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Medicine Feb 2019Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized, immune-mediated chronic fibrotic inflammation that can involve almost all organs, causing...
BACKGROUND
Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized, immune-mediated chronic fibrotic inflammation that can involve almost all organs, causing tumefaction and dysfunction. Its presence in pulmonary circulation is underestimated and has not yet been investigated.
OBJECTIVES
We describe a representative IgG4-RD patient with pulmonary artery stenosis and pulmonary embolism, leading to reversible pulmonary hypertension. Literature review of IgG4-RD with pulmonary circulation involvement was conducted.
DATA SOURCES
References for this review were identified through searches via PubMed, EBSCO, and Web of Science for published articles before November 2016.
RESULTS
There were 15 published cases of IgG4-RD with pulmonary vascular involvement, 3 with pulmonary arteritis, 2 with pulmonary artery aneurysm, 3 with pulmonary artery stenosis, 1 with obliterative phlebitis, and 1 with pulmonary embolism. Possible immunity and inflammation mechanisms were summarized.
CONCLUSIONS
IgG4-RD with pulmonary vascular involvement is rare. Echocardiogram and contrast-enhanced chest CT are helpful to screen the disease. Clinical manifestations were found from asymptomatic to dyspnea or even syncope. And nearly all cases had more than 1 organ affected, with significantly increased serum IgG4 levels. PET/CT aided in identifying affected organs and determining candidate biopsy sites. More awareness is urged to evaluate the pulmonary vascular manifestations of this disease.
Topics: Humans; Male; Middle Aged; Echocardiography; Hypertension, Pulmonary; Immunoglobulin G4-Related Disease; Pulmonary Embolism; Radiography, Thoracic; Stenosis, Pulmonary Artery
PubMed: 30732204
DOI: 10.1097/MD.0000000000014437 -
Reumatologia Clinica 2010To analyze published evidence about adalimumab use in autoimmune diseases.
OBJECTIVE
To analyze published evidence about adalimumab use in autoimmune diseases.
METHODS
Systematic review of MEDLINE database of citations included from January 1990 to December 2008 employing the terms "adalimumab" and the different systemic autoimmune diseases.
RESULTS
Our search identified 241 potentially relevant citations. 154 were retrieved for detailed evaluation. Finally, 18 were selected as relevant, including 54 patients. The reported diseases were as follow: Behçet disease in 16 patients, idiopathic uveitis in 13, sarcoidosis in 5, uveitis associated with rheumatologic diseases in 5 (psoriasis in 2, ankylosing spondylitis in 1, juvenile idiopathic arthritis in 1, Crohn disease in 1), Vogt-Koyanagi-Harada disease in 4, Birdshot uveitis in 4, vasculitis in 3 (1 temporal arteritis, 1 Takayasu's disease, 1 skin vasculitis associated with rheumatoid arthritis), adult onset Still disease in 2, relapsing polychondritis in 1 and systemic sclerosis in 1. The clinical spectrum included uveitis (39 cases), skin and/or mucosae (9), vasculitis (3), arthritis (6), lung (3). These patients were refractory to standard therapy, including corticosteroids (42 cases, 78%), immunosuppressants (42, 78%) and biologics (29, 54%). Fifty (93%) patients responded to adalimumab. The clinical response was similar in those patients who had been treated with other biologic and in those who had not received biologic therapy before adalimumab. The patients were followed for 11.9 months. Twelve (22%) patients relapsed. Five (9%) patients suffer some side effect (3 local skin reaction, 1 angioedema, 1 lung fibrosis). One patient (2%) died due to progression of her disease.
CONCLUSIONS
Available data about the use of adalimumab in autoinmune diseases come from case reports and uncontrolled studies, that include patients with severe disease and refractory to standard therapy. In this setting, it seems to be an effective and safe treatment option, especially in patients with uveitis and Behçet's disease. This initial data must be confirmed by controlled assays before extending adalimumab use.
PubMed: 21794697
DOI: 10.1016/j.reuma.2009.06.004 -
RMD Open 2019To analyse the current evidence for the management of large vessel vasculitis (LVV) to inform the 2018 update of the EULAR recommendations. (Comparative Study)
Comparative Study Meta-Analysis
OBJECTIVES
To analyse the current evidence for the management of large vessel vasculitis (LVV) to inform the 2018 update of the EULAR recommendations.
METHODS
Two systematic literature reviews (SLRs) dealing with diagnosis/monitoring and treatment strategies for LVV, respectively, were performed. Medline, Embase and Cochrane databases were searched from inception to 31 December 2017. Evidence on imaging was excluded as recently published in dedicated EULAR recommendations. This paper focuses on the data relevant to giant cell arteritis (GCA).
RESULTS
We identified 287 eligible articles (122 studies focused on diagnosis/monitoring, 165 on treatment). The implementation of a fast-track approach to diagnosis significantly lowers the risk of permanent visual loss compared with historical cohorts (level of evidence, LoE 2b). Reliable diagnostic or prognostic biomarkers for GCA are still not available (LoE 3b).The SLR confirms the efficacy of prompt initiation of glucocorticoids (GC). There is no high-quality evidence on the most appropriate starting dose, route of administration, tapering and duration of GC (LoE 4). Patients with GCA are at increased risk of dose-dependent GC-related adverse events (LoE 3b). The addition of methotrexate or tocilizumab reduces relapse rates and GC requirements (LoE 1b). There is no consistent evidence that initiating antiplatelet agents at diagnosis would prevent future ischaemic events (LoE 2a). There is little evidence to guide monitoring of patients with GCA.
CONCLUSIONS
Results from two SLRs identified novel evidence on the management of GCA to guide the 2018 update of the EULAR recommendations on the management of LVV.
Topics: Antibodies, Monoclonal, Humanized; Antirheumatic Agents; Biomarkers; Blindness; Drug Therapy, Combination; Female; Giant Cell Arteritis; Glucocorticoids; Humans; Male; Methotrexate; Observational Studies as Topic; Outcome Assessment, Health Care; Randomized Controlled Trials as Topic; Recurrence; Risk Management; Systemic Vasculitis; Takayasu Arteritis
PubMed: 31673411
DOI: 10.1136/rmdopen-2019-001003 -
PloS One 2014Radiation arteritis following neck irradiation as a treatment for head and neck malignancy has been well documented. The long-term sequelae of radiation exposure of the... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
Radiation arteritis following neck irradiation as a treatment for head and neck malignancy has been well documented. The long-term sequelae of radiation exposure of the carotid arteries may take years to manifest clinically, and extra-cranial carotid artery (ECCA) stenosis is a well-recognised vascular complication. These carotid lesions should not be regarded as benign and should be treated in the same manner as standard carotid stenosis. Previous studies have noted increased cerebrovascular events such as stroke in this cohort of patients because of high-grade symptomatic carotid stenosis resulting in emboli.
AIM
To evaluate the effect of radiation therapy on ECCA atherosclerosis progression.
METHODS
Online search for case-control studies and randomised clinical trials that reported on stenosis in extra-cranial carotid arteries in patients with neck malignancies who received radiation therapy (RT) comparing them to patients with neck malignancies who did not receive RT.
RESULTS
Eight studies were included in the final analysis with total of 1070 patients - 596 received RT compared to 474 in the control group. There was statistically significant difference in overall stenosis rate (Pooled risk ratio = 4.38 [2.98, 6.45], P = 0.00001) and severe stenosis (Pooled risk ratio = 7.51 [2.78, 20.32], P <0.0001), both being higher in the RT group. Pooled analysis of the five studies that reported on mild stenosis also showed significant difference (Pooled risk ratio = 2.74 [1.75, 4.30], 95% CI, P = 0.0001).
CONCLUSION
The incidence of severe ECCA stenosis is higher among patients who received RT for neck malignancies. Those patients should be closely monitored and screening programs should be considered in all patients who receive neck RT.
Topics: Carotid Artery Diseases; Carotid Artery, Internal; Giant Cell Arteritis; Head and Neck Neoplasms; Humans; Radiotherapy; Randomized Controlled Trials as Topic; Risk Factors; Ultrasonography, Doppler, Duplex
PubMed: 25329500
DOI: 10.1371/journal.pone.0110389