Lupus Flashcards
(48 cards)
1
Q
All hypersensitivity reactions are immune-mediated tissue injury resulting in a variety of diseases:
3 examples?
A
- Allergies
- Autoimmune disease
- A variety of other inflammatory disease
2
Q
Types of hypersensivty reactions (Gell and Coombs) Describe what is included in the following reactions: 1. Type I? 1 2. Type II? 5 3. Type III? 3 4. Type IV? 2
A
- Immediate (anaphalactic; atopic)
2.
- Cytotoxic-Target cell receptors;
- platelet-thrombocytopenia
- Target fixed tissue antigen;
- Goodpastures,
- M.G.
- Immune complex;
- Vasculitis;
- SLE.
- Cell mediated
a) Tuberculin reaction
b) Cytotoxic cells destroy target cells-allograft rej.
3
Q
- What is SLE?
- Deposition of what? And where?
- Can affect what parts of the body? 10
Is sometimes referred to as the “great imitator” because it affects many different body systems.
A
- Multisystem autoimmune disorder of unknown cause and is strongly associated with various autoantibodies.
- Deposition of antigen-antibody complexes along vascular and tissue basement membranes.
- Skin,
- joints,
- serosal surfaces,
- muscles,
- kidneys,
- heart,
- lung,
- CNS,
- red blood cells and
- platelets.
Is sometimes referred to as the “great imitator” because it affects many different body systems.
4
Q
Influenced by combination of the following:
1. Genetic? 3
- Environmental? 4
A
1. Genetic: Chromosome 6: -HLA-DR3 and linked specificities -DR2 and -DQw1 increase risk by a factor of three!
- Environmental:
- UV light (rash)
- Bacterial and viral infections
- Drugs
- Abnormal stimulation of immune system
5
Q
Factors that affect Lupus:
1. Sex hormones appear to play some role
Most cases of SLE develop after what and before what?
- Drug induced:
Lupus like syndrome
Which drugs? 2
A
- menarche, menopause
- Procainamide
- Hydralazine
6
Q
Pathogenesis of SLE
3 components?
A
- B-lymphocyte hyperactivity with exaggerated antibody production.
- Antigen-antibody complexes deposited along basement membranes of vasculature and tissue.
- Localized inflammatory responses:
7
Q
PP of SLE: Localized inflammatory responses include? 3
Course is characterized by what?
A
- complement
- neutrophil migration
- cell-mediated tissue injury.
exacerbations and remissions.
8
Q
- As in other autoimmune disorders-
the immune system attacks what? - Result in what? 2
- Type III Autoimmune reaction caused by what?
A
- body cells
- tissues.
- inflammation and
- tissue damage.
- antibody-immune complex formation.
9
Q
Most common symptoms of SLE:
1. Systemic? 2
- MOuth and nose? 1
- Muscles? 1
- Joints? 1
- Psychological? 2
- Face? 1
- Pleura? 1
- Pericardium? 1
- Fingers and toes? 1
A
- Low grade fever
- Photosensitivity
- Ulcers
- Muscles
- Arthritis
- Fatigue
- Loss of appetite
- Butterfly rash
- Inflammation
- Inflammation
- poor circulation
10
Q
Clinical Manifestations
1. Constitutional? 5
- Skin? 1
- Systemic? 3
- Digits, Hair and nail? 6
A
- Constitutional
- Fatigue
- Fever
- Weight loss
- Malaise
- Anorexia - Skin
- “butterfly” rash (malar rash) - Systemic
- polyarthritis,
- arthralgias,
- joint pain - Alopecia
- Fingertip lesions
- Periungual erythema
- Nail fold infarcts
- Splinter hemmorhages
- Raynaud’s phenomenon (about 20% of patients)
Polyarthritis is symmetrical, but does not involve articular destruction.
11
Q
Renal features of SLE
2 causes?
A
- Lupus Nephritis
2. Hypertension
12
Q
Lupus nephritis can cause?
2
A
- Glomerulonephitis
2. Nephrotic syndrome
13
Q
Nephrotic syndrome features? 2
A
- Proteinuria
2. Hyaline casts
14
Q
What can be prognostic for renal manifestations of SLE?
A
Renal biopsy
15
Q
SLE: Renal biopsy can be prognostic showing what?
4
What kind of renal failure is most common?
A
- glomerular sclerosis
- fibrous crescents
- interstitial fibrosis
- tubular atrophy = poor outcome.
Chronic renal failure more common than acute.
16
Q
What will the cytology show on the renal biopsy for lupus nephritis?
A
“wire loop” nephritis
17
Q
Ocular features of SLE
5
A
- Conjuntivitis
- Photophobia
- Transient or permanent monocular blindness
- Blurred vision
- Cotton wool spots on retina
18
Q
Musculoskeletal features SLE
3
A
- Transient polyarthritis with symmetric involvement
small and large joints. - Osteonecrosis of hips.
- Fibromyalgia often present.
19
Q
Musculoskeletal features of SLE: Transient polyarthritis with symmetric involvement
small and large joints.
- On examination of joints what will you not find?
- 10% develop what kind of deformities?
- What are not present?
A
- No signs of inflammation on examination of joints.
- 10% develop rheumatoid-like hand deformities.
- Bony erosions not present.
20
Q
Pulmonary features SLE
Occurs in 40-50% of patients
6
A
- Transient basilar pneumonic infiltrates = “lupus pneumonitis”
- Pleural effusions
- Pleuritis
- Bronchopneumonia
- Restrictive lung disease
- Rare: alveolar hemorrhage with massive hemoptysis, death
21
Q
Pulmonary features SLE: Transient basilar pneumonic infiltrates = “lupus pneumonitis” will present how? 3
A
- Nonproductive cough
- Dyspnea
- Hypoxemia
22
Q
Cardiac features of SLE
Occurs in 30-40% of patients
4
A
- Pericarditis
- Myocarditis
- Increased muscle enzymes
- Libman-Sacks endocarditis
23
Q
Cardiac features of SLE
1. Myocarditis features? 4
- Increased muscles enzymes: Which ones? 2
- Libman-Sacks endocarditis characterized by what?
A
- Tachycardia
- Ventricular arrhythmia
- Conduction problems
- CHF, Cardiomyopathy
- MB or CPK
- -V. surface vegetations on valves
Vegetation breakoff may allow colonization
24
Q
Serositis of SLE
3 examples
What would diagnose this? 2
A
- Pleuritis
- Pericarditis
- Peritonitis
- Fluid: less than 3000 WBC/ mm3 = monocytes and lymphocytes
- Reduced complement
25
Vascular features of SLE
| 4
1. Raynaud’s phenomenon
2. Arterial or venous thrombosis
3. Lupus vasculitis
4. Livedo reticularis
26
What is lupus vasculitis?
Palpable purpuric lesions on the shins in a patient with lupus and necrotizing vasculitis of the skin, kidney, and brain.
27
What is Livedo reticularis?
Patient with lupus and antiphospholipid antibodies with livedo reticularis (manifested by a reddish-cyanic, reticular pattern of the skin) which has resulted in ulcer formation.
28
GI Tract Symptoms SLE
| 3
1. Transient, nonspecific abdominal pain.
2. Increased incidence of primary biliary cirrhosis with SLE.
3. Vasculitis of mesentery can cause infarction or perforation of bowel with high mortality.
29
CNS Features SLE
1. Whats the most common neurophysical symptom?
2. Other symtpoms? 8
3. Why at higher risk for stroke?
1. Severe headaches, most common neuropsychological symptom, sometimes with scotomata like migraines.
2.
- Confusion,
- memory deficits,
- disorientation,
- hypomania,
- delirium
- schizophrenia.
- Grand mal seizures,
- temporal lobe seizures.
3. Stroke: anti-phospholipid antibodies.
30
1. Specific SLE-related deaths, such as those due to nephritis, usually occur within what time period?
2. What are common etiologies of late mortality?
3. What is now the most common cause of death in early active SLE?
1. the first 5 years of symptom onset.
2.
-Cardiovascular disease and
-malignancy,
which may be related to chronic inflammation and cytoxic therapies, are common etiologies of late mortality.
3. Infectious complications related to active SLE and immunosuppressive treatment are now
31
SLE Laboratory
| 4
1. CBC
2. ESR and CRP
3. UA
4. Antibody testing
32
What will the CBC show for SLE?
| 4
1. Anemia
2. Leukopenia
3. Thrombocytopenia
4. Prolonged PTT
33
CBC in SLE
1. Anemia: Which kinds? 3
2. Leukopenia: which kind and expressed what?
3. Thrombocytopenia: What levels?
4. Prolonged PTT: from what?
5. This could be a false positive test for what?
CBC
1. Anemia
- Normocytic
- Normochromic
- Hemolytic
2. Leukopenia
- Lymphocytopenia common,
- indicative of disease activity
3. Thrombocytopenia
- 50-100 K
4. Prolonged PTT
- from antiphospholipid antibodies
5. false positive tests for syphilis
34
ESR and CRP in SLE will be?
UA might show? 3
1. ESR and CRP
- Both elevated
2. UA
- Proteinuria
- RBC’s/WBC’s
- Cellular or hyaline casts
35
What antibody tests would we do for lupus?
| 8
1. ANA – (especially rim, cartwheel or peripheral) sensitivity 99%, spec 49%
2. Anti dsDNA (anti-deoxyribonuclic acid) **[double stranded]
3. Anti ssDNA (Anti-deoxyribonuclic acid)
4. Anti ENA [anti-extractable nuclear antigen]
- Anti SM **
- Anti RNP
5. Elevated nonspecific IgG, IgM levels
6. Reduced complement levels (C3, C4)
7. Decreased IgA levels
8. Anti-histone - Drug-induced lupus (DIL) ANA antibodies often this type
36
ANA shapes?
| 4
1. Rim or cartwheel
2. Diffuse
3. Speckled
4. Nucleolar
37
Differential Diagnosis SLE
| 13
1. Antiphospholipid Syndrome
2. Fibromyalgia
3. Hepatitis C
4. Lyme disease
5. Lymphoma, B-cell
6. Mixed Connective-Tissue
7. Disease
8. Polyarteritis Nodosa
9. Rheumatic Fever
10. Rheumatoid Arthritis
11. Scleroderma
12. Serum sickness
13. TTP
38
CRITERIA FOR DIAGNOSING SYSTEM LUPUS ERYTHEMATOSUS
| 11
1. Characteristic rash across the cheek
2. Discoid lesion rash
3. Photosensitivity
4. Oral ulcers
5. Arthritis
6. Inflammation of membranes in the lungs, the heart, or the abdomen
7. Evidence of kidney disease
8. Evidence of severe neurologic disease
9. Blood disorders, including low red and white blood cell and platelet counts
10. Immunologic abnormalities
11. Positive antinuclear antibody (ANA)
Note: A patient must experience four of the criteria before a doctor can classify the condition as SLE. These criteria, proposed by the American College of Rheumatology, are not to be relied upon solely for diagnosis, however.
39
Physical findings: SLE
| 4
1. Butterfly (malar rash)
2. Hypertension
3. Alopecia
4. Discoid lesions
40
Diagnosis SLE
Labs?
5
1. ANA (particularly cartwheel, rim or peripheral)
2. dsDNA
3. anti Sm ribonuclear protein
4. Hypocomplementemia.
5. UA
41
What are we looking for with the UA with SLE? 4
1. WBC
2. RBC
3. Proteinura
4. Hyaline casts
42
Treatment for SLE is aimed at?
| 3
Treatment aimed at:
1. Reducing inflammation
2. Suppressing the immune system
3. Closely following patients clinically to identify disease features as early as possible.
43
Patient education and prevention of disease flares:
| 5
1. Sunscreen and protective clothing
2. Protective, warm clothing
3. Avoidance of vasoconstrictive drugs
4. Psychological support
5. Routine immunization
44
Pharm treatment for SLE?
| 6
1. Glucocorticoids
2. Antimalarials
3. Immunosuppresants
4. Iv Immunoglobulin
5. Plasmapheresis (lupus nephritis)
6. Belimumab (FDA approved 2011) fully human
monoclonal antibody. (benlysta)
45
Drug Induced Lupus Syndromes
1. Primarily symptoms where? 3
2. What makes this different from? 4
3. Treatment?
1. Primarily
- musculoskeletal,
- pulmonary, and
- polyserositic symptoms.
2.
- Renal and neurologic disease uncommon.
- Antibodies themselves not harmful.
- No hypocomplementemia.
- Anti-nuclear antibodies present, but return to normal with withdrawal of drug.
3. Reversal with discontinuation of drug
46
Drug Induced Lupus Syndromes
Name the drugs in the following classes that could cause this?
1. Antihypertensives? 2
2. Antiarrhythmics? 2
3. Anticonvulsants? 4
Others?
1.
- Hydralazine *
- Methyldopa *
2.
- Procainamide *
- Quinidine *
3.
- Dilantin
- Zarontin
- Primidone
- Tegratol
```
Miscellaneous:
INH *
Sulfanamides
PCN
Thorazine
PTU
Tagamet
Levadopa
B-Blockers
```
47
1. What is discoid lupus?
2. Involves plaque-like lesions of what areas? 3
3. First appears as what?
4. Later?
5. ___% of discoid lupus patients have disease that involves the skin.
1. Chronic cutaneous lupus
2. head, scalp and neck.
3. First appear as red, swollen patches or skin
4. Later
- scarring,
- depigmentation,
- plugging of hair follicles.
5. 90
48
Discoid therapies?
| 3
Therapy with
1. sunscreens,
2. topical corticosteroids, and
3. antimalarial agents is usually effective.
