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European Journal of Internal Medicine Jun 2024Aortitis in Giant Cell Arteritis (GCA-aortitis) is a frequent complication that may lead to aneurysms. Tocilizumab (TCZ) was approved in GCA, but the efficacy in...
OBJECTIVE
Aortitis in Giant Cell Arteritis (GCA-aortitis) is a frequent complication that may lead to aneurysms. Tocilizumab (TCZ) was approved in GCA, but the efficacy in GCA-aortitis and aneurysms has not been analyzed to date. Our aim was to assess the effectiveness and safety of TCZ in a wide series of GCA-aortitis and aneurysms.
METHODS
Multicentre observational study with GCA-aortitis treated with TCZ. GCA was diagnosed by: a) ACR criteria, b) temporal artery biopsy, and/or c) imaging techniques. Aortitis was diagnosed mainly by PET/CT. Main outcomes were EULAR and imaging remission. Others were clinical remission, analytical normalization, corticosteroid-sparing effect, and the prevention and improvement of aneurysms.
RESULTS
196 patients with GCA-aortitis treated with TCZ. After 6 months, 72.2% reached EULAR remission but only 12% an imaging remission; increasing up-to 81.4% and 31.8%, respectively, at 24 months. A rapid clinical remission, ESR and CRP normalization was observed in 47.4%, 84.3% and 55.6%, at 1 month, increasing to 89.6%, 85.3% and 80.3% at 24 months, respectively. Aneurysms were present in 10 (5%) patients. Five of them required early surgery, while 3 others enlarged. No patient on TCZ therapy developed aneurysms during follow-up.
CONCLUSION
In patients with GCA-aortitis treated with TCZ, a rapid and maintained clinical and analytical improvement was observed. However, there was an uncoupling between clinical and EULAR remission with imaging remission.
PubMed: 38908981
DOI: 10.1016/j.ejim.2024.06.013 -
EJVES Vascular Forum 2024Primary aorto-enteral fistula (PAEF) is a connection between the gastrointestinal tract and the aorta that occurs without previous aortic surgery. The aetiological...
OBJECTIVE
Primary aorto-enteral fistula (PAEF) is a connection between the gastrointestinal tract and the aorta that occurs without previous aortic surgery. The aetiological factors include, but are not limited to, aneurysm, infection, and tumours. It is a life threatening condition if untreated and requires emergency vascular surgical repair. A secondary aorto-enteric fistula (AEF) can occur to a previously reconstructed aorta. This case report presents a unique case of a male patient who developed a primary AEF and subsequent secondary AEF with successful surgical outcomes, suggested to be due to tuberculous aortitis.
REPORT
The patient was diagnosed and treated for tuberculosis and developed a saccular aneurysm within six months. The PAEF was surgically corrected with a tube graft using a bovine pericardial patch, the defect in duodenum was sutured, and a retrocolic omental flap was created between the duodenum and aorta. He developed a small stable pseudoaneurysm during follow up, and then a secondary AEF two and a half years later, in which a connection between the pseudoaneurysm and duodenum was corrected using a new bovine aorto-aortic interposition graft using a bovine pericardium patch. The defect in the duodenum was also sutured in two layers and a new omental flap was created.
DISCUSSION
The mortality rate of AEF is high and it is very unlikely that a patient will survive two AEFs without major complications. It is believed that there are extremely few double AEF cases described in the literature. The aetiological factor in the development of PAEF in this case was most likely the patient's aortic aneurysm, which was most likely of mycotic origin due to tuberculosis. The patient developed a pseudoaneurysm during follow up and it is uncertain whether the pulsatile pressure of the pseudoaneurysm led to the recurrence of the AEF.
PubMed: 38884073
DOI: 10.1016/j.ejvsvf.2024.05.005 -
Frontiers in Medicine 2024Brucella infection is uncommon among peritoneal dialysis (PD) patients in non-endemic areas, and the occurrence of both peritonitis and abdominal aortitis is rare.
BACKGROUND
Brucella infection is uncommon among peritoneal dialysis (PD) patients in non-endemic areas, and the occurrence of both peritonitis and abdominal aortitis is rare.
CASE PRESENTATION
In December 2023, a 63-year-old male patient undergoing PD was admitted to Shaoxing Second Hospital due to fever, abdominal pain, and cloudy dialysate. Upon physical examination, diffuse mild abdominal pain and tenderness were observed. Subsequent investigation into the patient's medical history revealed consumption of freshly slaughtered lamb from local farmers 3 days prior to the onset of symptoms. Various diagnostic tests, including routine blood tests, procalcitonin levels, and PD fluid analysis, indicated the presence of infection. Abdominal computed tomography (CT) imaging revealed localized lumen widening of the abdominal aorta with surrounding exudative changes. On the sixth day in the hospital, blood and PD fluid cultures confirmed infection. The patient was diagnosed with brucella-associated peritonitis and aortitis. Treatment was adjusted to include rifampin and doxycycline for 6 weeks, and the decision was made to keep the PD catheter. Remarkably, the patient exhibited resolution of peritonitis and abdominal aortitis within the initial week of the adjusted treatment. Currently, the patient continues to receive ongoing clinical monitoring.
CONCLUSION
Brucella is rare but can cause PD-associated peritonitis and arteritis. Prompt diagnosis and treatment can lead to a good outcome in PD patients. Dual therapy is effective, but the need for catheter removal is unclear. Consider international guidelines and patient factors when deciding on catheter removal.
PubMed: 38854664
DOI: 10.3389/fmed.2024.1393548 -
The Canadian Journal of Cardiology Jun 2024We present the case of a 55-year-old male with a pseudoaneurysm in the right coronary sinus of Valsalva. He was found to have a complete heart block in the context of...
We present the case of a 55-year-old male with a pseudoaneurysm in the right coronary sinus of Valsalva. He was found to have a complete heart block in the context of large-vessel vasculitis involving the aortic root and basal interventricular septum. This case demonstrates the importance of generating a thorough differential diagnosis of complete heart block and using multimodality imaging to pursue the investigations for aortitis when a septal recess near the aortic root is detected. Considering to proceed to a surgical intervention earlier in the progression of the disease should also be part of the management.
PubMed: 38852846
DOI: 10.1016/j.cjca.2024.05.029 -
Revista Clinica Espanola Jun 2024Systematic review of current evidence to analyze the prevalence of extracranial large vessel vasculitis (LVV) using F-FDG PET/CT in patients with polymyalgia rheumatica...
OBJECTIVE
Systematic review of current evidence to analyze the prevalence of extracranial large vessel vasculitis (LVV) using F-FDG PET/CT in patients with polymyalgia rheumatica (PMR) or giant cell arteritis (GCA).
MATERIALS AND METHODS
PubMed and EMBASE were searched and the results were screened by two reviewers. Study quality was assessed using a modified version of the Newcastle-Ottawa scale. Heterogeneity between studies was assessed using the I statistic and the Q test. Further subgroup analyses were performed by disease type, study quality, and F-FDG PET/CT uptake criteria. Publication bias was assessed by funnel plot and Egger's test.
RESULTS
268 publications were identified, of which 17 met the selection criteria and were included in the meta-analysis. The overall pooled prevalence of extracranial LVV by F-FDG PET/CT was 54.5% [95% CI: 42.6%-66.1%]. In patients with GCA the prevalence was significantly higher than in patients with PMR (60.1% vs. 41.8%, P = 0.006). Likewise, studies with a lower risk of bias reported a higher prevalence of extracranial LVV (61.1% vs. 46.9%; P = 0.010). No publication bias was observed.
CONCLUSIONS
The F-FDG PET/CT test may be useful in the detection of extracranial LVV, both in patients with PMR or GCA. Such involvement is more frequent in patients with GCA, and may vary depending on the quality of the studies.
PubMed: 38852739
DOI: 10.1016/j.rceng.2024.06.005 -
Medicina Clinica Jun 2024IgG4-related disease (IgG4-RD) is a rare, systemic immune-mediated fibro-inflammatory condition with an unclear etiology and pathophysiology, potentially affecting...
BACKGROUND
IgG4-related disease (IgG4-RD) is a rare, systemic immune-mediated fibro-inflammatory condition with an unclear etiology and pathophysiology, potentially affecting multiple organs. It presents with common clinical, radiological, and serological characteristics. This study aims to compare the latest two IgG4-RD classification and diagnostic criteria: Umehara-Okazaki 2011 and ACR/EULAR 2019.
MATERIAL AND METHODS
In a retrospective cross-sectional study conducted across two centers from January 2010 to July 2023, we included patients suspected of having IgG4-RD from various hospital departments. Patients finally diagnosed with other pathologies were excluded. The remaining suspected IgG4-RD cases were evaluated using both Umehara-Okazaki 2011 and ACR/EULAR 2019 criteria.
RESULTS
Out of 34 patients with a clinical diagnosis of IgG4-RD, the Umehara-Okazaki 2011 classified 20 patients: 5 as definitive, 7 as probable, and 8 as possible cases. Applying the ACR/EULAR 2019 criteria to the same cohort resulted in the diagnosis of 9 patients. Notably, retroperitoneal fibrosis and aortitis were the most prevalent form of presentation, accounting for 25% and 22.2% of cases classified under the 2011 and 2019 criteria, respectively.
DISCUSSION
The more recent and stringent ACR/EULAR 2019 criteria focus on histopathology, various forms of presentation, and analytical data, allow for a more accurate classification of patients.
PubMed: 38849269
DOI: 10.1016/j.medcli.2024.03.026 -
JACC. Case Reports Jun 2024A 54-year-old woman with a diagnosis of Erdheim-Chester disease under therapy with dabrafenib presents with clinical signs of heart failure. After discontinuing the...
A 54-year-old woman with a diagnosis of Erdheim-Chester disease under therapy with dabrafenib presents with clinical signs of heart failure. After discontinuing the offending medication and initiating guideline-directed medical therapy for heart failure with reduced ejection fraction, the clinical picture improved.
PubMed: 38808127
DOI: 10.1016/j.jaccas.2024.102374 -
Caspian Journal of Internal Medicine 2024Intracranial inflammatory pseudotumours (IPT) are rare entities that frequently lead to misdiagnosis with malignant lesions. The identification of these lesions is...
BACKGROUND
Intracranial inflammatory pseudotumours (IPT) are rare entities that frequently lead to misdiagnosis with malignant lesions. The identification of these lesions is difficult, but important to avoid inadvertent iatrogenicity and to adjust therapeutic protocols.
CASE PRESENTATION
We report the case of a 30-year-old man who presented a single tonic-clonic seizure. Brain imaging showed a right frontal lesion with intra and extra axial components. Facing the radiologic presentation, a brain tumor was suspected, thus the patient underwent surgery. Pathological exam concluded to a plasma cell granuloma. A whole-body CT-scan showed only a thoracic aortitis. Complete blood work studies came back negative. The patient was also tested for an array of antibodies among which antinuclear antibodies were positive (blood level superior to 1/100). CSF evaluation revealed clear fluid with normal glucose concentration, normal protein levels and lymphocytic pleocytosis. Finally, IgG-4 plasma levels were elevated which led to the diagnosis of an IgG4-RD. The patient was put under prednisolone with a favorable outcome.
CONCLUSION
IPT have several etiologies, among which IgG4 related disease may be one of the less known as only 2 cases have previously been reported. Herein, we report a new case of a young man who presented for seizures related to an intracranial lesion of an IgG4 related disease. The challenge is to suspect such conditions to avoid unnecessary surgeries.
PubMed: 38807725
DOI: 10.22088/cjim.15.2.354 -
QJM : Monthly Journal of the... May 2024Giant cell arteritis (GCA) is one of the most common large vessel (LVV) vasculitis and is associated with a high risk of relapse and cardiovascular complications....
OBJECTIVE
Giant cell arteritis (GCA) is one of the most common large vessel (LVV) vasculitis and is associated with a high risk of relapse and cardiovascular complications. Improving risk stratification remains a significant issue in this patient population. We aimed to perform a cluster analysis among GCA to identify clusters and evaluate their prognostic value.
METHODS
In a multicenter cohort study, we performed hierarchical cluster analysis on the factor analysis of mixed data coordinates results with 283 GCA patients' characteristics to generate clusters and assess incidence of relapse, cardiovascular events and death.
RESULTS
Three clusters were identified: "Vascular relapsing profile" (23.0%), "Typical GCA profile" (47.7%), and "Ophthalmologic elderly profile" (29.3%). The "Vascular relapsing profile" cluster included younger patients with more frequent relapses and cardiovascular events, particularly thoracic aortic aneurysms. The "Typical GCA profile" was the largest, with classic cranial manifestations and frequently associated polymyalgia rheumatica. The "Ophthalmologic elderly profile" had the oldest patients with more visual loss and the highest mortality rate.
CONCLUSIONS
Our findings underline the varied prognostic landscape within GCA, emphasizing the poor cardiovascular prognosis of younger patients with LV involvement and the higher mortality among elderly patients. This reinforces the need for further research regarding the screening of aortic abnormalities and whether those patients might benefit from intensive treatment with biotherapy and cardiovascular risk factors management.
PubMed: 38806178
DOI: 10.1093/qjmed/hcae105 -
Khirurgiia 2024Syphilitic aortitis is a rare disease caused by Treponema pallidum affecting the aorta and leading to inflammation. Syphilitic aortitis is one of the causes of aortic...
Syphilitic aortitis is a rare disease caused by Treponema pallidum affecting the aorta and leading to inflammation. Syphilitic aortitis is one of the causes of aortic aneurysms. This article presents surgical treatment of a patient with syphilitic aortitis and thoracic aortic aneurysm. This clinical case confirms the difficulties of surgical treatment.
Topics: Humans; Syphilis, Cardiovascular; Male; Aortic Aneurysm, Thoracic; Treatment Outcome; Treponema pallidum; Blood Vessel Prosthesis Implantation; Middle Aged; Aortitis
PubMed: 38785248
DOI: 10.17116/hirurgia2024051123