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
Q

Treatment for Antiphospholipid Antibody Syndrome

A

Anticoagulation

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26
Q

Examples of SLE antibodies associated with Antiphospholipid Antibody Syndrome (3 total)

A

Anti-cardiolipin
Anti-B2-glycoprotein
Lupus anticoagulant

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27
Q

Pulmonary manifestations of SLE

A

Restrictive/Interstitial disease
Pleuritis + Effusion
Pulmonary Embolism
Pulmonary HTN and Hemorrhage

28
Q

Lupus Pneumonitis can look just like Infectious Pneumonia due to fever, cough, alveolar damage and edema (True or False)

A

True (requires lavage and biopsy to confirm)

29
Q

Most life-threatening Pulmonary manifestation of SLE

A

Pulmonary Hemorrhage (hemoptysis in only 50% of patients)

30
Q

Cardiac manifestations of SLE

A

Pericarditis
Myocarditis
Libman Sacks (verrucous/marantic) Endocarditis

31
Q

Fancy names for SLE induced Endocarditis

A

Libman Sacks
Verrucous
Marantic
Sterile/Non-bacterial

32
Q

Leading causes of Mortality in SLE

A

Heart disease
Infection
Malignancy

33
Q

Treatments for SLE

A

Sun avoidance
Hydroxychloroquine (antimalarial)
Corticosteroids

34
Q

Why is the antimalarial Hydroxychloroquine used for SLE

A

Antagonist activity against TLRs 3, 7, 8 and 9 dec. the activation of the innate immune system (dendritic cells)

35
Q

Examples of Immunosuppressives used in SLE

A
Cyclophosphamide
Azathioprine
Mycophenolate
Methotrexate
Leflunomide
Cyclosporine
Belimumab
Rituximab
Abatacept
36
Q

(Nephrotic/Nephritic) syndrome is characterized by proteinuria, edema and hyperlipidemia

A

Nephrotic

37
Q

(Nephrotic/Nephritic) syndrome is characterized by hematuria, HTN and oliguria

A

Nephritic

38
Q

When should you obtain a kidney biopsy in SLE

A

Inc. serum creatinine (no other cause)

Proteinuria >1g/day

39
Q

Renal manifestations of SLE

A

Nephritis
Thombotic Microangiopathy
Tubulo-Interstitial Nephritis (Type 1 RTA)
ANCA overlap with Lupus

40
Q

Treatments for SLE (besides immunosuppression)

A

ACEi/ARBs (Renal)
Statins (CAD)
Aspirin and/or Warfarin (APS)
PPI or H2 Blockers (GERD)

41
Q

Nephrotic/Nephritic syndromes associated with SLE

A

Membranous

Proliferative Lupus Nephritis

42
Q

Complement abnormality seen with SLE

A

Low C3 and C4

43
Q

Proteins produced in response to an antigen; function as chemical messengers for regulating the innate and adaptive immune systems

A

Cytokines

44
Q

Cytokine made by T and NK cells; responsible for activation of macrophages

A

IFN-y

45
Q

Cytokine responsible for the inhibition of T cells and differentiation of Treg cells

A

TGF-b

46
Q

Cytokine responsible for T cell proliferation

A

IL-2

47
Q

Cytokine responsible for the stimulation of acute inflammation

A

IL-17

48
Q

Cytokines responsible for stimulating production of IgE and eosinophils

A

IL-4 and 5

49
Q

Cytokine responsible for the recruitment of neutrophils

A

IL-8

50
Q

Cytokine responsible for the stimulation of Bone marrow stem cells

A

IL-3

51
Q

Hypersensitivity Reaction: characterized by IMMEDIATE hypersensitivity and allergic response via mast cells, IgE and eosinophils

A

Type 1

52
Q

Hypersensitivity Reaction; characterized by IgM/G antibodies against CELL SURFACE or matrix antigens; opsonization and phagocytosis of cells

A

Type 2

53
Q

Hypersensitivity Reaction: characterized by Immune COMPLEXES of circulating antigens depositing in vascular BASEMENT MEMBRANES; complement-mediated recruitment of leukocytes and resultant inflammation

A

Type 3

54
Q

Hypersensitivity Reaction: characterized by T-cell recruitment and direct target cell lysis; DELAYED onset

A

Type 4

55
Q

SLE is an example of Type (1/2/3/4) Hypersensitivity

A

3 (antibody complex mediated)

56
Q

Type 1 Diabetes Mellitus is an example of Type (1/2/3/4) Hypersensitivity

A

4 (T-cell mediated)

57
Q

Examples of Type 4 Hypersensitivity Reactions

A
Multiple Sclerosis
Rheumatoid Arthritis
Type 1 DM
Crohn's
Contact sensitivity (poison ivy)
58
Q

(Positive/Negative) Thymic selection is when T-cells that are at least somewhat reactive to MHC presentation

A

Positive

59
Q

(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

A

Negative

60
Q

MHC (1/2) present proteins from intracellular pathogens to CD8 T cells

A

MHC 1

61
Q

MHC (1/2) present proteins from extracellular pathogens to CD4 T cells

A

MHC 2

62
Q

Treg cells need the transcription factor _________ for proper function

A

FoxP3

63
Q

How do Treg cells suppress the immune response (3 ways)

A

Produce inhibitory cytokines
Express CTLA-4 (inhibit T-cell activation)
Express IL-2 receptor (capture IL-2)

64
Q

The secondary signal (B7/CD28) is located on the APC

A

B7

65
Q

The secondary signal (B7/CD28) is located on the T cell

A

CD28