The Pathogenesis and Natural History of Sjogren's Disease
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 发起方
- 入组人数
- 300
- 试验地点
- 1
- 主要终点
- Clinical phenotyping of SjD patients and controls over time Collection of samples for concurrent and future laboratory studies related to the pathogenesis Identification of biomarker candidates in SjD
研究概览
简要总结
Background:
-Sjogren s Disease (formerly: Sjogrens Syndrome, Sj(SqrRoot)(Delta)gren s syndrome) is a disease that affects about 1-4 million Americans. It is more common in women. It mainly affects the glands that produce saliva and tears, leading to dry eyes and dry mouth. The cause of Sjogren s Disease is unknown, but inflammation plays an important role. The purpose of this study is to learn more about Sjogren s Disease.
Objectives:
-To better understand how Sjogren s Disease begins and how it affects patients so that we can develop better ways to treat them.
Eligibility:
- Participants must be 16 years of age or older.
- They must have a diagnosis of Sjogren s Disease or have at least two symptoms of Sjogren s Disease.
Design:
- People taking part in the study will come to the NIH Clinical Center for at least three visits.
- During these visits, participants will have a medical history and physical exam. They will have oral and dental assessments, and saliva collection. Lab tests (blood and urine) and dry eye exams will be done. Participants will answer questionnaires and have salivary scintigraphy (adults only unless required for diagnosis).
- Other optional tests may also be done. Participants may have to come in for additional visits if they have these optional tests or if their disease changes.
- The only treatment provided as part of this study is for medical emergencies or complications that occur while you are at NIH for evaluation.
详细描述
Background
Sjogren's Disease (SjD; formely Sjogren's Syndrome) is an autoimmune disease characterized by chronic inflammation involving the exocrine glands. Salivary and lacrimal glands are predominantly affected leading to dry mouth and dry eyes but other exocrine organs are also frequently involved. It is one of the most common rheumatic autoimmune diseases, which effects between 1-4 million Americans, predominantly women with a female to male ratio of 9:1. Sjogren s Disease may occur alone (primary SjD), or may coexist with other systemic connective tissue disorders (i.e., secondary SjD). In many cases systemic manifestations, such as fatigue, arthritis, vasculitis, lung disease, peripheral or central neuropathy and autonomic nervous system dysfunction accompany glandular involvement. Patients with systemic manifestations are at higher risk of lymphoma, the incidence of which is increased in SjD. The treatment of sicca symptoms is mainly symptomatic, whereas management of extraglandular manifestations is similar to other autoimmune diseases.
The cause and pathogenesis of Sjogren's Disease is still largely unknown. In a genetically predisposed individual various environmental factors, such as viral infections, may lead to epithelial cell activation and a protracted inflammatory response with features of autoimmunity. Autoreactive lymphocytes and autoantibodies are considered important in this process although the pathogenic role of any particular autoantibody is still undefined. Although inflammation may contribute to the exocrinopathy of SjD, the relationship between inflammation and exocrine dysfunction is poorly understood. Moreover, the model does not explain many of the extraglandular manifestations of SjD patients, such as fatigue. Further studies are needed to better understand the pathogenesis of SjD.
Objectives
The primary objective of this study is to enable the collection of longitudinal clinical and laboratory data and biologic specimens to identify pathogenetic mechanisms of SjD by careful clinical phenotyping of SjD patients and Sjogren's-like conditions over time and collection of biologic samples for concurrent and future laboratory studies related to the pathogenesis of Sjogren's Disease. Another objective of the study is to identify biomarker candidates associated with the diagnosis, severity, prognosis, or organ involvement in SjD. The protocol will enable the study of the genetic basis and the mechanistic aspects of immunologic and non-immunologic abnormalities of SjD and their associations with various clinical phenotypes.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 年龄范围
- 16 Years 至 100 Years（Child, Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •INCLUSION CRITERIA:
- •Ability to sign informed consent form
- •Fulfilling one the definitions below:
- •Sjogren s defined by European-American (EA) classification criteria for primary or secondary Sjogren s Disease (SjD group)
- •Excluded from the EA criteria because of a comorbid condition but otherwise fulfilling the European-American classification criteria (EA excluded SjD group)
- •Incomplete SjD
- •i. at least 2 of the EA criteria with a common manifestation of SjD not included in these criteria (e.g., fatigue, vasculitis, arthritis, etc) or
- •ii. 2 or more common manifestations of SjD which are not included in the EA criteria (e.g.,: fatigue, vasculitis, arthritis, autonomic dysfunction, etc ) and are not explained by other conditions
排除标准
- •Age <16 years
- •inability or unwillingness to comply with follow up requirements
- •Any medical or psychological/psychiatric condition or treatment that, in the opinion of the Principal Investigator, would exclude the subjects from the research studies (e.g., alternative explanation for subjects signs and symptoms)
- •NIH employees who report directly to the principal investigator or who are a co-worker or relative of the principal investigator.
研究组 & 干预措施
incomplete sjogren's
patients who meet criteria as incomplete sjogren's
primary sjogren's
patients who meet criteria for classification for primary sjogren's
secondary sjogren's
patients who meet classification criteria for secondary sjogren's
结局指标
主要结局
Clinical phenotyping of SjD patients and controls over time Collection of samples for concurrent and future laboratory studies related to the pathogenesis Identification of biomarker candidates in SjD
时间窗: patients are followed up to 10 years
following the natural history of Sjogren's disease progression.
次要结局
- 1. To establish a teaching cohort of patients with SjD or SjD-like conditions treated with conventional treatments 2. To identify patients for other protocols related to Sjogren's Disease(Ongoing)