29 research outputs found
POS0250 SIGNIFICANT DAMAGE OCCURS EARLY IN THE COURSE OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS AND IS MAINLY DUE TO DISEASE-RELATED SEQUELAE
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 declare
AB0462 BEHCET'S DISEASE: CLINICAL FEATURES AND OFF-LABEL BIOLOGIC TREATMENT STRATEGIES
Background:The treatment of Behçet's disease (BD) is still mainly based on the evidence derived from case reports, case series, retrospective analyses, and few clinical trials suggesting the safety and potential efficacy of off-label use of biologic agents in refractory cases.1Objectives:To describe clinical manifestations and their management, with particular focus on treatment indications, outcomes and safety of biologic therapy, in a cohort of patients with BD.Methods:Patients with a diagnosis of BD who visited our outpatient clinic until December 2019 were included in the study. Clinical data were recorded since diagnosis until the latest follow-up visit, analyzing clinical features, flares and therapeutic strategies adopted.Results:A total of 95 patients were included in the study with a medium follow-up of 108.54 ± 169.59 months. 20 of them (21. 05%) were treated with biologic agents. Patients treated with biologic therapy compared to those on conventional non-biologic therapies had a higher proportion of musculoskeletal (80% vs 46.67%, p = 0.008), neurological (30% vs 10.67%, p = 0.031), intestinal involvement (40% vs 12%, p = 0.004), and they were treated with a higher dose of glucocorticoids at diagnosis (16.84 mg ±14.01 vs 8.89 mg ± 11.76, p = 0.012). The most frequent indications for biologic step-up therapy were musculoskeletal involvement (40%), eye involvement (25%), neurological involvement (15%) and intestinal involvement (10%). Most patients initiated a biologic treatment within the first year of follow-up. TNF-inhibitor (TNFi) were more frequently prescribed (95%) and one patient was treated with 8 therapeutic cycles of Rituximab (500 mg/weekly for 4 infusions to be repeated after at least 6 months) because of recurrent pancytopenia. All patients experienced non-biologic therapy before starting a TNFi. The preferred first-line TNFi was infliximab (50%), followed by adalimumab (40%) and etanercept (5%). As second line treatment were also prescribed certolizumab (10%) and golimumab (5%). 10 patients switched to a second line treatment because of inefficacy of the first biologic agent, mainly because of refractory arthritis, intestinal and mucocutaneous involvement. One patient switched from infliximab to certolizumab during pregnancy with subsequent worsening of arthritis.85% of patients treated with biologic agents reached a clinical remission by the time of the latest follow up visit without any safety or tolerability issues.Conclusion:A relevant proportion of patients in our BD cohort were treated with biologic therapy, because of severe or refractory manifestations. The most frequent indications were musculoskeletal, neurological or intestinal involvement. Biologic agents were a generally effective and safe therapeutic approach.References:[1]F. Alibaz-Oner, M. H. Sawalha, H. Direskeneli. Management of Behçet disease, Curr. Opin. Rheumatol, 2018Table 1.General characteristics and disease involvement at diagnosisBiologic therapyNo biologic therapyp value20 (21.05%)75 (78.95%)General characteristicsMediaSDMediaSDAge at disease onset(years ± SD)34.5± 10.4938.64± 13.18p = 0.1976Diagnostic delay(months ± SD)45.28± 67.4828.09± 48.42p = 0.1996Glucocorticoids at diagnosis (mg prednisone ± SD)16.84± 14.018.89± 11.76p = 0.0115Glucocorticoids at latest follow up visit (mg prednisone ± SD)6.38± 7.763.83± 4.81p = 0.0707N%N%F / M12 / 860 / 4054 / 4172 / 28p = 0.3030Disease involvement at diagnosisOral ulcers2010075100Genital ulcers11553749,33p = 0.6540Cutaneous lesions15755066,67p = 0.4787Eye involvement6302736p = 0.6184Musculoskeletal involvement16803546,67p = 0.0082Neurological involvement630810,67p = 0.0311Intestinal involvement840912p = 0.0039Thrombosis2101824p = 0.1747Disclosure of Interests:None declare
Bacterial isolation in samples of goat milk.
The aim of this study was to monitor the presence of pathogens causing caprine mastitis during three months in a properties situated in the region of the Zona da Mata of Minas Gerais. The presence of isolated microorganisms of goat milk stresses the importance of prophylaxis measures and control of subclinical mastitis in the flock. That?s because the isolated microorganisms exhibit great importance for public health, as they are possible producers of staphylococcal toxin, and therefore, possible causes of food poisoning
Diffuse Alveolar Hemorrhage
This article is made available for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic.Diffuse alveolar hemorrhage[DAH] is a serious condition that can be life threatening. It can be caused by a constellation of disorders which presents with hemoptysis, anemia, and diffuse alveolar infiltrates. Respiratory failure from DAH can be so severe that it has been called an ARDS mimic/imitator. Early recognition is crucial because prompt diagnosis and treatment are required for survival. DAH should be distinguished from other causes of pulmonary hemorrhage caused by localized pulmonary abnormalities and the bronchial circulation. Early bronchoscopy with bronchoalveolar lavage (BAL) is generally required to confirm the diagnosis of DAH and rule out infection. Progressively bloody bronchoalveolar lavage samples can distinguish DAH. Systemic vasculitis is one of the most common causes of DAH and can be pathologically defined by the presence of cellular inflammation, vessel destruction, tissue necrosis, and eventually, organ dysfunction. Corticosteroids and immunosuppressive agents remain the gold standard for the treatment.
The following case illustrates a patient who was dependent on dialysis, then presented with hemoptysis. Bronchoscopy demonstrated progressively bloody bronchoalveolar lavage samples consistent with diffuse alveolar hemorrhage. Serologic testing was consistent with microscopic polyangiitis. The patient experienced a clinical remission with cyclophosphamide and corticosteroids
Prevalence and incidence of osteoporotic fractures in patients on long-term glucocorticoid treatment for rheumatic diseases: The glucocorticoid induced OsTeoporosis TOol (GIOTTO) study
Osteoporosis and fractures are common and invalidating consequences of chronic glucorticoid (GC) treatment. Reliable information regarding the epidemiology of GC induced osteoporosis (GIOP) comes exclusively from the placebo group of randomized clinical trials while observational studies are generally lacking data on the real prevalence of vertebral fractures, GC dosage and primary diagnosis. The objective of this study was to evaluate the prevalence and incidence of osteoporotic fractures and to identify their major determinants (primary disease, GC dosage, bone mineral density, risk factors, specific treatment for GIOP) in a large cohort of consecutive patients aged >21 years, on chronic treatment with GC ( 655 mg prednisone - PN - equivalent) and attending rheumatology centers located all over Italy. Glucocorticoid Induced OsTeoporosis TOol (GIOTTO) is a national multicenter cross-sectional and longitudinal observational study. 553 patients suffering from Rheumatoid Arthritis (RA), Polymyalgia Rheumatica (PMR) and Connective Tissue Diseases (CTDs) and in chronic treatment with GCs were enrolled. Osteoporotic BMD values (T score <-2.5) were observed in 28%, 38% and 35% of patients with CTDs, PMR or RA at the lumbar spine, and in 18%, 29% and 26% at the femoral neck, respectively. Before GC treatment, prevalent clinical fractures were reported by 12%, 37% and 17% of patients with CTDs, PMR, or RA, respectively. New clinical fragility fractures during GC treatment were reported by 12%, 10% and 23% of CTDs, PMR and RA patients, respectively. Vertebral fractures were the prevailing type of fragility fracture. More than 30% of patients had recurrence of fracture. An average of 80% of patients were in supplementation with calcium and/or vitamin D during treatment with GCs. Respectively, 64%, 80%, and 72% of the CTDs, PMR and RA patients were on pharmacological treatment for GIOP, almost exclusively with bisphosphonates. The GIOTTO study might provide relevant contributions to clinical practice, in particular by highlighting and quantifying in real life the prevalence of GIOP and relative fractures, the frequency of the main risk factors, and the currently sub-optimal prevention. Moreover, these results emphasize the importance of the underlying rheumatic disease on the risk of GIOP associated fractures
Fast-Track Ultrasound Clinic for the Diagnosis of Giant Cell Arteritis Changes the Prognosis of the Disease but Not the Risk of Future Relapse
Background: Color Duplex sonography (CDS) of temporal arteries and large vessels (LV) is a recently validated diagnostic methodology for Giant Cell Arteritis (GCA). CDS combined with a fast-track approach (FTA) has improved the early diagnosis of the disease. Objectives: To assess FTA effects on the prevention of permanent visual loss (PVL), relapse and late complications of GCA compared to conventional practice. To assess the impact of COVID-19 pandemic on outcomes of GCA patients assessed with FTA. Methods: GCA patients diagnosed up to June 2020 at the Rheumatology Department, University of Pavia, were included. FTA was implemented since October 2016. FTA consists in the referral within 1 working day of a suspected GCA case to an expert rheumatologist who performs clinical evaluation and CDS. Results: One hundred sixty patients were recruited [female 120 (75%), mean age 72.4 ± 8.2 years]. Sixty-three (39.4%) evaluated with FTA, 97 (60.6%) with conventional approach. FTA patients were older (75.1 ± 7.6 vs. 70.6 ± 8.2 years old; p < 0.001). Median follow-up duration was shorter in the FTA group compared to the conventional one (0.9 vs. 5.0 years; p < 0.001). There was no difference between the two cohorts regarding major vessel district involvement (LV-GCA 17.5% vs. 22.7%; p = 0.4). PVL occurred in 8 (12.7%) FTA patients and 26 (26.8%) conventional ones (p = 0.03). The relative risk of blindness in the conventional group was 2.11 (95% C.I. 1.02–4.36; P = 0.04) as compared to FTA. Median symptom latency of patients experiencing PVL was higher in the conventional group (23 days IQR 12–96 vs. 7 days IQR 4–10, p = 0.02). During COVID-19 there was a significant increase in the occurrence of PVL (40%) including bilateral blindness despite a regularly operating FTA clinic. Cumulative incidence of relapses and time to first relapse did not change after FTA introduction (P = 0.2). No difference in late complications (stenosis/aneurysms) was detected. Conclusions: FTA including CDS evaluation contributed to a substantial reduction of PVL in GCA by shortening the time to diagnosis and treatment initiation. Relapse rate did not change upon FTA introduction, highlighting the need for better disease activity monitoring and treatment strategies optimization based on risk stratification that would predict the occurrence of relapse during glucocorticoid de-escalation
Cobalt hip prosthesis intoxication mimicking an autoimmune disease
none6noLes prothèses de hanche à couple de frottement métal-métal contenant du cobalt peuvent être à l'origine d'une toxicité systémique provoquée par le relargage d'ions cobalt dans la circulation sanguine. Des taux élevés de cobalt dans le sang peuvent provoquer diverses manifestations cliniques imitant d'autres maladies, notamment auto-immunes, hématologiques et infectieuses. Nous rapportons un cas d'intoxication au cobalt à partir d'une prothèse de hanche, imitant une maladie auto-immune avec des signes d'inflammation systémique, des douleurs articulaires et musculaires sans synovite et la positivité de plusieurs auto-anticorps. Une femme de 69 ans présentait depuis un an une douleur de hanche droite, une fièvre récurrente, des arthralgies et myalgies, des adénopathies médiastinale et iliaque droite. Elle avait une prothèse de hanche depuis sept ans. L'examen physique était normal, à l'exception d' une douleur de la hanche droite. Les analyses biologiques ont révélé une nette élévation des marqueurs de l'inflammation et tous les tests microbiologiques étaient négatifs. Une ponction articulaire guidée par échographie de la hanche droite a permis de prélever un liquide limpide dont la mise en culture s'est avérée négative. Des taux élevés de cobalt dans le plasma et les urines indiquaient la présence d'une intoxication à ce métal. L'imagerie par résonance magnétique en séquence de réduction des artéfacts métalliques (MARS) a confirmé la présence d'une masse périprothétique caractéristique d'une réaction à des débris métalliques. Les manifestations cliniques et les taux de cobalt se sont normalisés après le remplacement de la prothèse.restrictedBiglia A.; Morandi V.; Monti S.; Delvino P.; Cavagna L.; Montecucco C.Biglia, A.; Morandi, V.; Monti, S.; Delvino, P.; Cavagna, L.; Montecucco, C
Cobalt hip prosthesis intoxication mimicking an autoimmune disease
Cobalt-containing hip prosthesis may cause systemic toxicity due to the release of cobalt from metal-on-metal (MoM) joint arthroplasty into the bloodstream. High cobalt blood levels can lead to a variety of clinical manifestations, mimicking other disorders, especially autoimmune, hematologic, and infectious diseases. Our purpose is to describe a clinical case of cobalt hip prosthesis intoxication mimicking an autoimmune disease, with systemic inflammation signs, arthro-myalgias unrelated to overt synovitis, and multiple autoantibody positivity. A 69-years-old woman presented with a 1-year history of right coxalgia, recurrent fever, arthro-myalgias, mediastinal and right iliac reactive lymphadenopathy. She underwent hip replacement surgery seven years earlier. The physical examination was unremarkable except for right hip pain. Laboratory tests showed markedly increased inflammatory indices and microbiological tests were all negative. Ultrasound-guided arthrocentesis of right hip yielded limpid fluid with negative cultures. Increased cobalt levels in plasma and urine showed metal intoxication. Magnetic resonance imaging with metal artifact reduction sequence (MARS) confirmed a periprosthetic mass as usually seen in reaction to metal debris. Prosthesis substitution was performed with a resolution of the clinical picture and normalization of cobalt levels
Impact of delayed diagnoses at the time of COVID-19: Increased rate of preventable bilateral blindness in giant cell arteritis
The role of extra-corporeal membrane oxygenation (ECMO) in the treatment of diffuse alveolar haemorrhage secondary to ANCA-associated vasculitis : report of two cases and review of the literature
Diffuse alveolar haemorrhage (DAH) secondary to anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a rare life-threatening condition presenting with severe respiratory failure. The management of AAV-related DAH consists of remission induction immunosuppressive therapy, which requires time to be effective, with significant fatality rates despite appropriate treatment. Extracorporeal membrane oxygenation (ECMO) can support gas exchanges providing the time necessary for immunosuppressive treatment to control the underlying disease in cases refractory to the conventional ventilation techniques. Despite severe preexisting bleeding has been considered a relative contraindication, ECMO has proven to be life-saving in several cases of respiratory failure associated with pulmonary haemorrhage due to various causes, including AAV. We reviewed the clinical presentation and course of two patients affected by AAV-related DAH treated at our Institution between 2012 and 2017, whose management required the use of veno-venous ECMO. We reviewed the current literature on the role of ECMO in the support of these patients. In both patients, ECMO provided life support and allowed disease control, in combination with immunosuppressive treatment. Despite systemic anticoagulation, clinical improvement was achieved without exacerbation of the pulmonary bleeding. We performed a literature review, and summarized available data confirming the effectiveness and safety of ECMO in AAV-related DAH. ECMO has a life-saving role in the management of patients with severe respiratory failure due to ANCA-associated pulmonary capillaritis