scholarly journals Rheumatoid arthritis associated with Rendu — Osler — Weber disease: Second description

2021 ◽  
Vol 16 (3) ◽  
pp. 147-152
Author(s):  
Leonid Churilov ◽  
◽  
Jozélio Freire de Carvalho ◽  
Carlos Ewerton Maia Rodrigues ◽  
Cristiano Augusto de Freitas Zerbini ◽  
...  

The article presents second ever published description of rheumatoid arthritis case co-morbid with Rendu — Osler — Weber disease (hereditary hemorrhagic teleangiectasia) in a 63-yearsold female patient. The aim is describing the first case report of a patient who after a confirmed hereditary hemorrhagic teleangiectasia diagnosis developed rheumatoid arthritis one year later. Historical data and brief pathophysiological characteristic of both diseases and their possible intermingle are included: (i) mutant genes in hereditary hemorrhagic teleangiectasia all encode proteins involved in the TGF-beta signaling pathway, and increased plasma levels of TGF-beta-1 and vascular endothelial growth factor have been seen in such patients; (ii) TGF-beta plays a role in the development of synovial cell proliferation, inflammation, and angiogenesis in rheumatoid arthritis; (iii) TGF-β regulates thymic T-cell selection, inhibits cytotoxic T lymphocyte (CTL), Th1-, and Th2-cell differentiation while promoting peripheral * Contribution of L. P. Churilov was supported by the grant of the Government of Russian Federation for state support of scientific research carried out under the supervision of leading scientists, agreement 14.W03.31.0009.Treg-, Th17-, Th9-, and Tfh-cell generation, and T-cell tissue residence in response to immune challenges, all essential for pathogenesis of rheumatoid arthriitis. The defect in its reception as well as its compensatory increased blood content theoretically can alter autoimmunity thus facilitating in Rendu — Osler — Weber disease patients the development of systemic autoimmune diseases.

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Seongseok Yun ◽  
Nicole D. Vincelette ◽  
Iyad Mansour ◽  
Dana Hariri ◽  
Sara Motamed

Metastatic cutaneous melanoma has poor prognosis with 2-year survival rate of 10–20%. Melanoma cells express various antigens including gp100, melanoma antigen recognized by T cells 1 (MART-1), and tyrosinase, which can induce immune-mediated anticancer response via T cell activation. Cytotoxic T-lymphocyte associated antigen-4 (CTLA-4) is an immune check point molecule that negatively regulates T cell activation and proliferation. Accordingly, recent phase III clinical trials demonstrated significant survival benefit with ipilimumab, a human monoclonal antibody (IgG1) that blocks the interaction of CTLA-4 with its ligands. Since the efficacy of ipilimumab depends on T cell activation, it is associated with substantial risk of immune mediated adverse reactions such as colitis, hepatitis, thyroiditis, and hypophysitis. We report the first case of late onset pericarditis and cardiac tamponade associated with ipilimumab treatment in patient with metastatic cutaneous melanoma.


Author(s):  
Young Min Cho ◽  
Erik W. Anderson ◽  
Sara Guevara ◽  
Santiago J. Miyara ◽  
Aqsa Iqbal ◽  
...  

T-cell large granular lymphocyte (T-LGL) leukemia is a rare and indolent clonal disorder of LGLs, associated with rheumatoid arthritis and neutropenia. The authors present a case of a 62-year-old male with rheumatoid arthritis (RA) who was diagnosed with T-LGL leukemia, with predominant neutropenia, and a poor response to conventional treatment. Subsequently, tofacitinib (a Janus Kinase 1 and 3 inhibitor, [JAK1/3 inhibitor]) resulted in improvement of the patient’s RA symptoms and temporary improvement of the neutropenia. Ultimately, upadacitinib (a specific JAK1 inhibitor) resulted in further improvement of the neutropenia and control of his RA. To the best of our knowledge, this is the first case report of coexisting RA and LGL leukemia that was treated with upadacitinib and showed clinical improvement.


2021 ◽  
Vol 3 (3) ◽  
pp. 160-170
Author(s):  
Mohammad Nur Ullah

On March 8, 2020, the first case of covid-19 was discovered in Bangladesh. As a result, the government decided to shut down all sectors for a certain period when the whole world was battling the first wave of Covid-19.  After one year of the first wave, the second wave has recently hit the planet affecting almost all the countries including Bangladesh. Like all other sectors, street vending has been encountered and felt severe distress regarding the vendors’ livelihood. This study aims to explore the livelihood of street vendors during covid-19 in Bangladesh. This study features an in-depth interview of 15 street vendors to document their experiences during lockdown due to covid-19. This study is based on a qualitative approach with a purposive sampling technique. This study found lockdown is a significant burden for street vendors, leaving them with no other choice for earning money. During the lockdown, they expend savings, borrow money from others, take out loans, sell home furniture, and obtain money from their parents to fulfil their needs and demands. In order to improve this situation of street vendors, the government should take necessary steps with adequate financial and food assistance so that the vendors can survive during this lockdown.


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