Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Year 2 Clinical > Connective Tissue Disease 1 > Flashcards

Connective Tissue Disease 1 Flashcards

1
Q

What is Lupus (SLE)?

A

The archetypal connective tissue disease but there are others

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are connective tissue diseases?

A

Largely autoimmune, multi-system diseases that are often assoc. with specific auto-antibodies, which can help define the diagnosis

They are NOT diseases of connective tissue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Occurrence facts about SLE?

A
  • Females affected more than males (9:1)
  • Prevalence is lower in Caucasians and higher in those of ethnic minority, e.g: Asians, Afro-Americans, Afro-Caribbeans and Hispanic Americans
  • Uncommon in African blacks
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Aetiology of SLE?

A 
Combination of factors:
• Genetic
• Hormonal 
• Environmental 
• Immunological
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Describe genetic factors in SLE

A

High concordance in monozygotic twins and increased incidence amongst relatives

Identification of gene abnormalities predisposing to SLE may be useful

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Describe hormonal factors in SLE

A

Increased incidence in those with higher oestrogen exposure, e.g: early menstruation, oestrogen-containing contraceptives and HRT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Describe environmental factors in SLE

A
  • Viruses, e.g: Epstein-Barr
  • UV light may stimulate skin cells to secrete cytokines, stimulating B-cells; a manner of presentation is after a holiday, with a persistent rash
  • CIGARETTE SMOKE
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Pathogenesis of SLE?

A

An antigen is presented to a CD4+ T cell by an APC gene

CD4+ T cell produces cytokines which:
• Stimulate antibody production by B cells
• Stimulate cytokine release by CD8+ T cells (destroy cells that have these antigens on their surfaces, i.e: loss of self-tolerance)

There is cell death; normally, clearance of cell contents occurs quickly
In SLE, CLEARANCE IS SLOWER and the nuclear and intracellular auto-antigens, which remain for a prolonged period, are attacked by antibodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is defective in SLE?

A

There is INCREASED and DEFECTIVE APOPTOSIS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Pathogenesis of renal disease in SLE?

A

Due to deposition of immune complexes (nuclear antigens : anti-nuclear antibodies) in the mesangium

These activate complement, which attract WBCs that release cytokines (perpetuate inflammation leading to necrosis and scarring)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Sign of ACTIVE lupus?

A

Decreased complement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

5 factors that drive SLE?

A
  • Immune complex formation DRIVES disease
  • Defective apoptosis
  • Delayed clearance of cell contents
  • B cell function is abnormal (antibodies produced against internal antigens)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Classification criteria for SLE?

A

PICTURE 6

Patient must have 1 from each list but 4 in total for lupus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Systemic symptoms of SLE?

A
  • Fever
  • Malaise
  • Fatigue (levels are CONSTANT and do not fluctuate with disease activity)
  • Weight loss
  • Poor appetite
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Mucocutaneous features of lupus?

A

MALAR RASH that may/may not be assoc. with sun exposure (photosensitivity); it SPARES the NASO-LABIAL FOLDS

Discoid lupus erythematosus is confined to the skin but often SCARS

Subacute cutaneous lupus

Mouth ulcers (painless)

Alopecia (non-scarring); this may simply be thinning of hair or discrete patches

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Compare the appearances of SLE and discoid lupus erythematous?

A

Discoid lupus has better demarcated lesions with a SCALY/crusted surface

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

MSK features of lupus?

A
  • Non-deforming polyarthritis / polyarthralgia, i.e: may have a similar distribution and appearance as RA but there are no radiological changes (normal X-ray)
  • Deforming arthropathy, e.g: Jaccoud’s arthritis has ulnar deviation of finger, at MCPs, but the X-ray is normal and the deformity is reversible (can make fists, etc)
  • Erosive arthritis (rare)
  • Myopathy causes weakness, myalgia and myositis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Which systems are affected by SLE?

A
  • Mucocutaneous
  • Musculoskeletal
  • (Serositis) - inflammation of a lining of something
  • Renal
  • Neurological
  • Haematological
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Types of serositis in SLE?

A

Pericarditis and pericardial effusions

Pleurisy and pleural effusions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Testing for renal disease in SLE?

A

U&Es are not useful as they tend to be normal

The best test for early detection is URINALYSIS (look for protein and blood); suspicious if proteinuria is >500mg in 24 hrs

Then, RENAL BIOPSY

21
Q

Neurological features of SLE?

A
  • Depression/psychosis (not always related to disease activity)
  • Migraines (very common)
  • Seizures
  • Cranial/peripheral neuropathy
  • Mononeuritis complex (damage to 2 or more separate peripheral nerves in disparate areas of the body)
22
Q

Haematological features of SLE?

A

Lymphadenopathy

Leucopenia and lymphopenia

Haemolytic anaemia

Thrombocytopenia

23
Q

What is anti-phospholipid syndrome?

A

Autoimmune, hypercoagulable state with arterial and venous thrombosis

It can be a stand-alone disease or it may occur in assoc. with other autoimmune conditions, esp. SLE

24
Q

Signs of anti-phospholipid syndrome?

A

Recurrent miscarriage

DVT/stroke at a young age

Livedo reticularis, which tends to affect thighs and abdomen; described as a recticular (lace-like) mottled discolouration of the skin; looks like marble

Prolonged APTT (clotting time) and thrombocytopaenia (low platelets)

25
Q

In SLE, what intrinsic factors increase susceptibility to infection?

A
  • Low complements
  • Impaired cell mediated immunity
  • Defective phagocytosis
  • Poor antibody response to certain antigens
26
Q

In SLE, what extrinsic factors increase susceptibility to infection?

A
  • Steroids
  • Extrinsic factors
  • Other immunosuppressive drugs
  • Nephrotic syndrome
27
Q

Describe the spectrum of disease in SLE and the symptoms and signs at different parts

A

Mild disease may inv. joint pain, mouth ulcers, alopecia, etc

Moderate disease may inv. pericarditis, inflammatory arthritis, recurrent pleural effusions, etc

Severe disease inv. organ-threatening disease, e.g: renal and neurological disease, very low platelets, etc

28
Q

What are ANA antibodies? Limitation?

A

Anti-nuclear antibodies; present in a +ve titre in most patients (95%) but can also be present in healthy patients (20%)

Also found in conditions such as RA, other autoimmune conditions, HIV and Hep C

29
Q

When should a +ve ANA antibody be taken seriously?

A 
If other anti-nuclear antibodies are +ve:
• Anti-dsDNA
• Anti-Sm
• Anti-Ro
• Anti-RNP

When the patient presents with CTD features

30
Q

What are anti-dsDNA antibodies? Advantages?

A

Anti-double stranded DNA antibodies that occur in only 60% of SLE patients but are HIGHLY SPECIFIC to SLE

The ONLY antibody where the titre correlates overall disease activity

It may also be assoc. with lupus nephritis

31
Q

What are the anti-ENAs?

A

Anti-Ro is present in 60% of SLE patients; it is usually assoc. with anti-La and cutaneous manifestations

There may be secondary Sjogren’s features

32
Q

Importance of anti-ENA antibodies in neonates?

A

If a mother is anti-Ro +ve, the child has an increased risk of developing neonatal lupus and congenital heart block

33
Q

What is anti-Sm?

A

Only present in 10-20% of patients but highly specific and may have an assoc. with neurological inv.

34
Q

What is anti-RNP?

A

Present in 30% of patients; assoc. with overlap features, such as sclerodermatous skin lesions, Raynaud’s phenomenon and low grade myositis

35
Q

Types of anti-phospholipid antibodies?

A

2 types:
Anti-cardiolipin antibody
Lupus anti-coagulant
Anti-β2 glycoprotein

36
Q

How to use anti-phospholipid antibodies for diagnosis?

A

Must be +ve on 2 occasions 12 weeks apart

37
Q

Ix for organ involvement?

A

CXR, pulmonary function tests, CT chest

Urine protein quantification, renal biopsy

ECG

Nerve conduction studies and MRI brain

38
Q

Methods of monitoring SLE?

A

Clinical assessment, inc. BP

Anti-dsDNA level correlates with lupus activity

C3/C4 levels fall when there is a disease flare

URINALYSIS at every visit

FBC

Blood biochemistry

39
Q

Relationship of disease activity with:
Symptoms
Anti-dsDNA antibodies
Complement (C3/C4) level

?

A

Symptoms of facial rash, pericarditis, seizures and nephritis, etc, are preceded by a rise in anti-dsDNA antibody and a fall in complement levels

40
Q

General management of SLE?

A

Counselling

Regular monitoring

Avoiding excessive sun-exposure

Pregnancy issues

41
Q

Drug treatment of SLE?

A
  1. NSAIDs and simple analgesia
  2. Anti-malarials (HYDROXYCHLOROQUINE) are useful for arthritis, cutaneous manifestations and may reduce systemic complications
  3. Steroids (short courses and infrequently)
  4. Immunosuppressives
  5. Biologics
42
Q

How are steroids used in SLE?

A

Small doses (prednisolone <15 mg/d) for skin rashes, arthritis and serositis

Moderate doses (0.5 mg/kg/d) for resistant serositis, haematologic abnormalities and class V GN

High doses (1mg/kg/d or IV) for severe/resistant haematologic changes, diffuse GN and major organ inv.

43
Q

Types of immunosuppressives used in SLE?

A

Azathioprine

Cyclophosphamide

Methotrexate

Mycophenolate motefil

44
Q

Side effects of immunosuppressives?

A

Can cause bone marrow suppression

Can cause increased susceptibility to infection

Potentially teratogenic

45
Q

Examples of biologics used in SLE?

A

Rituximab

Belimumab

46
Q

Treatment of mild disease?

A

HCQ, topical steroids and NSAIDs

47
Q

Treatment of moderate disease?

A

Oral steroids, azathioprine and methotrexate

48
Q

Treatment of severe disease?

A

IV steroids, cyclophosphamide, rituximab

Year 2 Clinical (119 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions