Search results for "vasculiti"

Anti-ETAR and suPAR as markers of disease activity in renal ANCA-associated vasculitis.

2020

Purpose In anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), there is a lack of reliable biomarkers of disease activity. The aim of the study was to evaluate soluble urokinase plasminogen activator receptor (suPAR) and anti-endothelin-1 type A receptor (anti-ETAR) antibodies levels in active phase and remission of AAV. Patients and methods We enrolled 60 patients (median age 63.0 years) with renal AAV into this study. Plasma suPAR, urine suPAR (expressed as urine suPAR/creatinine ratio) and serum anti-ETAR antibodies were assayed by ELISA. Disease activity was assessed using Birmingham Vasculitis Activity Score (BVAS) and patients were divided into 2 subgroups based o…

Alpha-1 antitrypsin deficiency: outstanding questions and future directions

2018

BACKGROUND: Alpha-1 antitrypsin deficiency (AATD) is a rare hereditary condition that leads to decreased circulating alpha-1 antitrypsin (AAT) levels, significantly increasing the risk of serious lung and/or liver disease in children and adults, in which some aspects remain unresolved. METHODS: In this review, we summarise and update current knowledge on alpha-1 antitrypsin deficiency in order to identify and discuss areas of controversy and formulate questions that need further research. RESULTS: 1) AATD is a highly underdiagnosed condition. Over 120,000 European individuals are estimated to have severe AATD and more than 90% of them are underdiagnosed. CONCLUSIONS: 2) Several clinical and…

Idiopathic Seidlmayer's Purpura: A Case Report

2014

Acute hemorrhagic edema of infancy (AHEI) was considered a rare form of Henoch-Schönlein purpura; however, it is now regarded as an independent disease typically involving patients aged 4-24 months. The authors describe the clinical case of a toddler aged 8 months, with skin erythematous pomphoid<b> </b>lesions, treated at home with topical steroids without benefits. The appearance of new lesions and the worsening of the previous skin signs induced the parents to drive the child to the hospital. The medical history revealed the administration of a vaccine dose 2 months before.

Patogeneza zapaleń naczyń związanych z przeciwciałami przeciwko cytoplazmie neutrofilów

2018

Zapalenia naczyń związane z przeciwciałami przeciwko cytoplazmie neutrofilów (antineutrophil cytoplasmic antibodies-associated vasculitis – AAV) to grupa chorób zajmujących małe naczynia i charakteryzujących się ich martwiczym zapaleniem oraz obecnością przeciwciał przeciwko cytoplazmie neutrofilów (antineutrophil cytoplasmic antibodies – ANCA) przy braku lub obecności jedynie skąpej ilości immunoglobulin w ścianie naczyń. Przeciwciała przeciwko cytoplazmie neutrofilów to przeciwciała klasy G skierowane przeciwko składnikom ziarnistości granulocytów obojętnochłonnych. Dowody na udział ANCA w patogenezie AAV pochodzą zarówno z obserwacji klinicznych, badań in vitro, jak i badań na zwierzętac…

Mononeuropathy multiplex as a result of treatment with interferon-α and ribavirin in a patient with hepatitis C

2008

Hepatitic C virus infection and autoimmunity : clinical features, diagnostic tools and therapeutical aspects

1995

Summary Since the genom of the hepatitis C virus (HCV) was detected the pathogenesis of a distinct group of chronic liver disease and some non-hepatic disorders of sofar unknown origin could be newly established. A wide variety of clinical features including vasculitis, glomerulonephritis, porphyria and rheumatoid-like symptoms may become apparant separately or in combination. HCV can be precipitated in cryoglobulins and tissue-related HCV was found in liver and kidney specimen, for instance. HCV-related cryoglobulinemia or circulating immune complexes often occur with vasculitis. Hematological changes may be present such as thrombocytopenia. A considerable amount of patients show circulati…

Leukocytoclastic vasculitis associated with sulfuric acid inhalation

2013

Correlation Analysis of Anti-Cardiolipin Antibody/D Dimer/C-Reactive Protein and Coronary Artery Lesions/Multiple-Organ Damage in Children With Kawas…

2021

Aim: Kawasaki disease (KD) is a systemic vasculitis with unknown etiology. In addition to cardiovascular system involvement, it can also have other multiple organs involved. This study is aimed at investigating the correlation between anti-cardiolipin antibody (ACA)/D dimer/C reactive protein (CRP) and coronary artery lesions (CAL)/multiple-organ lesions in children with KD.Methods: Retrospective analysis was performed in 284 KD/IKD patients from May 2015 to April 2016. Among them, 175 were males (61.6%), with average age of 2 years and 5 months old. Patients were divided into ACA+ group and ACA- group, elevated D dimer group (DDE) and normal D dimer group (DDN), and coronary artery injury …

Enfermedad de Adamantiades-Behçet: Un proceso enigmático con manifestaciones orales

2006

La enfermedad de Adamantiades-Behçet es una vasculitis multisistémica crónica, potencialmente capaz de afectar a cualquier órgano o sistema del cuerpo humano y en la que la aparición repetida de úlceras orales es una de sus principales expresiones clínicas. La EAB es una patología de carácter universal, con una prevalencia variable en función de la población estudiada y que muestra una curiosa distribución geográfica. A pesar de ser un proceso conocido desde la antigüedad, su etiopatogenia en la que probablemente se hayan implicados factores genéticos, microbiológicos e inmunológicos, continua siendo enigmática. Su amplio espectro de manifestaciones clínicas orales, genitales, cutáneas, ocu…

Adamantiades-Behçet disease : an enigmatic process with oral manifestations

2006

La enfermedad de Adamantiades-Behçet es una vasculitis multisistémica crónica, potencialmente capaz de afectar a cualquier órgano o sistema del cuerpo humano y en la que la aparición repetida de úlceras orales es una de sus principales expresiones clínicas. La EAB es una patología de carácter universal, con una prevalencia variable en función de la población estudiada y que muestra una curiosa distribución geográfica. A pesar de ser un proceso conocido desde la antigüedad, su etiopatogenia en la que probablemente se hayan implicados factores genéticos, microbiológicos e inmunológicos, continua siendo enigmática. Su amplio espectro de manifestaciones clínicas orales, genitales, cutáneas, ocu…