
To present this rare finding and differentiate it from retroperitoneal fibrosis and vasculitis.
METHODS
Patients with abdominal vasculature cuffing were retrospectively collected (January 2011 to September 2017). We evaluated vessels involved, wall thickness, length of involvement and extra-vascular manifestations.
RESULTS
Fourteen patients with perivascular cuffing were retrieved: three with celiac and superior mesenteric artery (SMA) perivascular cuffing as the only manifestation of surgically proven PDAC, seven with abdominal vasculitis, and four with retroperitoneal fibrosis. PDAC patients exhibited perivascular cuffing of either or both celiac and SMA (3/3). Vasculitis patients showed aortitis with or without iliac or SMA cuffing (3/7) or cuffing of either or both celiac and SMA (4/7). Retroperitoneal fibrosis involved the aorta (4/4), common iliac (4/4), and renal arteries (2/4). Hydronephrosis was present in 3/4 of retroperitoneal fibrosis patients. PDAC and vasculitis demonstrated reduced wall thickness in comparison to retroperitoneal fibrosis (PDAC: 1.0 ± 0.2 cm, vasculitis: 1.2 ± 0.5 cm, retroperitoneal fibrosis: 2.4 ± 0.4 cm; P = 0.002). There was no significant difference in length of vascular involvement (PDAC: 6.3 ± 2.1 cm, vasculitis: 7.1 ± 2.6 cm, retroperitoneal fibrosis: 8.7 ± 0.5 cm).
CONCLUSIONS
Celiac and SMA perivascular cuffing can be the sole finding in PDAC and may be indistinguishable from vasculitis. This entity may differ from retroperitoneal fibrosis as it spares the aorta, iliac, and renal arteries and demonstrates thinner walls and no hydronephrosis.
Topics: Humans; Retroperitoneal Fibrosis; Retrospective Studies; Aorta; Vasculitis; Pancreatic Neoplasms
PubMed: 37461174
DOI: No ID Found -
Cureus Jun 2023Mycotic aneurysm is defined as an infection of the arterial wall either by fungi or bacteria. Although, a rare complication of infection, it is associated with high...
Mycotic aneurysm is defined as an infection of the arterial wall either by fungi or bacteria. Although, a rare complication of infection, it is associated with high morbidity and mortality. We describe a 69-year-old female with a rare thoracic aortic mycotic aneurysm, with no clear source of infection and a predominantly atypical presentation, manifesting primarily as heart failure, at a rural community hospital. Our case also depicts the rapid development of aortitis and mycotic aneurysms. This case highlights the challenges in the diagnosis and management of this condition.
PubMed: 37456379
DOI: 10.7759/cureus.40336 -
Annals of Vascular Surgery Nov 2023Endovascular repair of inflammatory abdominal aortic aneurysms (IAAAs) has emerged as an alternative to open surgery, but direct comparisons are limited. The aim of the...
BACKGROUND
Endovascular repair of inflammatory abdominal aortic aneurysms (IAAAs) has emerged as an alternative to open surgery, but direct comparisons are limited. The aim of the study was to compare clinical outcomes of endovascular and open repair for IAAA according with specific clinical characteristics.
METHODS
We performed a literature review of reports describing patients who had open or endovascular repair for IAAA. A literature search was performed in June 2022 by 2 investigators who conducted a review of papers reported in PubMed, Embase, MEDLINE, and Cochrane Database. The strings "Inflammatory aneurysm" and "Abdominal Aortic Aneurysms" were used. There was no language restriction and screened reports were published from March 1972 to December 2021. We identified 2,062 patients who had open (1,586) or endovascular repair (476) for IAAA. Primary outcomes were operative mortality and morbidity. Secondary outcomes were complications during follow-up (mean follow-up: 48 months). Propensity score matching was performed between patients who had open or endovascular surgery.
RESULTS
In Western countries, propensity-weighted postoperative mortality (in-hospital) (1.5% endovascular vs. 6% open) and morbidity rates (6% vs. 18%) were significantly lower in patients who had endovascular repair (P < 0.0001); patients with larger aneurysm (more than 7 cm diameter), signs of active inflammation, and retroperitoneal rupture of the aneurysm had better outcomes after endovascular repair than after open surgery. Hydronephrosis was present in 20% of the patients. Hydronephrosis regressed in most patients when signs of active inflammation were present suggesting an acute onset of the hydronephrosis itself (fever, elevated serum C Reactive Protein) either after endovascular or open surgery. Long-standing hydronephrosis as suggested by the absence of signs of active inflammation rarely regressed after endovascular surgery despite associated steroid therapy. During a mean follow-up of 48 months, propensity-weighted graft-related complications were more common in patients who had endovascular repair (20% vs. 8%). For patients from Asia, short-term and medium-term results were similar after open and endovascular repair. IAAAs related with aortitis were more common in Asia. In Western countries, IAAAs were commonly associated with atherosclerosis.
CONCLUSIONS
Patients with IAAA represent a heterogeneous population, suggesting biological differences from continent to continent; conservative therapy and endovascular or open surgery should be chosen according to the patient clinical condition. Endovascular repair presents advantages in patients with signs of active inflammation and contained rupture of the IAAA and larger aneurysms. Hydronephrosis, without signs of active inflammation, rarely regresses after endovascular repair associated with steroid therapy. Further studies are needed to establish the long-term results of endovascular repair.
Topics: Humans; Aortic Aneurysm, Abdominal; Aortitis; Endovascular Procedures; Hydronephrosis; Inflammation; Postoperative Complications; Retrospective Studies; Risk Factors; Steroids; Treatment Outcome
PubMed: 37454897
DOI: 10.1016/j.avsg.2023.06.036 -
Journal of Cardiothoracic Surgery Jul 2023Salmonella spp. cause infectious aortitis through the hematogenous spread of an intestinal Salmonella infection. Salmonella aortitis can result in extensive tissue...
BACKGROUND
Salmonella spp. cause infectious aortitis through the hematogenous spread of an intestinal Salmonella infection. Salmonella aortitis can result in extensive tissue damage in the aorta leading to complications including dissection, abscess formation, pseudoaneurysms, and rupture, which require early diagnosis and treatment with both surgery and antibiotic therapy.
CASE PRESENTATION
We report a case of Salmonella aortitis complicated by Stanford type A aortic dissection. A 62-year-old man with a history of heroin use presented with chest pain, epigastric pain and vomiting. The computed tomography scan showed Stanford type A aortic dissection without malperfusion. At the time of surgery, an aortic dissection with purulent fluid and contained rupture was noted in the ascending aorta. Fluid culture was consistent with Salmonella. A composite valve-graft conduit aortic root replacement with ascending aorta and hemiarch replacement was performed. The patient recovered well and was discharged on long-term antibiotics.
CONCLUSIONS
This rare case of a Stanford type A aortic dissection with contained rupture due to Salmonella aortitis was successfully treated with emergent surgery and antibiotic therapy.
Topics: Male; Humans; Middle Aged; Aortitis; Aortic Dissection; Aorta; Salmonella; Anti-Bacterial Agents
PubMed: 37452382
DOI: 10.1186/s13019-023-02318-x -
HCA Healthcare Journal of Medicine 2022Description Vasculitides are a group of diseases that produces vasculitis, which is characterized by inflammatory infiltrates within blood vessel walls and results in...
Description Vasculitides are a group of diseases that produces vasculitis, which is characterized by inflammatory infiltrates within blood vessel walls and results in intimal injury as well as progressive mural destruction. Infiltrates are characterized per the Chapel Hill classification, into large, medium, and small-vessel vasculitides. ANCA-associated vasculitis (AAV) is a disease that has been described as involving small-sized vessels. However, some cases of large vessel disease involvement have been documented. ANCA-associated aortitis is a rare entity poorly described in the literature. Due to the rarity of this pathology, there is no Level I evidence available regarding diagnosis and treatment. We present the rare case of an 80-year-old male presenting with ANCA-associated aortitis complicated by acute dissection of the left common iliac artery. His case was successfully managed by corticosteroid therapy and endovascular stenting of the involved iliac artery. ANCA-associated aortitis is a rare entity that has not been described well in the current literature. We believe this case to be the first involving ANCA-associated aortitis presenting with an acute dissection.
PubMed: 37426865
DOI: 10.36518/2689-0216.1262 -
Journal of Ayub Medical College,... 2023Williams syndrome (WS), is a multisystem disorder occurring in 1 in 10,000 live births with supravalvular aortic stenosis (SVAS) being the most common cardiovascular...
Williams syndrome (WS), is a multisystem disorder occurring in 1 in 10,000 live births with supravalvular aortic stenosis (SVAS) being the most common cardiovascular manifestation. We present the case of a 2.5 years old male, a known case of WS who presented with cognitive delay, a history of right-sided stroke and left hemiplegia. Echocardiography revealed severe SVAS with a gradient of 105 mmHg. The diameter of the Sino tubular junction was 4 mm. Computerized tomography angiogram showed diffuse stenosis of ascending aorta with intraluminal thrombus. At surgery, the ascending aorta was augmented with autologous pericardial patches and end-to-end anastomosis of the proximal and distal aorta completed the reconstruction. The patient was discharged in a stable condition. He presented 6 weeks post-op with a pulsating pseudoaneurysm through the sternal wound. Emergency surgery with the removal of fungal vegetation and reconstruction of the ascending aorta was performed. He expired due to fungal sepsis a week later.
Topics: Male; Humans; Child; Child, Preschool; Aortic Stenosis, Supravalvular; Williams Syndrome; Aortitis; Aorta; Echocardiography
PubMed: 37422830
DOI: 10.55519/JAMC-02-10055