S2: Ch 6 Connective Tissue 1-0

Front 1

1. Pathophys of SLE?

Back 1

a. Autoimmune- Causes inflammation and tissue damage involving multiple organ systems.
b. Has Genetic, environmental, and hormonal factors.
c. Pathophys involves autoantibody production, deposition of immune complexes, complement activation, and accompanying tissue destruction/vasculitis.

Front 2

2. 4 types of SLE?

Back 2

1. Spontaneous SLE
2. Discoid lupus
3. Drug-induced lupus
4. ANA Negative lupus

Front 3

3. Associated findings w/ANA-negative lupus?

Back 3

a. Arthritis
b. Raynaud’s
c. Subacute cutaneous lupus
d. Risk of neonatal lupus in infants of affected women.

Front 4

4. Serology results w/ANA negative lupus?

Back 4

a. Ro (anti-SS-A) antibody –positive
b. ANA-negative.

Front 5

5. Overview of systems affected by SLE (just to prep)?

Back 5

1. Constitutional symptoms
2. Cutaneous
3. Musculoskeletal
4. Cardiac
5. Pulmonary
6. Haematologic
7. Renal
8. Immunologic
9. GI
10. CNS

Front 6

6. Constitutional symptoms of SLE?

Back 6

a. Fatigue (often the sign of an impending exacerbation and prominent finding in most patients
b. Malaise
c. Fever
d. Weight loss

Front 7

7. Cutaneous symptoms of SLE?

Back 7

a. Butterfly rash- 1/3 of pts
b. Photosensitivity
c. Discoid lesions (erythematous raised patches w/keratotic scaling)
d. Oral or nasopharyngeal ulcers
e. Alopecia
f. Raynaud’s phenomenon (vasospasm of small vessels when exposed to cold, usually in fingers)- found in 20% of cases.

Front 8

8. Musculoskeletal symptoms of SLE?

Back 8

a. Joint pain –May be 1st sx of disease- found in 90% of pts.
b. Arthritis-inflammatory and symmetric, not erosive as in RA.
c. Arthralgias
d. Myalgias w/or w/o myositis.

Front 9

9. Cardiac symptoms of SLE?

Back 9

1. Pericarditis
2. Endocarditis (Libman-Sacks endocarditis is a serious complication)
3. Myocarditis

Front 10

10. Pulmonary complications of SLE?

Back 10

a. Pleuritis (most common pulmonary finding)
b. Pleural effusion
c. Pneumonitis (may lead to fibrosis)
d. Pulmonary HTN (rare)

Front 11

Haematologic complications of SLE?

Back 11

a. Haemolytic anaemia w/anaemia
b. Or
c. Reticulocytosis of chronic disease
d. Leukopenia
e. Lymphopenia
f. Thrombocytopenia

Front 12

12. Renal complications of SLE?

Back 12

a. Proteinuria >0.5g/day (may have nephrotic syndrome)
b. Cellular casts
c. Glomerulonephritis (may have haematuria)
d. Azotemia
e. Pyuria
f. Uraemia
g. HTN

Front 13

13. Immunologic complications of SLE?

Back 13

a. Impaired immune response due to many factors, including:
1. Autoantibodies to lymphocytes
2. Abnormal T cell function
3. Immunosuppressive medications

Front 14

14. GI Complications of SLE?

Back 14

a. N/V
b. Dyspepsia
c. Dysphagia
d. Peptic Ulcer Disease

Front 15

15. CNS Complications of SLE?

Back 15

a. Seizures
b. Psychosis (may be subtle)
c. Depression
d. HA
e. TIA
f. CVA

Front 16

16. Other Complications of SLE?

Back 16

a. Conjunctivitis and an increased incidence of Raynaud’s phenomenon and Sjogren’s syndrome.

Front 17

17. Who is most commonly affected by SLE?

Back 17

a. Women of childbearing age account for 90% of cases.
b. Blacks>whites
c. Very mild in elderly pts; more severe in children
d. Usually appears in late childhood or adolescence.

Front 18

18. Clinical findings associated w/neonatal lupus?

Back 18

1. Skin lesions
2. Cardiac abnormalities (AV block, transposition of great vessels)
3. Valvular and septal defects.

Front 19

19. Clinical course of SLE?

Back 19

a. SLE is a chronic disease characterized by exacerbation and remissions.

Front 20

20. Most common initial findings with SLE?

Back 20

a. Malar rash
b. joint pain
c. fatigue

Front 21

21. 10 Tests used in the diagnosis of SLE?

Back 21

1. Positive ANA screening test
2. anti-DS DNA and anti-S and anti-SM Ab
3. anti-ssDNA
4. anti-Histone ABS
5. Ro (SS-A) and La (SS-B)
6. Positive LE preparation
7. False-positive test result for syphilis
8. Complement levels
9. CBC, renal function (BUN, creatinine), UA, Serum electrolytes
10. anticardiolipin and lupus anticoagulant

Front 22

22. what is the value of a positive ANA screening test with SLE?

Back 22

a. It is sensitive but not specific
b. almost all patients with SLE have elevated serum ANA levels

Front 23

23. What is the significance of anti-dsDNA and anti-SM Abs in SLE?

Back 23

a. Anti-ds DNA (40% of pts)
b. Anti-Sm Ab (30% of pts)
c. the presence of either of these is diagnostic of SLE – very specific (but obviously not sensitive).

Front 24

24. How often is anti-ssDNA found in SLE?

Back 24

a. 70%

Front 25

25. How frequently are anti-histone antibodies found in SLE?

Back 25

a. 70%
b. 100% of cases of drug-induced lupus
c. if negative, drug-induced lupus can be excluded

Front 26

26. With what are Ro (SS-A) and La (SS-B) associated in a pt w/Lupus?

Back 26

1. Sjogren's syndrome
2. subacute cutaneous SLE
3. neonatal lupus (with congenital heart block)
4. complement deficiency (C2 and C4)
5. ANA – negative lupus

Front 27

27. Positive LE preparation?

Back 27

a. ANAs bind to nuclei of damage cells, producing LE bodies.

Front 28

28. Are complement levels usually elevated or decreased in SLE?

Back 28

a. Decreased

Front 29

29. Treatment of SLE?

Back 29

a. Avoid sun exposure because it can exacerbate cutaneous rashes.
b. NSAIDs-for less severe symptoms.
c. Either local or systemic corticosteroids – for acute exacerbations.
d. Systemic steroids for severe manifestations