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MSK Continued > Connective tissue disease (cortex) > Flashcards

Connective tissue disease (cortex) Flashcards

1
Q

SLE can affect any organ system

A

T

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2
Q

Pathogenesis of SLE?

A

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

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3
Q

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

A

T

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4
Q

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

A

SLE

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5
Q

There is no one diagnostic test for SLE

A

T

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6
Q

how diagnostic is anti-nuclear antibody for SLE?

A

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

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7
Q

how diagnostic is Anti-dsDNA antibody for SLE?

A

specific and varies with disease activity

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8
Q

What investigations should you do in patients with suspected SLE?

A

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

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9
Q

How are C3/C4 levles affected in active SLE?

A

Low levels - used up in complement cascade

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10
Q

management of SLE depends on its manifestations

A

T

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11
Q

In SLE, skin disease and athralgia is treated with?

A

hydroxychloroquine (a DMARD)
topic steroids
NSAIDs

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12
Q

In SLE when hould immunosupressants such as azathioprine be used?

A

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

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13
Q

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

A

IV steroids, chemotherapy

unresponsive cases other therapies such as IV immunoglobulin and rituximab

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14
Q

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.

A

T

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15
Q

What is Sjorgens syndrome?

A

autoimmune condition characterized by lymphocytic infiltrates in exocrine organs

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16
Q

What type of symptoms is Sjorgens assoc with?

A

Sicca symptoms - dryness of the eyes and mouth

17
Q

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

A

T

e.g. RA or SLE

18
Q

What is the diagnosis of Sjorgens based on?

A

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

19
Q

Management of sjorgens?

A

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

20
Q

What are characteristic symptoms of systemic sclerosis?

A

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

21
Q

What is the pathogenesis of systemic sclerosis?

A

Excessive collagen deposition causing skin and internal organ involvement

22
Q

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

A

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

23
Q

What is sclerodactyly?

A

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

24
Q

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

A

pinching of the skin of nose

25
Q

In the context of systemic sclerosis what is calcinosis?

A

subcutaneous depositis of calcium in the digits

26
Q

Systemic sclerosis can be divided into?

A

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.

27
Q

Anti-Scl-70 antibody association?

A

diffuse systemic sclerosis

28
Q

Anti-centromere antibody association?

A

Limited systemic sclerosis

29
Q

(Systemic Sclerosis) management for Renal involvemen?

A

ACE inhibitors

30
Q

(Systemic Sclerosis) management for GI involvement?

A

proton pump inhibitors for reflux

31
Q

What is mixed connective tissue disease?

A

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

32
Q

Anti RNP antibodies assoc with mixed connective tissue disease

A

T

33
Q

What is antiphospholipid syndrome?

A

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

34
Q

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

A

T

35
Q

What is livedo reticularis?

A

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

36
Q

treatment anti phospholipid syndrome?

A

anti-coagulation

recurrent pregnancy loss low molecular weight heparin is used during pregnancy (warfarin is teratogenic).

37
Q

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

A

T

MSK Continued (10 decks)

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