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SLE Flashcards

1
Q

Who are at the most risk for SLE

A

Women (9:1 ratio to men) (especially in REPRODUCTIVE years)

African, Native and Hispanic American

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2
Q

Examples of Autoimmune diseases

A 
Hashimotos (thyroid)
Multiple Sclerosis (CNS)
Pernicious Anemia (stomach)
Addisons (adrenal)
DM Type 1 (pancreas)
Pemphigus Vulgaris (skin)
Pleuritis*
Pericarditis*
Glomerulonephritis*
Raynaud Phenomenon*
Arthritis*

*Systemic

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3
Q

Examples of Immune Dysregulation involved with SLE

A

B-cells: defective selection and autoantibody production
T-cells: Inc. Th17 and dec. Tregs
Dendritic cells: lots of Interferon and activate autoreactive T/B cells

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4
Q

(Natural/Pathogenic) autoantibodies involve IgM effectively clear cellular debris in healthy individuals

A

Natural

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5
Q

(Natural/Pathogenic) autoantibodies involve IgG to form immune complexes and directly target cells through cross-reactivity with other antigens

A

Pathogenic

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6
Q

HALLMARK autoantibodies for SLE; SENSITIVE, not specific, because it is seen in many autoimmune disorders and some healthy patients; Autoantibodies against various components of the cell nucleus; best seen with Immunofluorescence

A

Anti-nuclear Antibodies

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7
Q

ANA are (sensitive/specific) for SLE

A

Sensitive (nearly all of SLE patients have it, but so do many other autoimmune diseases)

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8
Q

Autoimmune diseases that are associated with Anti-Nuclear Antibodies

A 
SLE (99%)
Scleroderma (95%)
Hashimotos (50%)
IPF (50%)
Normal Patients (4%)
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9
Q

Best detection method for Anti-Nuclear Antibodies

A

Immunofluorescence

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10
Q

Anti-DNA and Smith antibodies are highly (sensitive/specific) for SLE

A

Specific

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11
Q

SPECIFIC autoantibody for SLE; is highly associated with NEPHRITIS

A

Anti-ds DNA

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12
Q

Autoantibody for SLE; highly associated with Sicca and Neonatal Lupus

A

Anti-SSA (Ro)

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13
Q

Autoantibody for SLE; highly associated with Arthritis, Myositis and Lung Disease

A

Anti-RNP

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14
Q

Methods for detecting Anti-ds DNA antibodies

A 
Crithidia assay (kinetoplast with dsDNA) (specific)
Farr Assay (sensitive)
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15
Q

Signs/Symptoms of SLE

A 
Malar rash (cheeks and nose)
Discoid rash (red, disk-shaped patch)
Photosensitivity
Oral Ulcers
Arthritis
Kidney, Neuro or Blood Disorder

(need >4 or biopsy with positive ANA/Anti-DNA)

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16
Q

Diagnostic Criteria for SLE

A

> 4 signs/symptoms
OR
Biopsy-proven nephritis with positive ANA/Anti-DNA

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17
Q

Widespread muscular pain; seen in >30% of SLE patients; focuses on major joints (knees, elbows, neck, etc.)

A

Fibromyalgia

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18
Q

CNS manifestations of SLE

A 
AMS
Anxiety/Psychosis
Depression
CVA
Seizures
Polyneuropathy
Guillian Barre
Myasthenia Gravis
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19
Q

MAJOR risk of early SLE disease, especially in young onset SLE; may be due to thrombosis, dissection, atherosclerosis and fibromuscular dysplasia; 90% are ischemic

A

CVA

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20
Q

90% of SLE induces CVAs are (Ischemic/Hemorrhagic)

A

Ischemic

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21
Q

GI manifestations of SLE

A

Dysphagia/Odynophagia
GERD (assoc. with Raynauds)
Mesenteric Vasculitis/Ischemia

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22
Q

Hematologic manifestations of SLE

A

Anemia (Chronic disease, iron def., autoimmune)
Leukopenia (antibody mediated)
ITP

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23
Q

Examples of Thrombocytopenias in SLE

A

ITP (most common)
Antiphospholipid Antibody Syndrome
TTP

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24
Q

Cause of Thrombocytopenia in SLE; acquired disorder with autoantibodies against phospholipids; detected by presence of “lupus anticoagulant”; paradoxically causes THROMBOSES, abnormal blood flow and pregnancy loss; treat with anticoagulation

A

Antiphospholipid Antibody Syndrome

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25
Treatment for Antiphospholipid Antibody Syndrome
Anticoagulation
26
Examples of SLE antibodies associated with Antiphospholipid Antibody Syndrome (3 total)
Anti-cardiolipin Anti-B2-glycoprotein Lupus anticoagulant
27
Pulmonary manifestations of SLE
Restrictive/Interstitial disease Pleuritis + Effusion Pulmonary Embolism Pulmonary HTN and Hemorrhage
28
Lupus Pneumonitis can look just like Infectious Pneumonia due to fever, cough, alveolar damage and edema (True or False)
True (requires lavage and biopsy to confirm)
29
Most life-threatening Pulmonary manifestation of SLE
Pulmonary Hemorrhage (hemoptysis in only 50% of patients)
30
Cardiac manifestations of SLE
Pericarditis Myocarditis Libman Sacks (verrucous/marantic) Endocarditis
31
Fancy names for SLE induced Endocarditis
Libman Sacks Verrucous Marantic Sterile/Non-bacterial
32
Leading causes of Mortality in SLE
Heart disease Infection Malignancy
33
Treatments for SLE
Sun avoidance Hydroxychloroquine (antimalarial) Corticosteroids
34
Why is the antimalarial Hydroxychloroquine used for SLE
Antagonist activity against TLRs 3, 7, 8 and 9 dec. the activation of the innate immune system (dendritic cells)
35
Examples of Immunosuppressives used in SLE
``` Cyclophosphamide Azathioprine Mycophenolate Methotrexate Leflunomide Cyclosporine Belimumab Rituximab Abatacept ```
36
(Nephrotic/Nephritic) syndrome is characterized by proteinuria, edema and hyperlipidemia
Nephrotic
37
(Nephrotic/Nephritic) syndrome is characterized by hematuria, HTN and oliguria
Nephritic
38
When should you obtain a kidney biopsy in SLE
Inc. serum creatinine (no other cause) | Proteinuria >1g/day
39
Renal manifestations of SLE
Nephritis Thombotic Microangiopathy Tubulo-Interstitial Nephritis (Type 1 RTA) ANCA overlap with Lupus
40
Treatments for SLE (besides immunosuppression)
ACEi/ARBs (Renal) Statins (CAD) Aspirin and/or Warfarin (APS) PPI or H2 Blockers (GERD)
41
Nephrotic/Nephritic syndromes associated with SLE
Membranous | Proliferative Lupus Nephritis
42
Complement abnormality seen with SLE
Low C3 and C4
43
Proteins produced in response to an antigen; function as chemical messengers for regulating the innate and adaptive immune systems
Cytokines
44
Cytokine made by T and NK cells; responsible for activation of macrophages
IFN-y
45
Cytokine responsible for the inhibition of T cells and differentiation of Treg cells
TGF-b
46
Cytokine responsible for T cell proliferation
IL-2
47
Cytokine responsible for the stimulation of acute inflammation
IL-17
48
Cytokines responsible for stimulating production of IgE and eosinophils
IL-4 and 5
49
Cytokine responsible for the recruitment of neutrophils
IL-8
50
Cytokine responsible for the stimulation of Bone marrow stem cells
IL-3
51
Hypersensitivity Reaction: characterized by IMMEDIATE hypersensitivity and allergic response via mast cells, IgE and eosinophils
Type 1
52
Hypersensitivity Reaction; characterized by IgM/G antibodies against CELL SURFACE or matrix antigens; opsonization and phagocytosis of cells
Type 2
53
Hypersensitivity Reaction: characterized by Immune COMPLEXES of circulating antigens depositing in vascular BASEMENT MEMBRANES; complement-mediated recruitment of leukocytes and resultant inflammation
Type 3
54
Hypersensitivity Reaction: characterized by T-cell recruitment and direct target cell lysis; DELAYED onset
Type 4
55
SLE is an example of Type (1/2/3/4) Hypersensitivity
3 (antibody complex mediated)
56
Type 1 Diabetes Mellitus is an example of Type (1/2/3/4) Hypersensitivity
4 (T-cell mediated)
57
Examples of Type 4 Hypersensitivity Reactions
``` Multiple Sclerosis Rheumatoid Arthritis Type 1 DM Crohn's Contact sensitivity (poison ivy) ```
58
(Positive/Negative) Thymic selection is when T-cells that are at least somewhat reactive to MHC presentation
Positive
59
(Positive/Negative) Thymic selection is when any T cells that react to self-antigens with high affinity are killed; prevents possible auto-reactive T cells
Negative
60
MHC (1/2) present proteins from intracellular pathogens to CD8 T cells
MHC 1
61
MHC (1/2) present proteins from extracellular pathogens to CD4 T cells
MHC 2
62
Treg cells need the transcription factor _________ for proper function
FoxP3
63
How do Treg cells suppress the immune response (3 ways)
Produce inhibitory cytokines Express CTLA-4 (inhibit T-cell activation) Express IL-2 receptor (capture IL-2)
64
The secondary signal (B7/CD28) is located on the APC
B7
65
The secondary signal (B7/CD28) is located on the T cell
CD28

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