Aortitis Espitia, Olivier; Toquet, Claire; Jamet, Bastien ...
La revue de medecine interne,
07/2024
Journal Article
Recenzirano
Aortitis is a rare disease entity of unknown prevalence. Primary aortitis mainly affects the thoracic aorta. They are most often diagnosed on imaging by grade III 18-FDG uptake of the aortic wall on ...PET, or by circumferential thickening>2.2mm on CT or MRI with late-stage contrast. More rarely, aortitis is histologically proven, as in some cases of clinically isolated aortitis discovered after planned aortic aneurysm surgery or during aortic dissection surgery. The most common histological types are granulomatous/giant cell or lymphoplasmacytic. Clinical signs associated with aortitis are often non-specific: asthenia, fever, dry cough, chest, back, lumbar or abdominal pain. Aortitis can be divided into different etiological categories: primary aortitis, which includes vasculitis with a preferential or exclusive tropism for the aortic wall, aortitis secondary to systemic or iatrogenic diseases, and infectious aortitis. The main etiologies of primary aortitis are giant cell arteritis (GCA), Takayasu arteritis (TA) or clinically isolated aortitis. Aortitis secondary to systemic diseases is seen in atrophying polychondritis, systemic lupus and inflammatory rheumatic diseases such as spondyloarthropathy and rheumatoid arthritis. In both ACG and AT, aortitis is a negative factor, characterized by a higher risk of relapse, cardiovascular complications and increased mortality. The management of aortitis is insufficiently codified, and relies on the control of cardiovascular risk factors, with particular monitoring of blood pressure and LDL cholesterol, and on corticosteroid therapy and immunosuppressive drugs, the use of which will depend on the disease associated with the aortitis, the initial severity and comorbidities.Aortitis is a rare disease entity of unknown prevalence. Primary aortitis mainly affects the thoracic aorta. They are most often diagnosed on imaging by grade III 18-FDG uptake of the aortic wall on PET, or by circumferential thickening>2.2mm on CT or MRI with late-stage contrast. More rarely, aortitis is histologically proven, as in some cases of clinically isolated aortitis discovered after planned aortic aneurysm surgery or during aortic dissection surgery. The most common histological types are granulomatous/giant cell or lymphoplasmacytic. Clinical signs associated with aortitis are often non-specific: asthenia, fever, dry cough, chest, back, lumbar or abdominal pain. Aortitis can be divided into different etiological categories: primary aortitis, which includes vasculitis with a preferential or exclusive tropism for the aortic wall, aortitis secondary to systemic or iatrogenic diseases, and infectious aortitis. The main etiologies of primary aortitis are giant cell arteritis (GCA), Takayasu arteritis (TA) or clinically isolated aortitis. Aortitis secondary to systemic diseases is seen in atrophying polychondritis, systemic lupus and inflammatory rheumatic diseases such as spondyloarthropathy and rheumatoid arthritis. In both ACG and AT, aortitis is a negative factor, characterized by a higher risk of relapse, cardiovascular complications and increased mortality. The management of aortitis is insufficiently codified, and relies on the control of cardiovascular risk factors, with particular monitoring of blood pressure and LDL cholesterol, and on corticosteroid therapy and immunosuppressive drugs, the use of which will depend on the disease associated with the aortitis, the initial severity and comorbidities.
Aortitis, defined by aortic parietal thickening, is noted in about 50% of patients with giant cell arteritis (GCA). Aortic structural lesions, ectasia or aneurism, may occur with or without ...inflammatory aspect of the aorta, sometimes since the diagnosis of GCA, but more frequently during follow-up. Assessment of aortic involvement, which has to be searched in each patient at the diagnosis of GCA, can be assessed using aortic imaging, angio-CT, angio-MRI or PET-CT. Prognosis of aortitis and its complications remains poorly known, but mortality due to serious events like aneurism rupture or aortic dissection, could be potentially reduced with precocious diagnosis and regular monitoring. Treatment of GCA-related aortitis is based on high-dose glucosteroids, with an initial prednisone dose at 0.7 mg/kg/d, as recommended by the French Study Group for large vessel vasculitis (GEFA). Aortic ectasia and aneurisms have to be monitored, in order to propose aortic surgery in the best conditions as possible.
Episodic angioedema with eosinophilia (EAE) (Gleich syndrome) is a rare disorder consisting of recurrent episodes of angioedema, hypereosinophilia, and frequent elevated serum IgM level.
We conducted ...a retrospective multicenter nationwide study regarding the clinical spectrum and therapeutic management of patients with EAE in France.
A total of 30 patients with a median age at diagnosis of 41 years (range, 5-84) were included. The median duration of each crisis was 5.5 days (range, 1-90), with swelling affecting mainly the face and the upper limbs. Total serum IgM levels were increased in 20 patients (67%). Abnormal T-cell immunophenotypes were detected in 12 patients (40%), of whom 5 (17%) showed evidence of clonal T-cell receptor gamma locus gene (TRG) rearrangement. The median duration of follow-up was 53 months (range, 31-99). The presence of an abnormal T-cell population was the sole factor associated with a shorter time to flare (hazard ratio, 4.15; 95% confidence interval, 1.18-14.66; P = .02). At last follow-up, 3 patients (10%) were able to have all treatments withdrawn and 11 (37%) were in clinical and biologic remission with less than 10 mg of prednisone daily.
EAE is a heterogeneous condition that encompasses several disease forms. Although patients usually respond well to glucocorticoids, those with evidence of abnormal T-cell phenotype have a shorter time to flare.
Abstract
Objective
Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small-vessel vasculitis characterized by asthma, hypereosinophilia and ANCA positivity in 40% of patients. Renal ...involvement is rare and poorly described, leading to this renal biopsy-proven based study in a large EGPA cohort.
Methods
We conducted a retrospective multicentre study including patients fulfilling the 1990 ACR criteria and/or the 2012 revised Chapel Hill Consensus Conference criteria for EGPA and/or the modified criteria of the MIRRA trial, with biopsy-proven nephropathy.
Results
Sixty-three patients 27 women, median age 60 years (18–83) were included. Renal disease was present at vasculitis diagnosis in 54 patients (86%). ANCA were positive in 53 cases (84%) with anti-MPO specificity in 44 (83%). All patients had late-onset asthma. Peripheral neuropathy was present in 29 cases (46%), alveolar haemorrhage in 10 (16%). The most common renal presentation was acute renal failure (75%). Renal biopsy revealed pauci-immune necrotizing GN in 49 cases (78%). Membranous nephropathy (10%) and membranoproliferative GN (3%) were mostly observed in ANCA-negative patients. Pure acute interstitial nephritis was found in six cases (10%); important interstitial inflammation was observed in 28 (44%). All patients received steroids with adjunctive immunosuppression in 54 cases (86%). After a median follow-up of 51 months (1–296), 58 patients (92%) were alive, nine (14%) were on chronic dialysis and two (3%) had undergone kidney transplantation.
Conclusion
Necrotizing pauci-immune GN is the most common renal presentation in ANCA-positive EGPA. ANCA-negative patients had frequent atypical renal presentation with other glomerulopathies such as membranous nephropathy. An important eosinophilic interstitial infiltration was observed in almost 50% of cases.
Scalp, tongue and/or lip necrosis are rare complications of GCA.
To describe characteristics and outcome of patients with giant cell arteritis (GCA) -related scalp, tongue and/or lip necrosis.
A ...retrospective nationwide multicenter study included 20 GCA patients with scalp, tongue, and/or lip necrosis diagnosed between 1998 and 2021 and 80 GCA control patients matched for age, sex and management period. Logistic regression analyses were conducted to identify baseline characteristics associated with scalp, tongue and/or lip necrosis.
Compared to controls, patients with scalp, tongue and/or lip necrosis showed significantly more cranial manifestations (headache, p=0.045; scalp tenderness, p=0.006; jaw claudication, p=0.02). No differences were observed between both groups regarding the occurrence of visual symptoms or large vessel involvement. At diagnosis, GCA patients with necrosis more likely received IV methylprednisolone infusions and higher doses of oral prednisone. There were no differences regarding vascular complications during follow up. Compared to controls, survival was decreased in GCA patients with necrosis (p=0.003). In a multivariable logistic regression model, scalp tenderness odds ratio (OR) 4.81(95 % CI: 1.57, 14.79), p = 0.006 and cognitive disorder OR 6.42 (95 % CI: 1.01, 40.60), p=0.048 were identified as factors associated to scalp, tongue, and/or lip necrosis.
Our results suggest that scalp, tongue, and/or lip necrosis is associated to higher mortality in GCA patients. Scalp tenderness and cognitive disorder were significant factors associated to this very rare complication of GCA.
Giant cell arteritis (GCA) is the most common systemic vasculitis in adults. In recent years, colour Doppler ultrasound of the temporal arteries (CDU) has proven to be a powerful non-invasive ...diagnostic tool, but its place in the diagnosis of GCA remains to be defined. A limitation of the CDU is the inter-operator reproducibility. Image analysis from a different perspective is now possible with the development of artificial intelligence algorithms. We propose to assess this technology for the detection of the halo sign on CDU images.
Three public hospitals retrospectively collected data from 137 patients suspected of having GCA between January 2017 and April 2019. CDU images (n=1,311) were labelled with the VIA software. Three sets (training, validation and test) were created and analysed with a semantic segmentation technique using a U-Net convolutional neural network.
The area under the curve (AUC) was 0.931 and 0.835 on the validation and test set, respectively. An image positivity threshold was determined by focusing on the specificity. With this threshold, a specificity of 95% and a sensitivity of 60% were obtained for the test set. The analysis of the false interpretation showed that the acquisition modalities and the presence of thrombus caused confusion for the algorithm.
We propose an automated image analysis tool for GCA diagnosis. The 2018 EULAR guidelines for image acquisition must be respected before generalising this algorithm. After external validation, this tool could be used as an aid for diagnosis, staff training and student education.
Objective
Temporal arteritis (TA) is a typical manifestation of giant cell arteritis (GCA). Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitides (AAVs) are rarely revealed by TA ...manifestations, leading to a risk of misdiagnosis of GCA and inappropriate treatments. This study was undertaken to describe the clinical, biologic, and histologic presentations and outcomes in cases of TA revealing AAV (TA‐AAV) compared to controls with classic GCA.
Methods
In this retrospective case–control study, the characteristics of patients with TA‐AAV were compared to those of control subjects with classic GCA. Log‐rank test, with hazard ratios (HRs) and 95% confidence intervals (95% CIs), was used to assess the risk of treatment failure.
Results
Fifty patients with TA‐AAV (median age 70 years) were included. Thirty‐three patients (66%) presented with atypical symptoms of GCA (ear, nose, and throat involvement in 32% of patients, and renal, pulmonary, and neurologic involvement in 26%, 20%, and 16% of patients, respectively). Blood samples were screened for ANCAs at the time of disease onset in 33 patients, and results were positive in 88%, leading to a diagnosis of early TA‐AAV in 20 patients. The diagnosis of AAV was delayed a median interval of 15 months in 30 patients. Compared to controls with GCA, patients with TA‐AAV were younger (median age 70 years versus 74 years), were more frequently men (48% versus 30%), and had high frequencies of atypical manifestations and higher C‐reactive protein levels (median 10.8 mg/dl versus 7.0 mg/dl). In patients with TA‐AAV, temporal artery biopsy (TAB) showed fibrinoid necrosis and small branch vasculitis in 23% of patients each, whereas neither of these characteristics was evident in controls with GCA. Treatment failure–free survival was comparable between early TA‐AAV cases and GCA controls, whereas those with delayed TA‐AAV had a significantly higher risk of treatment failure compared to controls (HR 3.85, 95% CI 1.97–7.51; P < 0.0001).
Conclusion
TA‐AAV should be considered diagnostically in cases of atypical manifestations of GCA, refractoriness to glucocorticoid treatment, or early relapse. Analysis of TAB specimens for the detection of small branch vasculitis and/or fibrinoid necrosis could be useful. Detection of ANCAs should be performed in cases of suspected GCA with atypical clinical features and/or evidence of temporal artery abnormalities on TAB.
Une aortite, définie par un épaississement pariétal de l’aorte, est observée chez environ 50 % des patients ayant une artérite à cellules géantes (ACG).
Des lésions structurales de l’aorte, ectasie ...ou anévrisme, peuvent survenir, avec ou sans aspect inflammatoire de l’aorte, parfois dès le diagnostic d’ACG, mais le plus souvent au cours du suivi.
La mise en évidence de l’atteinte aortique, à rechercher chez tout patient dès le diagnostic d’ACG, repose sur une imagerie de l’aorte, angio-TDM, angio-IRM, ou TEP-TDM.
Le pronostic de l’aortite et de ses complications reste mal connu, mais la mortalité liée aux évènements graves, rupture d’anévrisme ou dissection de l’aorte, pourrait potentiellement être réduite par un diagnostic plus précoce et une surveillance régulière.
Le traitement de l’aortite de l’ACG repose sur la corticothérapie à forte dose, avec une dose d’attaque de prednisone de 0,7 mg/kg/j selon les recommandations du Groupe d’étude français de l’artérite à cellules géantes (GEFA).
Les ectasies et anévrismes de l’aorte sont à surveiller afin de poser l’indication d’une chirurgie aortique dans les meilleures conditions possibles.
Aortitis, defined by aortic parietal thickening, is noted in about 50% of patients with giant cell arteritis (GCA).
Aortic structural lesions, ectasia or aneurism, may occur with or without inflammatory aspect of the aorta, sometimes since the diagnosis of GCA, but more frequently during follow-up.
Assessment of aortic involvement, which has to be searched in each patient at the diagnosis of GCA, can be assessed using aortic imaging, angio-CT, angio-MRI or PET-CT.
Prognosis of aortitis and its complications remains poorly known, but mortality due to serious events like aneurism rupture or aortic dissection, could be potentially reduced with precocious diagnosis and regular monitoring.
Treatment of GCA-related aortitis is based on high-dose glucosteroids, with an initial prednisone dose at 0.7 mg/kg/d, as recommended by the French Study Group for large vessel vasculitis (GEFA).
Aortic ectasia and aneurisms have to be monitored, in order to propose aortic surgery in the best conditions as possible.
ABSTRACTCardiac valvular involvement in antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis is very rare. We report the case of a patient seen in 2019, followed for ANCA-associated ...vasculitis for 15 years, who had a first relapse with cardiac valvular involvement in 2012, and who underwent a second histologically proven vasculitis relapse involving mitral periprosthetic endocardium. PET/CT imaging showed an intense and focal FDG activity of paramitral bioprosthesis leak site. Mitral bioprosthesis was replaced, and the patient improved with steroids and rituximab. Through this exceptional case, we suggest that FDG PET/CT could be of interest in the follow-up of ANCA-associated vasculitis with cardiac valvular involvement.
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