Vasculitis: From Target Molecules to Novel Therapeutic Approaches

Chung, Sang Wan

doi:10.3390/biomedicines9070757

Cited by 5 publications

(9 citation statements)

References 103 publications

(106 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…El uso de agentes biológicos como el rituximab surge como nueva terapia de tratamiento tanto para la fase de inducción o recaída, como para el mantenimiento de la enfermedad [15][16][17] . Desde el año 2010 es una opción en inducción o mantenimiento cada vez más atractiva debido a su perfil de seguridad 29,[64][65][66][67][68][69] .…”

Section: Discussionunclassified

“…El tratamiento de inducción más común fue la combinación de corticoides en forma de pulsos asociados a ciclofosfamida endovenosa, debido en parte al compromiso clínico de la enfermedad, hecho que coincide con reportes argentinos 18 . La tasa de remisión inducida por este esquema fue del 50%, que es menor a la reportada, estimada en el seguimiento en 75% 45,67,68 . En cuanto a los corticoides en inducción se están reevaluando las dosis óptimas extrapolando lo que ocurre en el lupus eritematoso sistémico.…”

Section: Discussionunclassified

See 1 more Smart Citation

Vasculitis asociadas a ANCA: análisis de 26 pacientes

Young¹

2022

RFEM

View full text Add to dashboard Cite

Las vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos (ANCA) comprenden un subgrupo de afección de pequeños vasos que comparten en forma variable la presencia de ANCA. Se analizaron todos los pacientes con vasculitis asociadas a ANCA que fueron atendidos en el Hospital Británico de Buenos Aires durante los años 2000 a 2016 con el objetivo de describir la casuística de nuestro centro. Se incorporaron un total de 26 pacientes: 11 (42.31%) granulomatosis con poliangeítis (GPA), 6 (23.08%) poliangeítis microscópica (MPA) y vasculitis limitada al riñón (VLR) y 3 (11.54%) granulomatosis eosinofílica con poliangeítis (EGPA). La edad media al diagnóstico fue de 51.73±18.85 años (18-78) con predominio de sexo femenino (57.61%). El compromiso inicial más frecuente fue renal (73.08%), seguido por compromiso pulmonar (42.31%) y rinosinusal (26.92%). Cinco pacientes (19.23%) debutaron con síndrome pulmón-riñón y 6 (23.08%) presentaron afectación de más de dos órganos al inicio. La clasificación clínica más común fue la generalizada (34.62%), seguido por la severa (30.77%), con una media de BVAS de 16.6. El 92.31% presentaron determinaciones de ANCA positivas y 76.92% biopsia diagnóstica positiva. Los 26 pacientes realizaron tratamiento de inducción, la adición de ciclofosfamida a glucocorticoides fue la terapia dual más frecuente. En forma global el 50% de los pacientes que ingresaron al estudio lograron remisión, el 61.53% luego de la inducción inicial y el resto luego del tratamiento de la recaída. La mortalidad global fue de 15.38%, ocurriendo durante la inducción inicial, no reportándose eventos durante la remisión o recaída. Del análisis de nuestra serie se desprende que a pesar del compromiso clínico severo es posible reducir la mortalidad con los nuevos esquemas de tratamiento.

show abstract

Section: Discussionunclassified

Vasculitis asociadas a ANCA: análisis de 26 pacientes

Young¹

2022

RFEM

View full text Add to dashboard Cite

show abstract

“…Owing to non-inferiority to CYC, RTX with a 375 mg/m 2 weekly × 4 regimen was approved as a first-line therapy at induction in severe AAV [ 1 , 2 ]. Since RTX is less toxic than CYC and has lower relapse rates than other IS for maintenance, the 2021 ACR/Vasculitis Foundation Guideline has recommended this biologic agent over CYC for remission induction and over other IS for remission maintenance in active, severe AAV patients [ 11 ].…”

Section: Discussionmentioning

confidence: 99%

“…Eosinophilic granulomatosis with polyangiitis (EGPA), a rare autoimmune disorder occurring exclusively in asthmatic patients, is classified as anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) [ 1 , 2 ]. Among the three AAV-related disorders, cardiac involvement presenting as myocarditis is most commonly observed in EGPA with serious complications, including overt heart failure and sudden cardiac death [ 3 , 4 ].…”

Section: Introductionmentioning

confidence: 99%

B-Cell-Depleting Therapy Improves Myocarditis in Seronegative Eosinophilic Granulomatosis with Polyangiitis

Wang

Tsai

et al. 2021

JCM

View full text Add to dashboard Cite

Cardiac involvement is a major mortality cause in eosinophilic granulomatosis with polyangiitis (EGPA), requiring novel therapeutics to spare the use of cyclophosphamide with known cardiotoxicity. Despite the observed efficacy of B-cell-depleting therapy in myocarditis of seropositive microscopic polyangiitis, it remains to be elucidated in seronegative EGPA. A retrospective study was performed in 21 hospitalized active patients aged 20 to 70 years with five-factor score 1 or 2, eosinophil counts 10,034 ± 6641/μL and vasculitis scores 27 ± 6. Overt myocarditis was identified in 10 cases, at disease onset in 6 and relapse in 4, with endomyocarditis in 4 and myopericarditis in 4. Five seronegative and one seropositive patient received rituximab with an induction regimen 375 mg/m2 weekly × 4 for refractory or relapse disease, and the same regimen for annual maintenance therapy. All cases had lower eosinophil counts, improved cardiac dysfunction and clinical remission with a relapse-free follow-up, 48 ± 15 months after the induction treatment. One seronegative endomyocarditis patient had eosinophilia and disease relapse with asthma attack and worsening cardiac insufficiency 24 months after induction, achieving clinical remission under anti-IL-5 therapy. Our findings suggest the suppression of IL-5-mediated eosinophilia as an action mechanism of B-cell-depleting therapy in seronegative EGPA myocarditis.

show abstract

“…SV is a rare SRD characterized by inflammation of vascular walls, resulting in a broad spectrum of clinical manifestations dependent on the site, type, and size of involved vessels[ 155 ]. Although the diagnosis relies on clinical presentations confirmed by histopathological findings, large/medium and small vessel involvement can be supported by angiographical examinations and laboratory tests ( e.g ., ANCA), respectively[ 155 , 156 ]. Owing to hepatic vascular involvement[ 2 , 53 ], polyarteritis nodosa (referred to herein as PAN), a medium-vessel SV associated with HBV infection[ 157 ], may have elevated liver enzymes.…”

Section: Svmentioning

confidence: 99%

Autoimmune liver diseases in systemic rheumatic diseases

Wang¹,

Tsai²

2022

WJG

View full text Add to dashboard Cite

Systemic rheumatic diseases (SRDs) are chronic, inflammatory, autoimmune disorders with the presence of autoantibodies that may affect any organ or system. Liver dysfunction in SRDs can be associated with prescribed drugs, viral hepatitis, alternative hepatic comorbidities and coexisting autoimmune liver diseases (AILDs), requiring an exclusion of secondary conditions before considering liver involvement. The patterns of overlap diseases depend predominantly on genetic determinants with common susceptible loci widely distributing in both disorders. In AILDs, it is important to identify the overlapping SRDs at an early stage since such a coexistence may influence the disease course and prognosis. Commonly co-occurring SRDs in AILDs are Sjögren syndrome (SS), rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE) in autoimmune hepatitis (AIH), and SS, RA or systemic sclerosis in primary biliary cholangitis. Owing to different disease complications and therapies, it is imperative to differentiate between SLE liver involvement and SLE-AIH overlap disease. Therapeutic options can be personalized to control coexisting conditions of liver autoimmunity and rheumatic manifestations in AILD-SRD overlap diseases. The collaboration between hepatologists and rheumatologists can lead to significant advances in managing such a complex scenario. In this review, we provide a comprehensive overview on coexisting AILDs in different SRDs and the therapeutic approach in managing these overlap diseases.

show abstract

Vasculitis: From Target Molecules to Novel Therapeutic Approaches

Cited by 5 publications

References 103 publications

Vasculitis asociadas a ANCA: análisis de 26 pacientes

Vasculitis asociadas a ANCA: análisis de 26 pacientes

B-Cell-Depleting Therapy Improves Myocarditis in Seronegative Eosinophilic Granulomatosis with Polyangiitis

Autoimmune liver diseases in systemic rheumatic diseases

Contact Info

Product

Resources

About