Polyarteritis Nodosa

Researchers are evaluating the effectiveness of three standard medications—colchicine, dapsone, and azathioprine—in treating patients with isolated skin vasculitis. This randomized, multicenter trial aims to compare these treatments over a two-stage process, focusing on patients with skin-limited vasculitis conditions such as small or medium vessel vasculitis, cutaneous polyarteritis nodosa, and IgA vasculitis without significant kidney involvement. The study is sponsored by the University of Pennsylvania and is conducted as a Phase 2 trial. Participants are initially randomized to receive one of the three study drugs for six months. The primary endpoint is the response to treatment at six months. If a participant needs to stop their assigned medication due to lack of response, disease flare, or side effects within the first six months or during follow-up up to twelve months, they will be re-randomized to one of the remaining two drugs for an additional six months. Response to therapy is evaluated again at the end of this second stage. During the study, participants will undergo regular assessments including physician and patient global response evaluations, quality of life measurements, and specific skin-related questionnaires at multiple time points up to twelve months. Researchers will monitor treatment response at months 3, 6, 9, and 12, with safety and side effects carefully observed. Total participation can last up to one year, with ongoing follow-up to assess the effectiveness and tolerability of the study medications.

Researchers are gathering detailed real-world information on people with chronic wounds and ulcers to better understand their medical journey and treatment outcomes. This observational registry collects data from over 1,400 healthcare providers across the USA and Puerto Rico using a specialized electronic health record system. It includes all major wound types such as diabetic foot ulcers, venous leg ulcers, pressure ulcers, arterial ulcers, surgical wounds, traumatic wounds, and inflammatory ulcers. Participants receive care as usual from their clinicians, who use standard and advanced wound treatments like specialized dressings, compression therapy, offloading devices, cellular and tissue-based products, negative pressure therapy, ultrasound therapy, topical oxygen, hyperbaric oxygen, growth factors, debridement, and topical antibiotics. Data on treatment practices, wound characteristics, patient health conditions, and complications are collected continuously during routine care. Patients may be followed for over five years to track long-term outcomes. During the study, researchers collect information on patient demographics, wound details, treatment methods, visit frequency, and complications. They monitor healing progress, amputations, deaths, wound recurrence, hospitalizations, and other outcomes. The registry uses a wound healing index to adjust for risk and supports quality improvement and research by linking clinical data with insurance claims for comprehensive analysis. This ongoing effort helps to better understand wound healing and treatment effectiveness in everyday healthcare settings.

Researchers are investigating autoimmune diseases, systemic vasculitis, and autoinflammatory diseases to find biomarkers in cytokines and lymphocytes that show disease activity. The goal is to develop follow-up markers that help personalize monitoring and treatment for each patient. These diseases affect the immune system with varying severity, and current treatments mainly control inflammation but often lead to relapses without clear ways to predict them. The study collects blood and tissue samples as part of regular patient care to analyze immunological data. Conducted at a specialized medical center certified for rare autoimmune and inflammatory diseases, this observational study focuses on advancing fundamental knowledge and identifying prognostic markers through blood tests. Participants include patients with various autoimmune and autoinflammatory diseases who will provide additional blood samples beyond routine care. Participants will undergo biological sample collection during their usual care visits, with researchers monitoring cytokine and lymphocyte profiles over approximately nine years. The study aims to correlate these immune profiles with disease activity, progression, clinical presentation, and relapse frequency. This long-term observation helps understand disease evolution and supports personalized care. Safety and usual medical care continue throughout the study period.

Researchers are conducting a multicenter retrospective observational study to identify and describe vocal cord lesions that cause dysphonia in patients with systemic autoimmune diseases. The study focuses on understanding the types of lesions, such as "bamboo nodes" and other specific or non-specific lesions, their diagnosis timing, and treatment approaches in conditions like rheumatoid arthritis, systemic lupus erythematosus, and other connective tissue diseases. The study involves reviewing medical records from January 2014 to December 2024 to characterize vocal cord lesions responsible for dysphonia. The research examines patient histories for lesions like fibrosis, immune complex deposits, rheumatoid nodules, granulomas, and other mucosal abnormalities. Treatment methods like vocal therapy, oral steroids, surgery, and avoiding irritants are noted, though management varies due to the rarity of these conditions. Participants' records will be reviewed for lesion type as diagnosed by specialists such as otorhinolaryngologists or rheumatologists. The study assesses diagnosis delays, lesion management, and associated risk factors. Participants will not undergo new treatments but will have their medical histories analyzed to better understand dysphonia causes in autoimmune disease contexts.

Researchers are studying whether monitoring certain blood markers like circulating B-cell reconstitution and serum rituximab levels can help detect relapse in patients with autoimmune diseases treated with rituximab. The study focuses on autoimmune conditions such as rheumatoid arthritis, systemic lupus erythematosus, and autoimmune cytopenia, aiming to identify risk factors for clinical relapse based on these blood markers. Participants receive routine care monitoring after rituximab treatment, with clinical visits every three months during the first year and every six months thereafter. At each visit, blood tests measure total gammaglobulins, IgG, CD19+ cells, and additional markers such as CD27+ and CD38+ B cells, serum rituximab, and anti-rituximab antibodies. The study compares these markers between patients who relapse and those who remain stable over time. During the study, leftover blood samples from routine care are used to analyze these immune markers over the first year of follow-up. Patients will be monitored regularly for up to five years, with assessments at each consultation to track their disease status. The main outcome is to identify the relationship between these blood markers and clinical relapse in various autoimmune disorders.

Researchers are studying a special type of coronary artery disease called inflammation-associated non-rapidly-progressive coronary artery disease (INR-CAD). This condition differs from typical atherosclerotic coronary artery disease by affecting mainly women, starting earlier in life, lacking traditional risk factors, showing signs of chronic inflammation, and responding differently to treatments. The study aims to understand the clinical features, current treatments, and outcomes in patients with INR-CAD through an observational cohort design. Patients diagnosed with INR-CAD are identified based on specific clinical criteria including coronary artery lesions and evidence of chronic inflammation. They receive follow-up care over 24 months according to a protocol designed for INR-CAD. Treatments under evaluation include healthy lifestyle changes, secondary prevention medications for atherosclerosis, immunosuppressive therapy, coronary revascularization procedures like PCI or CABG, and supportive therapies to manage side effects. Participants will undergo various assessments during the 24-month follow-up, including monitoring for major adverse cardiovascular events (MACE) such as heart attacks and hospitalizations. Researchers will also measure exercise capacity, detailed angiographic features of coronary lesions, and inflammatory markers like ESR, hs-CRP, IL-6, and TNF-alpha. Safety outcomes like major bleeding and severe infections will be tracked. The study will compare outcomes between patients with different diagnoses and treatments to better understand INR-CAD's progression and management.

This research aims to better understand cutaneous vasculitis by studying skin lesions in patients with various types of idiopathic vasculitis. It involves a multi-center approach with both primary vasculitis care providers and dermatologists working together to select suitable patients and collect lesion samples for analysis. The study focuses on evaluating the histopathology and gene expression profiles (transcriptome) of these skin lesions. Participants with active vasculitis skin lesions will undergo a punch biopsy to collect tissue samples. These samples will be examined using standardized methods to assess the histopathology and transcriptomic characteristics of the cutaneous vasculitis. The study does not involve any experimental treatments but gathers detailed clinical data and biopsy specimens for analysis. Participants will be evaluated by specialists, who will collect clinical information and perform skin biopsies at affected sites. The study will review clinical data linked to biopsy samples over one year. Researchers will monitor the samples to better characterize the disease at the tissue level. The total participation time varies, with follow-up focused on the evaluation of biopsy results and clinical data.

Researchers are collecting biological and clinical data from patients with systemic autoimmune diseases to better understand these complex conditions. These diseases affect a notable portion of adults, mostly women, and involve the immune system attacking the body. The study aims to identify new biomarkers and improve diagnosis and treatment options by exploring the clinical, biological, and genetic differences among patient groups. Participants will provide blood samples during their routine diagnosis and follow-up visits, with a larger amount of blood taken specifically for this study. This observational study focuses on patients with rare systemic autoimmune diseases or atypical forms who may be undergoing innovative treatments such as new drugs, gene therapy, or cell therapy. During the study, researchers will collect biological samples and clinical data and analyze them to find new diagnostic and prognostic markers, predict relapses or treatment responses, and understand disease mechanisms. The study will monitor patients for about one year, with the goal of improving personalized medicine approaches for these autoimmune diseases.

Researchers are conducting a natural history study to collect blood samples from healthy adults to support research on diseases involving blood, blood vessels, and the immune system. The study aims to establish normal physiological parameters and validate technical tests for vascular inflammation and thrombosis. This research will help improve understanding and testing methods for vascular and immunologic diseases. Participants will provide blood samples during visits to the NIH Clinical Center, where about 10 tablespoons of blood will be drawn from a vein. Blood tests may include complete blood count and blood typing, and the samples will be used for genetic studies and other research assays. Some participants may return for repeat visits over a period of up to 10 years to provide additional samples and updated medical history. During the study, participants will have their medical history reviewed remotely via telehealth or telephone and in person during visits. The research team will monitor the collected blood samples to optimize assays and study vascular biomarkers. Participants will receive $50 for each blood collection visit. The study is designed to follow Good Clinical Practices and does not have a specific outcome measure but supports ongoing and future research.

Large vessel vasculitis (LVV) is an autoimmune disease that causes inflammatory damage to large blood vessels, which can lead to serious complications like heart failure and stroke. Patients with LVV have an increased risk of cardiovascular disease, possibly due to a chemical called endothelin that causes blood vessels to stiffen and raises blood pressure. Researchers are studying whether blocking endothelin and using another drug that improves vessel function can help improve blood vessel health in people with LVV. In this study, participants with LVV will receive the drug Bosentan, which blocks endothelin receptors, together with Dapagliflozin, a medication that helps improve blood vessel function, for 6 weeks. After this, participants will continue on Dapagliflozin alone for 4 weeks. Blood vessel function will be assessed through tests including forearm blood flow studies and blood pressure measurements. The study also includes a comparison group of people without LVV who will undergo similar assessments but do not receive the medications. Participants will have their blood vessel function measured using venous occlusion plethysmography, along with tests of blood pressure, arterial stiffness, eye microvasculature, and blood cell analysis before and after treatment. The study will track changes from baseline to week 6 in these measures to evaluate the effects of the treatments. The total treatment period for those with LVV is 10 weeks, with close monitoring during and after medication use. Participants will also have assessments to understand fibrinolytic capacity and inflammatory cell balance, helping researchers understand treatment impact on vessel health.