Connective tissue disease (cortex)

Question 1

SLE can affect any organ system

Answer 1

T

Question 2

Pathogenesis of SLE?

Answer 2

T3 hypersensitivty: Immune complexes form in the small blood vessels, leading to complement activation and inflammation

Question 3

More than 90% of cases of SLE occur in women, mostly child bearing age

Answer 3

T

Question 4

21 y/old woman presents with fever, fatigue, weight loss, arthralgia, butterfly malar rash

Answer 4

SLE

Question 5

There is no one diagnostic test for SLE

Answer 5

T

Question 6

how diagnostic is anti-nuclear antibody for SLE?

Answer 6

positive in >95% of patients, not specific to SLE though

Question 7

how diagnostic is Anti-dsDNA antibody for SLE?

Answer 7

specific and varies with disease activity

Question 8

What investigations should you do in patients with suspected SLE?

Answer 8

FBC - check anaemia, leukopenia, thrombocytopenia
Urinalysis - check glomerulonephritis
Imaging for organ involvement (CT, MRI,echo)

Question 9

How are C3/C4 levles affected in active SLE?

Answer 9

Low levels - used up in complement cascade

Question 10

management of SLE depends on its manifestations

Answer 10

T

Question 11

In SLE, skin disease and athralgia is treated with?

Answer 11

hydroxychloroquine (a DMARD)
topic steroids
NSAIDs

Question 12

In SLE when hould immunosupressants such as azathioprine be used?

Answer 12

inflammatory arthritis or evidence of some types of organ involvement e.g. pericardial disease or interstitial lung disease

Question 13

In severe organ disease (SLE) which treatment is used?

Answer 13

IV steroids, chemotherapy

unresponsive cases other therapies such as IV immunoglobulin and rituximab

Question 14

In SLE It is common practice to check anti-dsDNA antibodies and complement levels regularly, as these vary with disease activity and may give some warning of a disease flare.

Answer 14

T

Question 15

What is Sjorgens syndrome?

Answer 15

autoimmune condition characterized by lymphocytic infiltrates in exocrine organs

Question 16

What type of symptoms is Sjorgens assoc with?

Answer 16

Sicca symptoms - dryness of the eyes and mouth

Question 17

Sjogrens syndrome can be a primary condition or can occur secondary to other autoimmune conditions

Answer 17

T

e.g. RA or SLE

Question 18

What is the diagnosis of Sjorgens based on?

Answer 18

sicca symptoms, positive anti-Ro and anti-La antibodies and biopsy

Question 19

Management of sjorgens?

Answer 19

Symptomatic - lubricants and eyedrops
Hydroxychloroquine (DMARD) for arthralgia
immunosuprresion if organ involvement

Question 20

What are characteristic symptoms of systemic sclerosis?

Answer 20

vasomotor disturbances (Raynauds); fibrosis and atrophy of the skin

Question 21

What is the pathogenesis of systemic sclerosis?

Answer 21

Excessive collagen deposition causing skin and internal organ involvement

Question 22

In systemic sclerosis, what are the 3 stages of cutaneous involvement?

Answer 22

(1) oedematous, (2) indurative, and (3) atrophic. Skin becomes thickened and tight

Question 23

What is sclerodactyly?

Answer 23

localized thickening and tightness of the skin of the fingers or toes - systemic sclerosis

Question 24

In the context of systemic sclerosis what is “beaking”?

Answer 24

pinching of the skin of nose

Question 25

In the context of systemic sclerosis what is calcinosis?

Answer 25

subcutaneous depositis of calcium in the digits

Question 26

Systemic sclerosis can be divided into?

Answer 26

Limited: skin involved tends to be confined to face, hands and forearms and feet. Organ involvement tends to occur later. Anti-centromere antibody association. Diffuse: skin changes develop more rapidly and can involve the trunk. Early significant organ involvement. Anti-Scl-70 antibody association.

Question 27

Anti-Scl-70 antibody association?

Answer 27

diffuse systemic sclerosis

Question 28

Anti-centromere antibody association?

Answer 28

Limited systemic sclerosis

Question 29

(Systemic Sclerosis) management for Renal involvemen?

Answer 29

ACE inhibitors

Question 30

(Systemic Sclerosis) management for GI involvement?

Answer 30

proton pump inhibitors for reflux

Question 31

What is mixed connective tissue disease?

Answer 31

defined condition which features symptoms also seen in other connective tissue diseases (organ involvement, athralgia, raynauds)

Question 32

Anti RNP antibodies assoc with mixed connective tissue disease

Answer 32

T

Question 33

What is antiphospholipid syndrome?

Answer 33

disease that provokes blood clots in veins and arteries, assoc with pregnancy related complications

Question 34

Some patients with antiphospholipid syndrome have no evidence of any definable associated disease

Answer 34

T

Question 35

What is livedo reticularis?

Answer 35

mottled reticulated vascular pattern that appears as a lace-like purplish discoloration of the skin assoc apls

Question 36

treatment anti phospholipid syndrome?

Answer 36

anti-coagulation | recurrent pregnancy loss low molecular weight heparin is used during pregnancy (warfarin is teratogenic).

Question 37

Patients who are found to have positive anti phospholipid antibodies but who have never had had an episode of thrombosis do not require anti-coagulation

Answer 37

T