Non-complicated giant-cell arteritis is defined by the absence of ischemic complications, a remarkable response to corticosteroid treatment, and the absence of corticodependence. It is the most ...common type of giant-cell arteritis. Treatment relies on corticosteroids (prednisone or prednisolone) at an initial dosage of 0.7 mg/kg/j. The median duration of treatment is 2 years, but many patients need a more prolonged course. Side effects are frequent, especially atherosclerosis and osteoporosis. There is no demonstrated efficiency of corticosteroid sparing agents such as hydroxychloroquine or methotrexate. Dapsone may be efficient, but is not indicated because of its serious side-effects.
La polychondrite chronique atrophiante (PCA) est une maladie exceptionnelle, inflammatoire, d’évolution capricieuse. Le diagnostic est avant tout clinique, devant l’inflammation typique du pavillon ...de l’oreille, du nez et/ou l’atteinte trachéobronchique. L’atteinte rhumatismale est principalement une polyarthrite périphérique non destructrice et non déformante. Les atteintes extracartilagineuses (aortite, valvulopathie, atteinte cochléaire, ophtalmologique, cutanée, du système nerveux central) font de la PCA une maladie systémique. Le syndrome inflammatoire biologique est inconstant et il n’y a pas de biomarqueur validé de PCA. Une EFR avec boucle débit-volume et un scanner thoracique (ou une IRM) en inspiration et expiration sont systématiques, pour rechercher un syndrome obstructif et un épaississement, une prise de contraste des cartilages laryngés et/ou trachéobronchique et une éventuelle malacie. La place de la TEP-FDG est discutée. Certains examens (échocardiographie, audiogramme, examen ophtalmologique) peuvent être réalisés au diagnostic pour servir de référence. Depuis 2020, l’individualisation du VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome), caractérisé par la présence d’une mutation dans le gène UBA1, détectable dans le sang ou la moelle osseuse, a modifié la perception du sous-groupe de patients atteints de PCA et porteurs de myélodysplasie. Les patients porteurs de PCA dans le cadre d’un VEXAS, avec ou sans myélodysplasie, sont presque tous des hommes au-delà de 50 ans. Leur maladie est remarquable par la fréquence de la fièvre, des infiltrats pulmonaires, des signes cutanés, des manifestations thrombotiques, la rareté de l’atteinte trachéobronchique, un volume globulaire moyen>100 fL ou une numération plaquettaire<200 G/L et une résistance aux traitements.
Relapsing polychondritis (RP) is a rare inflammatory disease. The diagnosis is based on the evidence of inflamed cartilage, especially of the ears, nose, larynx and the tracheobronchial tractus. Non-cartilaginous organs can be involved, such as the aorta, the heart, the cochlea, the eyes, the skin and the central nervous system. C-reactive protein can be normal in RP, including in case of severe flare and there is no biological marker to help diagnosis. Cartilage biopsies should be avoided. Flow-volume loop and thoracic CT-scan (or MRI) with inspiratory and expiratory sequences are mandatory at diagnosis to detect airway involvement characterized by an obstructive syndrome and thickening with contrast enhancement of the cartilaginous portions of the larynx, trachea and/or bronchia, as well as malacia. The usefulness of PET-scanner is debated. Other examinations (echocardiography, audiometry, ophthalmological examination) are discussed at diagnosis to obtain reference values in case of future organ involvement. Since 2020, the individualization of VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome), characterized by the presence of a mutation in the UBA1 gene detectable in blood or bone marrow, has changed the perception of the subgroup of patients with PCA and myelodysplasia. Patients with PCA in the VEXAS setting, with or without myelodysplasia, are almost all men over 50 years of age. Their disease is remarkable for the frequency of fever, pulmonary infiltrates, skin involvement, thrombotic manifestations, rare tracheobronchial involvement and a mean globular volume >100 fL or a platelet count <200×109/L with frequent resistance to treatment.
Purpose
In the evolution of the minimally invasive treatment of vertebral compression fractures, vertebral body stenting (VBS) was developed to reduce intraoperative and secondary loss of vertebral ...height. Particularly in combination with the usage of biodegradable cement, the influence of VBS on the rate of intraoperative complications and long-term outcome is unclear. The purpose of this study was to investigate the differences between balloon kyphoplasty (BKP) and VBS regarding their long-term clinical and radiological outcome in combination with calcium phosphate (CaP) application instead of polymethyl methacrylate (PMMA).
Methods
This retrospective study included 49 patients with fresh mono-segmental thoracolumbar fractures without neurological signs treated with VBS or BKP and CaP cement (Calcibone). The outcome was evaluated with the visual analogue pain scale (VAS), the Oswestry disability score (ODI), and radiologically assessed.
Results
In the course of the radiological follow-up, the VBS group showed statistically significant less vertebral height loss than the BKP group. However, with respect to VAS and ODI scores there were no statistically significant differences between the VBS and BKP group in the clinical follow-up. The rate of cement leakage was comparable in both groups.
Conclusions
Both techniques facilitated good clinical results in combination with absorbable cement augmentation. In particular, the VBS enabled us to benefit from the advantages of the resorbable isothermic CaP cement with an improved radiological outcome in the long term compared to BKP. However, there was a mentionable loss of reduction in the follow-up in both groups compared to previously published data with PMMA cement.
Graphical abstract
These slides can be retrieved under Electronic Supplementary Material.
Les complications ischémiques de l’artérite à cellules géantes surviennent essentiellement chez des patients présentant une vascularite encore non diagnostiquée ou mal contrôlée par le traitement.
...Lorsque le traitement contrôle symptômes et signes biologiques, les complications sont généralement attribuées à l’athérosclérose.
Les complications ophtalmiques sont les plus fréquentes, dominées par la neuropathie optique ischémique antérieure aiguë.
Les accidents vasculaires cérébraux se localisent de façon privilégiée dans le territoire vertébral postérieur.
Les complications coronariennes par vascularite sont exceptionnelles.
Le diagnostic des complications ischémiques de membre bénéficie largement des progrès de l’imagerie (signe du halo à l’échographie, hypermétabolisme au TEP-scanner).
Le traitement repose sur la corticothérapie (initialement au moins 1mg/kg d’équivalent prednisone, précédé de bolus intraveineux selon les cas), le contrôle des facteurs de risque cardiovasculaire traditionnels et la prescription d’antiagrégant plaquettaire ; l’héparinothérapie est à envisager pour les complications ischémiques des membres.
GCA ischemic complications occur generally in patients with a yet undiagnosed or uncontrolled disease.
When disease control is fair, ischemic complications may be due mostly to atheromatosis.
Ophtalmic complications are most frequent and are dominated by anterior ischemic optic neuropathy.
Vasculitic strokes occur essentially in the vertebrobasilar arterial territory.
Overt vasculitic coronary disease is exceptional.
The diagnosis of upper and lower limbs ischemic complications benefit from advances in echography (halo sign) and positron emission tomography imaging.
Treatment relies on corticosteroids (initially 1mg/kg prednisone or more, preceded by intravenous methylprednisolone gigadoses if necessary), the control of cardiovascular risk factors and antiplatelet drugs; heparin may be indicated for threatening limbs ischemia.
In most patients with nonsevere systemic necrotizing vasculitides (SNVs), remission is achieved with glucocorticoids alone, but one-third experience a relapse within 2 years. This study was ...undertaken to determine whether the addition of azathioprine (AZA) to glucocorticoids could achieve a higher sustained remission rate of newly diagnosed nonsevere eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), microscopic polyangiitis (MPA), or polyarteritis nodosa (PAN).
All patients included in this double-blind trial received glucocorticoids, gradually tapered over 12 months, and were randomized to receive AZA or placebo for 12 months, with stratification according to SNV (EGPA or MPA/PAN). The primary end point was the combined rate of remission induction failures and minor or major relapses at month 24.
Ninety-five patients (51 with EGPA, 25 with MPA, and 19 with PAN) met the inclusion criteria, were randomized, and received at least 1 dose of AZA (n = 46) or placebo (n = 49). At month 24, 47.8% of the patients receiving AZA versus 49% of the patients receiving placebo had remission induction failures or relapses (P = 0.86). Secondary end points were comparable between the AZA and placebo arms. These included initial remission rate (95.7% versus 87.8%), total relapse rate (44.2% versus 40.5%), and glucocorticoid use. Two patients in the placebo arm died; 22 patients in the AZA arm (47.8%) and 23 patients in the placebo arm (46.9%) experienced ≥1 severe adverse event. For EGPA patients, the primary end point (48% in the AZA arm versus 46.2% in the placebo arm) and the percent of patients who experienced asthma/rhinosinusitis exacerbations (24% in the AZA arm versus 19.2% in the placebo arm) were comparable between treatment arms.
Addition of AZA to glucocorticoids for the induction of remission of nonsevere SNVs does not improve remission rates, lower relapse risk, spare steroids, or diminish the EGPA asthma/rhinosinusitis exacerbation rate.
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