Publications by authors named "Jan Portegys"

Article Synopsis
  • The study aimed to create a standardized scoring system called the Magnetic Resonance Vasculitis Activity Score (MRVAS) to evaluate the extent of Giant Cell Arteritis (GCA) using MRI images of cranial vessels and the aorta.
  • A total of 80 patients were assessed with different levels of readers (experienced and inexperienced), who rated inflammation in the vessels, leading to a score ranging from 0 to 20.
  • Results showed excellent agreement among readers in scoring inflammation levels, indicating that the MRVAS effectively allows for a reliable assessment of GCA inflammation.
View Article and Find Full Text PDF

Autologous hematopoietic stem cell transplantation (aHSCT) represents an effective treatment option in patients with severe forms of systemic sclerosis (SSc) by resetting the immune system. Nevertheless, secondary autoimmune disorders and progressive disease after aHSCT might necessitate renewed immunosuppressive treatments. This is particularly challenging when organ dysfunction, i.

View Article and Find Full Text PDF

Objectives: The spectrum of giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) represents highly inflammatory rheumatic diseases. Patients mostly report severe physical impairment. Possible consequences for mental health have been scarcely studied.

View Article and Find Full Text PDF

Erdheim-Chester disease (ECD) is a non-Langerhans cell histiocytosis characterised by clonal expansion of histiocytes in various organs. These induce an inflammatory environment, which leads to damage of the affected areas. Recently, a new disease entity was proposed encompassing key features of ECD but also of Rosai-Dorfman-Destombes disease, another histiocytosis.

View Article and Find Full Text PDF

Background: Genital human papillomavirus (HPV)-infections are common in the general population and are responsible for relevant numbers of epithelial malignancies. Much data on the HPV-prevalence is available for secondary immunodeficiencies, especially for patients with human immunodeficiency virus (HIV)-infection. Little is known about the genital HPV-prevalence in patients with primary immunodeficiencies (PIDs).

View Article and Find Full Text PDF

The 2-deoxy-d-[F]fluoro-D-glucose (FDG) positron emission tomography/computed tomography (PET/CT) is widely utilized to assess the vascular and articular inflammatory burden of patients with a suspected diagnosis of rheumatic disease. We aimed to elucidate the impact of [F]FDG PET/CT on change in initially suspected diagnosis in patients at the time of the scan. Thirty-four patients, who had undergone [F]FDG PET/CT, were enrolled and the initially suspected diagnosis prior to [F]FDG PET/CT was compared to the final diagnosis.

View Article and Find Full Text PDF

Systemic sclerosis (SSc) is a severe chronic disease with a broad spectrum of clinical manifestations. SSc displays disturbed lymphocyte homeostasis. Immunosuppressive medications targeting T or B cells can improve disease manifestations.

View Article and Find Full Text PDF

Background: Digital health applications/apps (DiGA) are entering many medical disciplines and have the potential to revolutionize patient care. In rheumatology, the use for axial spondyloarthritis (axSpA) would be conceivable in the form of an exercise app. Therefore, a representative survey among axSpA patients was conducted to determine the need for an axSpA exercise app.

View Article and Find Full Text PDF

Background: The provision of compatible blood products to patients is the most essential task of transfusion medicine. Besides ABO and Rh, a number of additional blood group antigens often have to be considered for the blood supply of immunized or chronically transfused patients. It also applies for platelet antigens (HPA) and neutrophil antigens (HNA) for patients receiving platelet or granulocyte concentrates.

View Article and Find Full Text PDF

Background: The detection and identification of antibodies to red blood cell (RBC) antigens is one of the most important and challenging issues in transfusion medicine. Up to date there are 354 RBC antigens recognized by the International Society of Blood Transfusion (ISBT). The reagent RBCs used in commercial antibody screening and identification panels however are usually serologically typed for up to 40 clinically important antigens.

View Article and Find Full Text PDF