scholarly journals Juvenile idiopathic arthritis-associated uveitis in the era of biological therapy: how the disease changed in more than 20 years of observation in a tertiary referral center in Rome (Italy)

2021 ◽  
Author(s):  
E. Del Giudice ◽  
C. Simio ◽  
A. Scala ◽  
A. Di Coste ◽  
G. La Torre ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Biological Therapy ◽  
Ocular Complications ◽  
Tertiary Referral Center ◽  
The Past ◽  
Systemic Corticosteroids ◽  
Band Keratopathy ◽  
Current Therapies ◽  
Disease Modifying ◽  
Care Improvement

Abstract Objectives To describe the ophthalmological characteristics in a Juvenile idiopathic arthritis (JIA) cohort and to evaluate how therapeutic advances have changed the course of the uveitis. Methods Analysis of a retrospective cohort study of consecutive JIA pediatric patients including JIA-associated uveitis (JIA-U) and comparison with a previous study in the same uveitis center assessed before the wide-spread of biological therapy. Results The total of 49 JIA patients were analyzed, of whom 18 JIA-U, compared with a JIA-U past cohort of 66 patients. Systemic corticosteroids were used significantly less in the current JIA-U group (p = 0.008) than in the past one. JIA-U present cohort was on therapy more frequently with conventional synthetic disease-modifying anti-rheumatic drugs (csDMARDs) than the past group (p = 0.039), mostly treated with methotrexate (93.3%). Furthermore, a larger use of biologic disease-modifying anti-rheumatic drugs (bDMARDs) was described in the current JIA-U group (p = 0.005) also associated with csDMARDs (p = 0.003). Adalimumab was used more (72.7%) in the present JIA-U cohort compared to a larger treatment with infliximab (61.5%) in the past (p = 0.005). Higher number of uveitis recurrences was observed in the previous cohort compared to the current one (p = 0.005). Fewer complications were described in this study than in the previous: posterior synechiae (p = 0.007), cataract (p < 0.001), band keratopathy (p < 0.001), and elevated intraocular pressure (IOP) (p = 0.047). Conclusion Current therapies reduced the uveitis recurrences and ocular complications including cataract due also to the lower use of corticosteroids. The new close collaboration with the pediatric rheumatologic center in the same University has contributed to the care improvement and decrease of uveitis complications.

scholarly journals Juvenile idiopathic arthritis-associated uveitis in the era of biological therapy: how the disease changed in 14 years of observation in a tertiary referral center in Rome (Italy)

2021 ◽  
Author(s):  
Emanuela Del Giudice ◽  
Claudia Simio ◽  
Andrea Scala ◽  
Annalisa Di Coste ◽  
Giuseppe La Torre ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Biological Therapy ◽  
Ocular Complications ◽  
Tertiary Referral Center ◽  
The Past ◽  
Systemic Corticosteroids ◽  
Band Keratopathy ◽  
Current Therapies ◽  
Disease Modifying ◽  
Care Improvement

Abstract Objectives To describe the ophthalmological characteristics in a Juvenile idiopathic arthritis (JIA) cohort and to evaluate how therapeutic advances have changed the course of the uveitis. Methods Analysis of a retrospective cohort study of consecutive JIA pediatric patients including JIA-associated uveitis (JIA-U) and comparison with a previous study in the same uveitis center assessed before the wide-spread of biological therapy. Results 49 JIA patients analyzed, of whom 18 JIA-U, compared with a JIA-U past cohort of 66 patients. Systemic corticosteroids were used significantly less in the current JIA-U group (p = 0.008) than in the past one. JIA-U present cohort were on therapy more frequently with conventional synthetic disease-modifying anti-rheumatic drugs (csDMARDs), than the past group (p = 0.039), mostly treated with methotrexate (93.3%). Furthermore, a larger use of biologic disease-modifying antirheumatic drugs (bDMARDs) was described in the current JIA-U group (p = 0.005) also associated with csDMARDs (p = 0.003). Adalimumab was used more (72.7%) in the present JIA-U cohort compared to a larger treatment with infliximab (61.5%) in the past (p = 0.005). Higher number of uveitis recurrences was observed in the previous cohort compared to the current one (p = 0.005). Fewer complications were described in this study than in the previous: posterior synechiae (p = 0.007), cataract (p < 0.001), band keratopathy (p < 0.001), elevated intraocular pressure (IOP) (p = 0.047) Conclusion. Current therapies reduced the uveitis recurrences and ocular complications including cataract due also to the lower use of corticosteroids. The new close collaboration with the pediatric rheumatologic center in the same University has contributed to the care improvement and decrease of uveitis complications.

Principles of management of juvenile idiopathic arthritis

2013 ◽  
pp. 725-733 ◽  
Author(s):  
Nicolino Ruperto ◽  
Angelo Ravelli
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Disease Control ◽  
Physical Therapist ◽  
Well Being ◽  
Standards Of Care ◽  
Pharmacological Interventions ◽  
American College Of Rheumatology ◽  
Systemic Corticosteroids ◽  
Biologic Response ◽  
Disease Modifying

The management of juvenile idiopathic arthritis (JIA) is based on a combination of pharmacological interventions, physical and occupational therapy, and psychosocial support. Ideally, the management is conducted by a multidisciplinary team composed by a paediatric rheumatologist, specialist nurse, physical therapist, occupational therapist, and psychologist. The treatment is aimed to achieve disease control, to relieve pain, to foster normal nutrition and growth, to maintain physical and psychological well-being, and to prevent long-term damage related to the disease or its therapy. First-line pharmacological interventions are based on non-steroidal anti-inflammatory drugs and intra-articular corticosteroids. Patients who are refractory to these therapies are candidates to receive disease-modifying anti-rheumatic medications, namely methotrexate or, in case of enthesitis-related arthritis, sulfasalazine. If therapeutic response is inadequate or suboptimal, the introduction of a biologic response modifier is considered. Systemic corticosteroids are used in selected instances, which include the management of extra-articular manifestations of systemic arthritis or the achievement of quick disease control while are awaiting the full therapeutic effect of a disease-modifying agent in patients with severe polyarthritis. To help physician select the safest and most effective treatment for JIA, the American College of Rheumatology (ACR) has issued a set of recommendations that were meant to be as evidence based as possible. The British Society for Paediatric and Adolescent Rheumatology (BSPAR) has developed the standards of care for patients with JIA, which are aimed to help the paediatric rheumatology teams to improve the service they provide.

Abatacept in the Treatment of Severe, Longstanding, and Refractory Uveitis Associated with Juvenile Idiopathic Arthritis

2015 ◽  
Vol 42 (4) ◽  
pp. 706-711 ◽  
Author(s):  
Christoph Tappeiner ◽  
Elisabetta Miserocchi ◽  
Bahram Bodaghi ◽  
Kaisu Kotaniemi ◽  
Friederike Mackensen ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Immunosuppressive Treatment ◽  
Primary Outcome Measure ◽  
Secondary Outcome ◽  
Ocular Complications ◽  
Systemic Corticosteroids ◽  
Before And After ◽  
Active Arthritis ◽  
Factor Α ◽  
Antigen Presenting

Objective.Abatacept (ABA), a selective T cell costimulation modulator that binds to CD80 and CD86 on antigen-presenting cells, was investigated for its antiinflammatory effect in treating severe chronic uveitis associated with juvenile idiopathic arthritis (JIA).Methods.Our retrospective study was conducted by members of the Multinational Interdisciplinary Working Group for Uveitis in Childhood (MIWGUC). Patients with JIA who are receiving ABA treatment for active uveitis were included. In all patients, uveitis had been refractory to previous topical and systemic corticosteroids, immunosuppressives, and at least 1 tumor necrosis factor–α inhibitor. A standardized protocol was used to document uveitis (MIWGUC) and arthritis. Baseline visit and visits at 3, 6, 9, and 12 months before and after ABA start were evaluated. Primary outcome measure was defined as achievement of uveitis inactivity; secondary outcome measures were tapering of corticosteroid and/or immunosuppressive treatment, and occurrence of complications.Results.In all, 21 patients (16 female) with active uveitis (n = 21) and arthritis (n = 18) were included (mean age 11.8 ± 3.6 yrs). In 7 of 18 patients with active arthritis at baseline, inactivity was achieved following ABA treatment. Uveitis inactivity was achieved in 11 patients, but recurred later in 8 of them, and remained active in another 10 cases. Systemic corticosteroids or immunosuppression were tapered in 3 patients, but uveitis recurred in all of them during further followup. Ocular complications secondary to uveitis were present in 17 patients at baseline, while 3 patients developed new ocular complications during followup.Conclusion.A sustained response to ABA was uncommon in patients with severe and refractory uveitis.

scholarly journals Use of Drugs for ATTRv Amyloidosis in the Real World: How Therapy Is Changing Survival in a Non-Endemic Area

2021 ◽  
Vol 11 (5) ◽  
pp. 545
Author(s):  
Massimo Russo ◽  
Luca Gentile ◽  
Vincenzo Di Stefano ◽  
Gianluca Di Bella ◽  
Fabio Minutoli ◽  
...  
Keyword(s):  
Medical Records ◽  
Early Stage ◽  
Symptomatic Treatment ◽  
New Drugs ◽  
Familial Amyloidotic Polyneuropathy ◽  
The Past ◽  
Disease Modifying Therapy ◽  
Current Therapies ◽  
Disease Modifying ◽  
Disease Modifying Treatment

Background: Over the past decade, three new drugs have been approved for the treatment of hereditary amyloid transthyretin (ATTRv) polyneuropathy. The aim of this work was to analyze whether current therapies prolong survival for patients affected by ATTRv amyloidosis. Methods: The study was conducted retrospectively, analyzing the medical records of 105 patients with genetic diagnoses of familial amyloidotic polyneuropathy followed at the two referral centers for the disease in Sicily, Italy. Of these, 71 received disease-modifying therapy, while 34 received only symptomatic treatment or no therapy. Results: The most used treatment in our patient cohort was tafamidis, followed by liver transplantation, patisiran, inotersen, and diflunisal. The median survival was significantly longer for treated vs. untreated patients (12 years vs. 8 years). In the 71 patients who received disease-modifying treatment, the presence of cardiac involvement, weight loss, or autonomic dysfunction at diagnosis was not related to survival. Conversely, patients diagnosed in the early stage of the disease (PND 1) had significantly longer survival than those diagnosed in the late stage (PND 2–4).

Biological therapy of allergic diseases during the COVID-19 pandemic

2020 ◽  
Vol 17 (3) ◽  
pp. 115-120
Author(s):  
Elena S. Fedenko ◽  
Olga G. Elisyutina ◽  
Natalia I. Il`ina
Keyword(s):  
Biological Therapy ◽  
Treatment Approach ◽  
Allergic Diseases ◽  
Scientific Data ◽  
Biological Therapies ◽  
Active Infection ◽  
The Past ◽  
Type 2 Immune Response ◽  
Antiviral Medications

The outbreak of the SARS-CoV-2-induced Coronavirus Disease 2019 (COVID-19) pandemic started in December 2019 in Wuhan, China, continued to spread across the globe and spanned 188 countries. Under the new circumstances treatment approach for T2 allergic diseases such as asthma, chronic hives, atopic dermatitis, and sinusitis with polyps has been changed. In the past years, new biological therapies monoclonal antibodies for these diseases have been developed targeting different aspects of the type 2 immune response. New knowledge on the COVID-19 disease course raises many issues around the safety of biologicals in patients with active infection, as well as their interactions with antiviral medications. In Russia new biological therapies entered clinical practice but its effectiveness and safety still are not known. This newsletter is based on Considerations on Biologicals for Patients with allergic disease in times of the COVID-19 pandemic: an EAACI Statement and the latest scientific data.

scholarly journals Re-treatment with etanercept is as effective as the initial firstline treatment in patients with juvenile idiopathic arthritis

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Jens Klotsche ◽  
Ariane Klein ◽  
Martina Niewerth ◽  
Paula Hoff ◽  
Daniel Windschall ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Response To Therapy ◽  
Inactive Disease ◽  
Window Of Opportunity ◽  
Younger Age ◽  
Synthetic Dmards ◽  
Disease Modifying ◽  
Mean Time ◽  
Active Disease ◽  
Subsequent Risk

Abstract Objectives To determine (i) correlates for etanercept (ETA) discontinuation after achieving an inactive disease and for the subsequent risk of flare and (ii) to analyze the effectiveness of ETA in the re-treatment after a disease flare. Methods Data from two ongoing prospective registries, BiKeR and JuMBO, were used for the analysis. Both registries provide individual trajectories of clinical data and outcomes from childhood to adulthood in juvenile idiopathic arthritis (JIA) patients treated with biologic disease-modifying anti-rheumatic drugs (bDMARDs) and conventional synthetic DMARDs (csDMARDs). Results A total of 1724 patients were treated first with ETA treatment course (338 with second, 54 with third ETA course). Similar rates of discontinuation due to ineffectiveness and adverse events could be observed for the first (19.4%/6.2%), second (18.6%/5.9%), and third (14.8%/5.6%) ETA course. A total of 332 patients (+/−methotrexate, 19.3%) discontinued ETA after achieving remission with the first ETA course. Younger age (hazard ratio (HR) 1.08, p < 0.001), persistent oligoarthritis (HR 1.89, p = 0.004), and shorter duration between JIA onset and ETA start (HR 1.10, p < 0.001), as well as good response to therapy within the first 6 months of treatment (HR 1.11, p < 0.001) significantly correlated to discontinuation with inactive disease. Reoccurrence of active disease was reported for 77% of patients with mean time to flare of 12.1 months. We could not identify any factor correlating to flare risk. The majority of patients were re-treated with ETA (n = 117 of 161; 72.7%) after the flare. One in five patients (n = 23, 19.7%) discontinued ETA again after achieving an inactive disease and about 70% of the patients achieved an inactive disease 12 months after restarting ETA. Conclusion The study confirms the effectiveness of ETA even for re-treatment of patients with JIA. Our data highlight the association of an early bDMARD treatment with a higher rate of inactive disease indicating a window of opportunity.

scholarly journals Citrobacter freundii sepsis in an immunosuppressed patient with pemphigus vulgaris

2018 ◽  
Vol 11 (1) ◽  
pp. e227091 ◽  
Author(s):  
Martina Ferranti ◽  
Giulia Tadiotto Cicogna ◽  
Andrea Sattin ◽  
Mauro Alaibac
Keyword(s):  
Immunosuppressive Agents ◽  
Pemphigus Vulgaris ◽  
Immunosuppressed Patient ◽  
Citrobacter Freundii ◽  
Bullous Disease ◽  
Autoimmune Bullous Disease ◽  
Systemic Corticosteroids ◽  
Mucous Membranes ◽  
Current Therapies ◽  
Anti Cd20

Pemphigus vulgaris is an autoimmune bullous disease that involves the skin and mucous membranes. Current therapies aim to decrease antibody production by means of the use of systemic corticosteroids, immunosuppressive agents and, recently, rituximab, an anti-CD20 monoclonal antibody. However, the chronic immune suppression could entail complications, like infections and secondary malignancies. We describe a case of a patient with pemphigus who developed a sepsis due to Citrobacter freundii infection.

Superior ophthalmic vein thrombosis following coil embolization of posterior communicating artery aneurysm: Report of two cases

2020 ◽  
pp. 112067212095144
Author(s):  
Kaveh Abri Aghdam ◽  
Naveed Nilforushan ◽  
Amin Zand ◽  
Rahim Kohansal
Keyword(s):  
Coil Embolization ◽  
Artery Aneurysm ◽  
Posterior Communicating Artery ◽  
Superior Ophthalmic Vein ◽  
Ocular Complications ◽  
Ipsilateral Side ◽  
Systemic Complications ◽  
Vein Thrombosis ◽  
Systemic Corticosteroids ◽  
Posterior Communicating Artery Aneurysm

Introduction: The purpose was to present two patients with superior ophthalmic vein thrombosis following coil embolization of posterior communicating artery aneurysm, that have not been reported after this particular procedure yet. Methods: We present two patients with subarachnoid hemorrhage due to posterior communicating artery aneurysms, who were treated with stent-assisted coil embolization. Shortly after the procedure, both cases demonstrated painful proptosis, ophthalmoplegia, increased intraocular pressure, intraretinal hemorrhage and macular edema on the ipsilateral side, with congestion of intraorbital tissues and thickened and dilated superior ophthalmic vein in neuroimaging investigation. The occlusion was confirmed by digital subtraction angiography. Results: Patients were treated with anticoagulant agents and systemic corticosteroids immediately and then received medical treatment by an ophthalmologist for ocular complications of superior ophthalmic vein thrombosis. Conclusion: Isolated superior ophthalmic vein thrombosis (SOVT) is rare, but may be associated with sight-threatening complications. After intracranial interventions on cerebral vessels, clinical features of SOVT including choroidal effusion syndrome should be investigated, and the patients should receive prompt and proper care including ophthalmic medications and systemic anticoagulants to restrict the ocular and systemic complications.

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