Immune System Related Kidney Disease

Study Overview
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Brief Summary
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Kidney diseases related to the immune system include, nephrotic syndrome, glomerulonephritis, membranous nephropathy, lupus nephritis, and nephritis associated with connective tissue disorders. This study will allow researchers to admit and follow patients suffering from autoimmune diseases of the kidney. It will attempt to provide information about the causes and specific abnormalities associated with autoimmune kidney disease. Patients with kidney disease as a result of their immune system, and patients with diseases of the immune system who may later develop kidney disease, will be potential subjects for this study. Patients will undergo a history and physical examination, and standard laboratory test to more closely understand the causes, signs, symptoms, and responses to medication of these diseases. Based on these evaluations the patients may qualify as candidates for other experimental studies. At any time these patients may be asked to submit blood or urine samples for further research.

Detailed Description
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Study Description: This study evaluates participants with known or suspected immunologically-mediated kidney diseases, including but not limited to nephrotic syndrome, glomerulonephritis, membranous nephropathy, lupus nephritis, and nephritis associated with other systemic or connective tissue disorders. Participants who have immunologically-mediated diseases with potential for kidney disease will be evaluated as well. Studies will include characterization of the clinical and laboratory features of these disorders, studies of natural history and complications, evaluation of responses to standard treatment. Blood and urine samples will be collected for immunological research conducted under this protocol. Objectives: To facilitate the program of research on immunologically-mediated kidney diseases: Better understand rare forms of immunologically-mediated renal disorders, those with atypical renal disease, or those whose renal diseases have not been fully characterized. Enrolled participants will serve as a catalyst for development of new therapeutic interventions. Exploratory: To conduct further research to fully characterize immunologicallymediated kidney diseases: Development of assays for novel antibodies. Search for additional antibodies involved in the pathogenesis of membranous nephropathy. Assess changes in metabolic and endocrine function during nephrotic state and during remission. Identify disease-causing memory B cells in participants with membranous nephropathy and correlation with membranous nephropathy relapse. Assess biological effects of phospholipase A2-receptor antibody epitope on the isolated memory B-cells. Computational analysis of renal structures which may provide predictive method that would discriminate progressors from non-progressors beyond sclerosis in membranous nephropathy.

Official Title
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Studies of Immunologically-Mediated Kidney Diseases

Conditions
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Kidney Disease, Nephrotic Syndrome

Participation Criteria
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Eligibility Criteria
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INCLUSION CRITERIA: Male or female participants greater than or equal to 18 years of age and children 10-17 years of age. Participants with known, proven, or suspected immunologically-mediated kidney diseases, including but not limited to nephrotic syndrome, glomerulonephritis, membranous nephropathy, lupus nephritis, and nephritis associated with other systemic or connective tissue disorders. Participants who have Immunologically-mediated diseases with the potential for kidney disease. Ability of the participant or guardian to understand and the willingness of the participant or guardian to provide written informed consent. EXCLUSION CRITERIA: Concomitant medical problems which would confound the interpretation of studies of the immunologic kidney disorder. Concomitant medical, surgical or other conditions for which inadequate facilities or funds are available to support their care at the NIH.

Ages Eligible for Study
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Minimum Age: 10 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Patients<br>Patients with known or suspected immunologically-mediated kidney diseases or immunologically-mediated diseases with potential for kidney disease. | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Provision of biospecimens | Patients with autoimmune diseases for whom there are no current experimental treatment protocols can be admitted for study and follow-up; they will provide a ready pool of candidates who can be asked to provide blood specimens for ongoing laboratory studies. | ongoing | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Kidney Disease, Chronic Kidney Disease, Natural History