Natural History of Coronavirus Disease (COVID-19) in Systemic Autoimmune Diseases
Natural History of Coronavirus Disease (COVID-19) in Systemic Autoimmune Diseases
Background:

 Viral infections such as COVID-19 may lead to flare-ups in people with systemic autoimmune
 diseases (SAD). These infections may also change the function of their immune system and/or
 cause problems with their blood vessels. Researchers want to learn how people with SAD
 respond to treatments or vaccines for COVID-19.

 Objective:

 To understand how COVID-19 affects inflammation, the immune system, and blood vessels in
 adults and children with autoimmune diseases.

 Eligibility:

 People ages 15 and older who have been diagnosed with an autoimmune disease or are a healthy
 volunteer

 Design:

 Participants will have a screening visit. This will include:

 Medical history and physical exam

 EKG

 Chest x-ray

 COVID-19 test. A swab will be put in the participant s nose or the back of their mouth.

 Blood and urine tests

 Participants will be placed into 1 of 4 groups:

 1. Those with previously documented COVID-19 infection or COVID vaccination

 2. Those with a recently known COVID-19 exposure or vaccination

 3. Those with no known COVID-19 exposure or vaccination

 4. Those who developed an acute COVID-19 infection

 Depending on their group, participants will have 1 to 5 more visits. These will occur over 12
 to 18 months. Visits may include:

 FDG PET/CT scan. Participants will lie in a doughnut-shaped machine. The machine creates
 pictures of the body. For the scan, they will have a radioactive substance injected into
 their arm through an IV.

 Kidney function tests

 Non-invasive vascular studies test. These tests are similar to what it feels like to have
 blood pressure checked.
This is an observational study to characterize how COVID-19 modulates systemic inflammation,
 autoimmune features and vasculopathy in adult and pediatric patients with a prior diagnosis
 of systemic autoimmunity, and their overall outcomes including response to potential
 antiviral treatments or vaccines.

 Primary Objective:Characterize how COVID-19 modulates systemic inflammation, autoimmunity
 features, organ damage and vasculopathy in adult and pediatric patients with a previous
 diagnosis of systemic autoimmunity. Assess how subjects with systemic autoimmunity respond to
 COVID-19 regarding antiviral and/or proinflammatory responses and overall outcomes

 Secondary Objectives:Understand prevalence and severity of COVID-19 in individuals with
 autoimmune diseases, and the variables that associate/predict these responses.

 Primary Endpoint: Immune dysregulation and vasculopathy modulation following COVID-19.

 Secondary Endpoints: Immunologic and clinical response to potential antiviral/immune
 modulator treatments and/or vaccines that are used during COVID19 for clinical purposes.
 Overall outcome following exposure to COVID19 (response to virus, status of rheumatologic
 disease)

 200 subjects ages 15 years old and above with systemic autoimmune diseases and 60 healthy
 volunteers ages 15 years old and above.
-  INCLUSION CRITERIA:

 In order to be eligible to participate in this study, an individual must meet all of the
 following criteria as per respective group:

 1. Stated willingness to comply with all study procedures and availability for the
 duration of the study

 2. Male or female age greater than or equal to 15 years old with no upper age limit.

 3. Ability of subject to understand and the willingness to sign a written informed
 consent and/or assent document.

 4. For Healthy Volunteers Group:

 -  Age greater than or equal to 15 with no upper age limit.

 -  No history of autoimmune diseases and in good general health as evidenced by
 medical history.

 5. For symptomatic COVID-19 group must have laboratory evidence (positive PCR for
 SARSCOV-2 or other test developed after this proposal gets approved and is available
 through the Clinical Center) and one of the signs and symptoms associated with
 COVID-19 infection (e.g. fever or chills, cough, shortness of breath or difficulty
 breathing, fatigue, muscle or body aches, headache, new loss of taste or smell, sore
 throat, congestion or runny nose, nausea or vomiting, diarrhea).

 6. For post COVID-19 study visits must have laboratory evidence (e.g. positive PCR for
 SARS- COV-2, antibody against SARS-COV-2 or other test developed after this proposal
 gets approved) of current or prior exposure to COVID-19. Alternatively, patients
 should have documented evidence of having received one of the SARS-COV-2 vaccines that
 may become available during the study.

 7. For subjects with known COVID exposure, they must fulfill one of the following
 criteria:

 -  A minimum of 28 days has passed since the initial positive test date AND B.
 Resolution of fever was greater than or equal to7 days ago (without the use of
 fever-reducing medications) AND C. Resolution of respiratory symptoms was greater
 than or equal to7 days ago, OR

 -  A minimum of 10 days has passed since the initial positive test AND B. A minimum
 of 2 consecutive oropharyngeal swabs OR 2 consecutive nasopharyngeal swabs or 2
 saliva testing collected greater than or equal to 24 hours apart that are
 negative for SARS CoV-2 PCR A positive PCR requires restarting the series of
 swabs (wait 5 days to retest)

 8. Specific Inclusion Criteria for Systemic Autoimmunity Diseases Group:

 -  Age greater than or equal to 15 years with no upper age limit

 -  Systemic lupus erythematosus (SLE): Meets at least 4 of 11 modified American
 College of Rheumatology (ACR) (1997) Revised Criteria for the Classification of
 Systemic Lupus Erythematosus or Systemic Lupus International Collaborating
 Clinics (SLICC) classification criteria for systemic lupus erythematosus.

 -  Anti-neutrophil cytoplasmic antibody associated vasculitis (AAV): Meet Revised
 1990 ACR criteria for Granulomatosis with polyangiitis (GPA) or the 2012 Chapel
 Hill Nomenclature for microscopic polyangiitis (MPA).

 -  Idiopathic inflammatory myopathies (IIM):Meets the 2017 EULAR/ACR Classification
 Criteria for Adult and Juvenile idiopathic inflammatory myopathies and their
 major subgroups.

 -  Primary Sjogren's syndrome (SS): Meets 2016 ACR criteria.

 -  Progressive systemic sclerosis (PSS): ACR/EULAR 2013 Classification criteria.

 -  Immunologically mediated kidney diseases (IKD): Based on results from kidney
 biopsy consistent with immunologically mediated nephrotic syndrome and/or
 glomerulonephritis

 -  Rheumatoid arthritis (RA): Meet the 2010 ACR/EULAR Classification Criteria.

 -  Systemic autoimmunity syndrome not otherwise specified (SA-NOS): Patients not
 utoimmune disease but with evidence of autoantibodies and clinical features
 suggestive of a systemic autoimmune disorder.

 EXCLUSION CRITERIA:

 An individual who meets any of the following criteria will be excluded from participation
 in this study:

 1. Pregnant and lactating subjects will be excluded because pregnancy and lactation
 modify immune responses and may interfere with correlations.

 2. Concomitant medical problems which would confound the interpretation of studies
 gathered by this protocol. Included in this is the presence of HIV in the blood,
 active malignancies, or other significant medical conditions that may interferes with
 interpretation of studies.

 3. Concomitant medical, surgical or other conditions for which inadequate facilities are
 available to support their care at NIH.

 4. Inability or unwillingness to comply with follow up requirements (e.g. distance,
 social, physical limitations).

 5. Any comorbidity of 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. Patient requiring urgent and/or acute medical care, surgical or
 other procedures)

 6. Unwilling to participate in research studies or to provide research samples or data

 7. Exclusion criteria for the (optional) vascular studies:

 -Healthy volunteers with known history of coronary artery disease, peripheral vascular
 disease or atherosclerosis.

 8. Exclusion criteria for the (optional) FDGPET/CT scan:

 -Individuals younger than 18 years old will be excluded given the radiation exposure

 9. Subjects with SAD and Healthy volunteers younger than 18 years old will be excluded
 from chest x-rays unless clinically indicated.