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MSK lectures > Connective Tissue Diseases 09/10/18 > Flashcards

Connective Tissue Diseases 09/10/18 Flashcards

1
Q

Name some connective tissue diseases

A 
SLE
Sjogren's syndrome
Systemic sclerosis
Dermatomyositis
Polymositis
Mixed connective tissue disease
Anti-phospholipid syndrome
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2
Q

What are connective tissue diseases?

A

NOT diseases of the connective tissue
Spontaneous over activity of the immune system
Specific auto-antibodies
Evolve over years

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3
Q

What is Systemic Lupus Erythematosus (SLE)?

A

Systemic autoimmune disease

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4
Q

Where does SLE affect?

A

Any part of the body

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5
Q

What is the pathogenesis of SLE?

A

The immune system attacks the bodys cells and tissue resulting in inflammation and tissue damage
Antibody-immune complexes precipitate and cause a further immune response
Loss of immune regulation
Increased and defective apoptosis
Necrotic cells act as auto-antigens
Autoimmunity

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6
Q

Who gets SLE?

A

Females more than males

Asians, Afro-americans and afro-caribbeans

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7
Q

What is the pathogenesis of renal disease from SLE?

A

Deposition of immune complexes in mesangium
Complexes of nuclear antigens and anti-nuclear antibodies
Complexes form in circulation then are deposited
Activate complement which attracts leucocytes which release cytokines
Cytokine release perpetuates inflammation which causes necrosis and scarring

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8
Q

What are the clinical criteria for SLE?

A 
Acute cutaneous lupus
Chronic cutaneous lupus
Oral or nasal ulcers
Non-scarring alopecia
Arthritis
Serositis
Renal
Neurological
Haemolytic anaemia
Leukopenia
Thrombocytopaenia
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9
Q

What are the immunologicall criteria for SLE?

A 
ANA
Anti-DNA
Anti-SM
Antiphospholipid Ab
Low complement
Coomb's test
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10
Q

What systems feature in the SLE classification criteria/

A 
Mucocutaneous
Musculoskeletal
Serositis
Renal Neurological
Haematological
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11
Q

What are continuous symptoms of SLE?

A 
Fever
Malaise
Poor appetite
Weight loss
Fatigue
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12
Q

What are some mucocutaneous features of SLE?

A 
Photosensitivity
Malar rash
Discoid Lupus
Subacture cutaneous lupus
Mouth ulcers
Alopecia
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13
Q

What are some musculoskeletal features of SLE?

A 
Non-deforming polyarthritis/polyarthralgia
Deforming arthropathy
Myopathy
Weakness
Myalgia
Myositis
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14
Q

What is serositis?

A

Inflammation of the serous membrane

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15
Q

What does serositis cause?

A

Pericarditis
Pleurisy
Pleural effusion
Pericardial effusion

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16
Q

What are renal features of SLE?

A

Proteinuria of >500mg in 24 hours

Red cell casts

17
Q

What are the neurological factors of SLE?

A 
Depression/Psychosis
Migranous headaches
Seizures
Cranial or peripheral neuropathy
Mononeuritis multiplex
18
Q

What are haematological features of SLE?

A 
Lymphadenopathy
Leucopenia
Lymphopenia
Haemolytic anaemia
Thrombocytopenia
19
Q

What is anti-phospholipid syndrome?

A 
Venous and arterial thrombosis
Recurrent misscarriage
Livido reticularis
Association with SLE
Thrombocytopaenia
Prolonged APTT
20
Q

What intrinsic factors increase susceptibility to infection?

A

Low complement
Impaired cell mediated immunity
Defective phagocytosis
Poor antibody response to antigens

21
Q

What extrinsic factors increase suscptibility to infection?

A

Steroids
Immunosuppression
Nephrotic syndrome

22
Q

How sensitive is ANA in lupus?

A

98% of lupus patients

20% of normal

23
Q

When should +ve ANA be taken seriously

A 
\+Ve:
Anti-DsDNA
Anti-SM
Anti-Ro
Anti-RNP
24
Q

How sensitive is anti dsDNA in lupus?

A

60%

Highly specific

25
Q

Anti-ENA?

A 
Anti-Ro (60%)
Usually associated with anti-La
Cutaneous manifestations
Secondary Sjogren’s features
Congenital heart block and neonatal LE
26
Q

What must be done once diagnosis is established?

A

Screen for organ involvement

27
Q

What are some investigations for SLE?

A 
CXR
Pulmonary function tests
CT chest
Urine protein quantifications
Renal biopsy
Echocardiogram
Nerve conduction studies
MRI brain
28
Q

How is SLE activity monitered?

A

Clinical assessment
Anti-dsDNA level positively correlates with activity
Urine examination - protein, cells and casts
FBC
Blood biochemistry

29
Q

What is the general management for lupus?

A

Counselling
Monitoring
Avoid excess sun
Pregnancy issues?

30
Q

What is the drug treatment for lupus?

A

NSAIDs and analgesia

Anti-malarials - hydroxychloroquinine

31
Q

Are steroids used for Lupus?

A

Useful but associated with numerous side effects

Variable doses

32
Q

Name 4 immunosuppressants

A

Azathiprine
Cyclophosphamide
Methotrexate
Mycopheolate mofetil

33
Q

What are 3 side effects to immunosuppresion?

A

Bone marrow suppression
Increased susceptibility to infection
Teratogenic

34
Q

Name 2 biologics

A

Anti-CD20 - Rituximab

Anti-Blys (Belimumab)

35
Q

What is the treatment of mild lupus?

A

HCQ
Topical steroids
NSAIDs

36
Q

What is the treatment for moderate lupus?

A

Oral steroids
Azathioprine
Methotrexate

37
Q

What is the treatment for sever lupus?

A

IV steroids
Cyclophophamide
Rituximab
Belimumab

MSK lectures (50 decks)

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