Noninfectious, Severe Cryoglobulinemic Vasculitis with Renal Involvement - Safety and Efficacy of Long-Term Treatment with Rituximab

CONCLUSION: Data from our observation show the efficacy of rituximab in the refractory, nonviral cryoglobulinemic vasculitis with a severe course of the disease. However, the therapy is associated with the risk of SAEs, especially in elderly patients with kidney failure and significant immunologic alterations.PMID:34295176 | PMC:PMC8291846 | DOI:10.2147/IJNRD.S315388
Source: International Journal of Nephrology and Renovascular Disease - Category: Urology & Nephrology Authors: Source Type: research

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Background: Kawasaki disease (KD) is an acute vasculitis of young children. A comparison of US hospitalization rates and epidemiologic features of KD in 2020 to those of precoronavirus disease years has yet to be reported. Methods: Using a large, inpatient database, we conducted a retrospective cohort study and analyzed data for patients with (1) diagnosis coding for KD, (2) IV immunoglobulin treatment administered during hospitalization and (3) discharge date between January 1, 2016, and December 30, 2020. Severe cases were defined as those requiring adjunctive therapy or IV immunoglobulin–resistant thera...
Source: The Pediatric Infectious Disease Journal - Category: Infectious Diseases Tags: Original Studies Source Type: research
ConclusionsThe PCT concentrations below 0.25  ng/ml may be useful for discriminating KD from sepsis, and moreover, the PCT concentrations of 0.25–0.50 ng/ml may be helpful in predicting IVIG nonresponsiveness.
Source: Clinical and Experimental Medicine - Category: Research Source Type: research
We describe four cases of mitochondrial strokes to highlight the considerable overlap that exists with other neurological disorders, including viral and autoimmune encephalitis, ischemic stroke, and central nervous system (CNS) vasculitis, and discuss the clinical, laboratory, and imaging features that can help distinguish MELAS from these differential diagnoses.
Source: Genes - Category: Genetics & Stem Cells Authors: Tags: Article Source Type: research
To the Editor We have some concerns about the recent study about rituximab and reduced-dose glucocorticoids vs high-dose glucocorticoids in antineutrophil cytoplasm antibody (ANCA) –associated vasculitis. In addition to the limitations of being an open-label, single-country study recruiting only new patients and having a wide noninferiority margin of −0 percentage points, the patients enrolled in this study were older (more likely to have adverse effects with high-dose glu cocorticoids), had a higher proportion of myeloperoxidase (MPO) positivity (which is associated with lower chance of relapse), had a higher ...
Source: JAMA - Category: General Medicine Source Type: research
In Reply Dr Chandwar and colleagues comment that the patients in our study had normal kidney function. However, we disagree because the median eGFR was 52 mL/min/1.73 m2, which is lower than the eGFR of the general Japanese population aged 70 to 74 years (median, 68 mL/min/1.73 m2). Second, the higher prevalence of MPO-ANCA positivity has been previously reported in Japanese patients. We agree that the applicability of our regimen to patients with PR3-ANCA positivity should be further investigated, although in our study the regimen was fairly effective in this subgroup of patients. Third, our study ’s treatment regim...
Source: JAMA - Category: General Medicine Source Type: research
Discussion PACNS is associated with disabling symptoms, frequent relapses, and significant mortality. Differences in symptoms and neuroimaging results and low overlap between biopsy and angiogram suggest that biopsy- and angiogram-confirmed cases represent different histopathologic types of PACNS. The optimal treatment is unknown.
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Vasculitis, All Cerebrovascular disease/Stroke Views [amp ] Reviews Source Type: research
Chest. 2021 Oct 14:S0012-3692(21)04099-X. doi: 10.1016/j.chest.2021.09.041. Online ahead of print.ABSTRACTBACKGROUND: Alpha-1 antitrypsin deficiency (AATD) is an autosomal co-dominant condition that predisposes to emphysema, cirrhosis, panniculitis, and vasculitis. Under-recognition has prompted efforts to enhance early detection and testing of at-risk individuals. Direct-to-consumer (DTC) genetic testing represents an additional method of detection.RESEARCH QUESTION: The study addressed three questions: 1) Does a DTC testing service identify previously undetected individuals with AATD? 2) What was the time interval betwee...
Source: Chest - Category: Respiratory Medicine Authors: Source Type: research
ConclusionThe Ar-BDCAF is a measurable, easy to calculate, and reliable index for assessing disease activity in Egyptian BD. The Ar-BDCAF score can be used in daily clinical practice to assess BD activity and its use can be extended to other Arab countries for possible regional validation and adaptations.Key Points• The Arabic version of the BDCAF can be extended to other Arab countries for development of a Pan-Arab score.• This is the first study to provide a reliable and valid Arabic version of the BDCAF-Egyptian dialect for measuring current disease activity in BD patients.
Source: Clinical Rheumatology - Category: Rheumatology Source Type: research
Circ Res. 2021 Oct 15;129(9):840-842. doi: 10.1161/CIRCRESAHA.121.320131. Epub 2021 Oct 14.NO ABSTRACTPMID:34648364 | DOI:10.1161/CIRCRESAHA.121.320131
Source: Circulation Research - Category: Cardiology Authors: Source Type: research
Takayasu arteritis is a chronic inflammatory vasculitis affecting mainly the aorta and its branches. Stenosis and occlusion of the involved vessels usually develop; however, their dilation and aneurysmal formation are extremely rare. Although aneurysmal disease has been reported in adults with Takayasu arteritis, it is a rare entity in children. The present report described an 11-year-old male found to have the subclavian-axillary, abdominal aortoiliac, lower extremity artery aneurysms with mural thrombi.
Source: Annals of Vascular Surgery - Category: Surgery Authors: Source Type: research
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