scholarly journals French recommendations for the management of systemic necrotizing vasculitides (polyarteritis nodosa and ANCA-associated vasculitides)

2020 ◽  
Vol 15 (S2) ◽  
Author(s):  
Benjamin Terrier ◽  
◽  
Raphaël Darbon ◽  
Cécile-Audrey Durel ◽  
Eric Hachulla ◽  
...  

AbstractSystemic necrotizing vasculitis comprises a group of diseases resembling polyarteritis nodosa and anti-neutrophil cytoplasmic antibody-associated vasculitis (ANCA): granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis. The definitive diagnosis is made in cooperation with a reference center for autoimmune diseases and rare systemic diseases or a competency center. The management goals are: to obtain remission and, in the long term, healing; to reduce the risk of relapses; to limit and reduce the sequelae linked to the disease; to limit the side effects and the sequelae linked to the treatments; to improve or at least maintain the best possible quality of life; and to maintain socio-professional integration and/or allow a rapid return to school and/or professional activity. Information and therapeutic education of the patients and those around them are an integral part of the care. All health professionals and patients should be informed of the existence of patient associations. The treatment of vasculitis is based on variable combinations of glucocorticoids and immunosuppressants, chosen and adapted according to the disease concerned, the severity and/or extent of the disease, and the underlying factors (age, kidney function, etc.). Follow-up clinical and paraclinical examinations must be carried out regularly to clarify the progression of the disease, detect and manage treatment failures and possible relapses early on, and limit sequelae and complications (early then late) related to the disease or treatment. A distinction is made between the induction therapy, lasting approximately 3–6 months and aimed at putting the disease into remission, and the maintenance treatment, lasting 12–48 months, or even longer. The role of the increase or testing positive again for ANCA as a predictor of a relapse, which has long been controversial, now seems to have greater consensus: Anti-myeloperoxidase ANCAs are less often associated with a relapse of vasculitis than anti-PR3 ANCA.

Author(s):  
Ashish Kumar Dixit ◽  
Nibha Giri ◽  
Shishir Singh

Abstract Objectives The long-term consequences of COVID-19 survivors care and post-coronavirus infection are not yet well understood. The review aims to see whether homoeopathy can help COVID-19 survivors recover from its consequences and improve their quality of life. Content A systematic search of published articles for post-COVID sequelae and the impact of Homoeopathy were conducted. For the literature search, the major electronic bio-medical database PubMed/MEDLINE was used. In addition, supplementary searches were conducted through the references of those published articles. Summary A total of 113 records were identified of which 61 studies included for this review. Homoeopathy is effective in the treatment of mental disorders including anxiety and depressive disorder (ADD), some research studies have found, although systematic reviews disagree. Likewise, some medical societies denounce homoeopathy for pain management; other literature shows that it can be used to treat pain effectively. Homoeopathy can aid in the treatment of cardiovascular diseases, as Crataegus, a homoeopathic medication, was found to be just as effective as a standard angiotensin-converting enzyme (ACE) inhibitor and diuretic treatment for minor cardiac insufficiency. The outcomes for Chronic Fatigue Syndrome (CFS), Influenza, and Acute Respiratory Tract Infections (ARTIs) are also promising. Outlook Based on the results of homoeopathy in non-COVID conditions, it can be thought of in the management of post-COVID-19 outcomes. Consequently, we propose that while investigating post-COVID-19 patient rehabilitation, homoeopathic management may be included as part of the follow-up route and as much data as possible in the context of homoeopathy should be collected, so that in future, the role of homoeopathy in dealing with it can be better demonstrated.


Rheumatology ◽  
2019 ◽  
Vol 58 (12) ◽  
pp. 2107-2116 ◽  
Author(s):  
Xavier Puéchal ◽  
Christian Pagnoux ◽  
Gabriel Baron ◽  
François Lifermann ◽  
Loïk Geffray ◽  
...  

Abstract Objective In a previous controlled trial, 1-year adjunction of AZA to glucocorticoids (GC) for patients with non-severe, newly diagnosed eosinophilic granulomatosis with polyangiitis (EGPA) failed to lower remission failure, vasculitis relapse and isolated asthma/rhinosinus exacerbation rates, or cumulative GC use at month (M) 24. The aim of this study was to analyse longer-term outcomes to determine whether subsequent vasculitis relapse or isolated asthma/rhinosinus exacerbation (IARE) rates differed. Methods After M24, patients were followed prospectively, being treated based on physicians’ best judgment. Flares and reasons for increased GC dose or immunosuppressant use were recorded, and reviewed according to randomization group to distinguish vasculitis relapses from IAREs according to EGPA Task Force recommendations. Results Fifty EGPA trial participants were followed for a median (interquartile range) of 6.3 (5.4–7.6) years; two (4%) died 11 months post-inclusion. By M24, vasculitis had relapsed in 21/49 (43%) patients and 14/50 (28%) had IAREs. Another patient died 4.8 years post-inclusion (infection). Among nine patients with subsequent vasculitis relapses, three had a major relapse and three had their first relapse after M24; among 25 patients with later IAREs, 17 occurred after M24. At 5 years, respective vasculitis relapse and IARE rates were 48% (95% CI 34.0, 62.6) and 56% (95% CI 41.7, 70.8), with no between-arm differences (P = 0.32 and 0.13). No entry clinical or biological parameter was associated with these outcomes during follow-up. Conclusion These results confirmed that 1-year AZA and GC induction obtained good overall survival but no long-term benefit for non-severe EGPA patients. Vasculitis relapses, occurring mostly during the first 2 years, and IAREs, occurring throughout follow-up, require other preventive treatments. Trial registration ClinicalTrials.gov, https://clinicaltrials.gov, NCT00647166.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 347.1-347
Author(s):  
P. Delvino ◽  
A. Milanesi ◽  
F. Brandolino ◽  
S. Monti ◽  
C. Montecucco

Background:Following the introduction of effective immunosuppressive treatments, ANCA-associated vasculitides (AAV) have become chronic diseases with a remitting-relapsing course. Therefore, preventing chronic damage accrual during follow-up is critical, as relapses, treatment-related side effects, and comorbidities may significantly affect the long-term outcomes of AAV patients. At present, no study specifically evaluated the burden of damage in patients with eosinophilic granulomatosis with polyangiitis (EGPA).Objectives:To describe short-term (6 months) and long-term (5 years) damage accrual in patients with newly diagnosed EGPA.Methods:Patients diagnosed with EGPA, according to ACR criteria and/or Chapel Hill definitions and regularly followed-up in our vasculitis center for ≥5 years were included. Damage accrual was assessed with the Vasculitis Damage Index (VDI). Short-term and long-term damage accrual was defined by VDI at 6 months and at 5 years, respectively, and categorized as related to vasculitis or its treatment.Results:VDI data at 6 months were available for 45 EGPA patients: 24 (53.3%) female, mean age at diagnosis 51.6±13.0 years. ANCA were positive in 17 patients (37.8%), with MPO being the only detected enzyme immunoassay (EIA)-specificity. At 6 months mean VDI was 2.8±1.3; 25/45 (55.6%) and 6/45 patients (13.3%) presented ≥3 and ≥5 items, respectively, whilst only 1 patient (2.2%) showed no items of damage. VDI data at 5 years were available for 32/45 EGPA patients (71.1%): 16 (50%) female, mean age at diagnosis 51.5±13.1 years. MPO-ANCA were positive in 13 patients (40.6%). At 5 years mean VDI was 3.5±1.3, with 26/32 (81.3%) and 7/32 patients (21.9%) presenting ≥3 and ≥5 items, respectively; notably, no patients presented a VDI=0 at 5 years.The most frequent disease-related VDI items at 6 months and at 5 years were asthma, chronic sinusitis, peripheral neuropathy, cardiomyopathy, pulmonary function tests abnormalities and nasal blockage (Figure 1). Osteoporotic fractures, diabetes and systemic hypertension were the most commonly reported treatment-related items at 6 months and at 5 years (Figure 1). Damage accrual progressively rose during the 5-year follow-up (P=0.023), mainly due to disease-related items rather than treatment-related items both at 6 months (disease related VDI 2.6±1.2, treatment-related VDI 0.3±0.6) and at 5 years (disease related VDI 2.9±1.2, treatment-related VDI 0.6±0,7). No significant difference in terms of damage accrual was observed between ANCA-positive and ANCA-negative patients (P >0.5).Conclusion:In our cohort of EGPA patients damage accrual occurs early, with more than half of the patients displaying ≥3 VDI items already at 6 months. Poor control of previous disease activity, particularly ENT and respiratory manifestations, contributes to progressive damage accrual more than treatment side effects.Figure 1.Disease-related and treatment-related VDI items at 6 months and at 5 years in patients with EGPA.Disclosure of Interests:None declared


2021 ◽  
Vol 2 (4) ◽  
Author(s):  
Husam Al sultani ◽  
Komal Hafeez ◽  
Muhammed Hafeez ◽  
Aziz Shaibani

Background: While the role of telemedicine is well established in certain fields of medicine, its role in disciplines like Neuromuscular medicine is not clear. COVID 19 pandemic compelled the medical community to utilize telemedicine and policies were rapidly changed to continue patient care during the pandemic. However, to guide the future of telemedicine in this field where a physical exam is an integral part of the visit, it is imperative to get a physician's opinion on this matter. We designed this study to assess the opinion of neuromuscular physicians about telemedicine, their preference, and factors influencing their decision. Methods: We used an online form composed of eleven questions to survey 94 neuromuscular specialists across the USA and Canada during September 2020. Results:  90.43% of participating neuromuscular specialists preferred physical visits with new patients versus 44.68% preferred physical visits with follow-up patients. The majority thought that telemedicine reduces revenue (58.51%), quality of service (57.45%), and quality time spent with patients (62.77%). Nevertheless, most surveyed physicians agreed that telemedicine is time-efficient (84.04%), improves patient compliance (70.21%), and will be a long-term solution in clinical practice (67.02%). Finally, 58.51% revealed that telemedicine does not affect workload. Conclusion: Neuromuscular specialists preferred seeing new patients and revealing a new diagnosis to the patient in physical visits, but they also considered telemedicine a long-term method that would continue to increase in the post-pandemic future, emphasizing the need to address their concerns to facilitate telemedicine.   


2021 ◽  
pp. 2101404
Author(s):  
Brigitte Fauroux ◽  
François Abel ◽  
Alessandro Amaddeo ◽  
Elisabetta Bignamini ◽  
Elaine Chan ◽  
...  

Long term noninvasive respiratory support, comprising continuous positive airway pressure (CPAP) and noninvasive ventilation (NIV), in children is expanding worldwide, with increasing complexities of children being considered for this type of ventilator support and expanding indications such as palliative care. There have been improvements in equipment and interfaces. Despite growing experience, there are still gaps in a significant number of areas: there is a lack of validated criteria for CPAP/NIV initiation, optimal follow-up and monitoring; weaning and long term benefits have not been evaluated. Therapeutic education of the caregivers and the patient is of paramount importance, as well as continuous support and assistance, in order to achieve optimal adherence. The preservation or improvement of the quality of life of the patient and caregivers should be a concern for all children treated with long term CPAP/NIV. As NIV is a highly specialised treatment, patients are usually managed by an experienced pediatric multidisciplinary team. This Statement written by experts in the field of pediatric long term CPAP/NIV aims to emphasize on the most recent scientific input and should open up to new perspectives and research areas.


Nutrients ◽  
2020 ◽  
Vol 12 (4) ◽  
pp. 1060
Author(s):  
Anna Alakoski ◽  
Kaisa Hervonen ◽  
Eriika Mansikka ◽  
Timo Reunala ◽  
Katri Kaukinen ◽  
...  

The treatment of choice for dermatitis herpetiformis (DH), a cutaneous manifestation of coeliac disease, is a life-long gluten-free diet (GFD). In a GFD, wheat, rye and barley should be strictly avoided, but the role of oats is more controversial. This study aimed to investigate the safety and long-term quality of life and health effects of oat consumption in 312 long-term treated DH patients. Baseline data were gathered from patient records and follow-up data from questionnaires or interviews, and validated questionnaires were used to assess quality of life. We found that altogether 256 patients (82%) were consuming oats as part of their GFD at the follow-up. Long-term follow-up data showed that there were no differences in the presence of long-term illnesses, coeliac disease complications or the usage of medication between those consuming and not consuming oats. However, oat consumers had a better quality of life and reported ongoing gastrointestinal symptoms less frequently (4% vs 19%, p = 0.004) at the follow-up than those not consuming oats. The study established that oats are safe for DH patients and in the long-term seem to improve the quality of life of DH patients.


2013 ◽  
Author(s):  
Francesca Menegazzo ◽  
Melissa Rosa Rizzotto ◽  
Martina Bua ◽  
Luisa Pinello ◽  
Elisabetta Tono ◽  
...  

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