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MSK > Connective tissue disease > Flashcards

Connective tissue disease Flashcards

1
Q

How can SLE present - signs and symptoms?

A 
sharp chest pain worse on inspiration - pleuritic chest pain 
pain in joints eg hands and knees - synovitis 
headaches 
ankle swelling 
fatigue and fever - constitutional 
poor appetite 
butterfly rash on face
skin rash
mucosal ulceration 
pleurisy 
pericarditis 
Raynaud's phenomenon 
venous thrombosis
arterial thrombosis 
vasculitis 
depression and psychosis 
recurrent abortion
lymphadenopathy  
high BP
dullness in the base of the lung with reduced breath sounds - pleural effusion 
protein and blood in the urine - glomerulonephritis
arthritis 
oral ulcers 
phostosensitivity
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2
Q

what are the differential diagnoses of SLE?

A 
basically the possible causes of multi-system disease:
infection
auto-immune diseases
malignancy 
endocrine diseases
metabolic diseases
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3
Q

What may be seen on investigations of sb with SLE?

A 
low WCC
anaemia 
raised ESR and raised CRP
raised creatinine - renal impairment 
pleural effusion on CXR
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4
Q

What test is done in SLE pts presenting with pleuritic chest pain to exclude PE?

A

CT angiogram

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5
Q

what is the treatment for SLE?

A

prednisolone

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6
Q

What are the two causes of connective tissue diseases?

A

autoimmune

inherited

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7
Q

Give examples of inherited connective tissue disorders

A

marfans

Ehler Danlos syndrome

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8
Q

what are the features of Marfan’s

A 
arachnodactyly
high arch palate
lens dislocation 
joint pain due to hyperpermeability 
aortic aneurysm 
pectus excavatum 
larger arm span than height
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9
Q

What are the features of Ehler Danlos syndrome?

A

hypermobile joints

skin hyperelasticity

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10
Q

Give examples of autoimmune connective tissue diseases

A 
SLE
systemic sclerosis 
primary Sjörgen's syndrome 
dermatomyositis/ polymyositis 
undifferentiated connective tissue diseases - which don't fit into a specific category
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11
Q

Are autoimmune connective tissue disorders inflammatory?

A

yes - inflammation leads to scarring and damage

can lead to organ failure

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12
Q

The damage is reversible in autoimmune connective tissue disorders? T or F

A

false - it is irreversible

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13
Q

Is the butterfly rash with SLE permanent?

A

no it comes and goes

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14
Q

Does SLE have male or female predilection?

A

female

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15
Q

Does SLE have any genetic associations?

A

yes with HLA

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16
Q

Explain the pathophysiology behind SLE

A

autoantibodies formed by a defective immune system bind to nuclear antigens (ie substances in the nucleus of our own cells) to form immune complexes
the immune complexes enter the bloodstream and go to to other organs causing tissue damage
Ag-Ab complexes activate complement and cause inflammation and cell death
phospholipid antibodies are also produced that cause thrombosis

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17
Q

How does the SLE arthritis compare with RA?

A

both are symmetrical
both can be deforming
less proliferative than RA
non-erosive unlike RA

18
Q

what are the clinical features of lupus nephritis?

A

hypertension
proteinuria
renal failure
nephritic syndrome

19
Q

what are the haematological features of lupus?

A 
anaemia 
thrombocytopenia 
Lymphocytopenia
neutropenia 
pancytopenia
20
Q

Name some autoantibodies that may be detected in a pt with Lupus

A

ANA
dsDNA antibody
rheumatoid factor
antiphospholipid antibodies: anticardiolipin Ab, lupus anticogaulant Ab

21
Q

What happens in antiphospholipid syndrome?

A

the antiphospholipid antibodies will cause a hypercoagulable state and there is arterial and venous thrombosis, with recurrent abortion and thrombocytopenia

22
Q

What are the 11 criteria the help diagnose Lupus and how many of these criteria need to be met for a diagnosis?

A 
at least 4 of:
Malar rash
Discoid rash
Photosensitivity
Oral ulcers
Arthritis
Serositis
Renal disorder
Neurological disorder
Haematological disorder
Immunological disorder
Anti-nuclear antibody
23
Q

How is SLE managed not in terms of drugs?

A 
pt education and support 
UV protection
Assessment of lupus activity: Clinical and Immunological
Screening for major organ involvement
Assessment of damage
24
Q

What is the drug management of SLE?

A 
No Treatment
Topical: Sunscreens, Steroids, Cytotoxic
NSAIDs
Antimalarial
Steroids
Cytotoxic drugs
Anticoagulants
Biological 
Plasmapheresis
Stem cell transplant
25
Q

Give examples of cytotoxic drugs that may be used for Lupus

A 
Azathioprine
Mycophenolate
Ciclosporin
Methotrexate
Cyclophosphamide
26
Q

What is the name of the biological agent use in Lupus and what does it target?

A

Rituximab

targets CD20 on the B cell surface

27
Q

From what conditions does secondary raynaud’s come from?

A 
Systemic sclerosis 
SLE 
RA
dermatomyositis and polymyositis 
mixed connective tissue disease (a mix of both the above)
28
Q

What factors can cause secondary Raynaud’s

A

connective tissue diseases
drugs eg beta blockers
vascular damage - atherosclerosis, frostbite and vibrating tools

29
Q

what is the triphasic colour change in raynaud’s

A

white to blue to pink

30
Q

What are the features of limited scleroderma?

A 
CREST + pulmonary hypertension 
Calcinosis 
Raynaud's 
Oesophageal reflux/strictures 
sclerodactyly
telangectasia 
pilmonary hypertension
31
Q

what antibody is present in CREST syndrome?

A

anti-centromere Abs

32
Q

What are the features of diffuse scleroderma?

A 
Proximal scleroderma
Pulmonary fibrosis
Bowel involvement
Myositis
Renal crisis: hypertension
33
Q

what are the points of pathogenesis of scleroderma?

A

Vasculopathy
Excessive collagen deposition
Inflammation
Auto-antibody production

34
Q

How is systemic sclerosis managed?

A 
Raynaud's:
Physical protection
Vasodilators eg Nifedipine
Sympathectomy
Fluoxetine

reflux
PPIs for life

prevention of renal crisis:
ACEIs

early detection of PAH through annual echos and pulmonary function tests

pulmonary fibrosis:
treated with cyclophosphamide

35
Q

what conditions can lead to secondary Sjörgen’s?

A 
SLE
RA
scleroderma 
primary biliary cholangitis 
other autoimmune diseases
36
Q

Name 2 autoantibodies in Sjörgens

A

ANA

rheumatoid factor

37
Q

what are the systemic features of Sjörgen’s

A 
inflammatory arthritis 
sub-acute lupus rash
neuropathies 
vasculitis 
neonatal lupus 
increased risk of lymphoma
38
Q

How is primary Sjorgen’s managed?

A

tear and salivary replacement
immunosuppression for systemic complications
needs a joint approach between specialities - rheumatology, oral medicine, opthalmology, neurology, dermatology

39
Q

What are the signs and symptoms of dermatomyositis and polymyositis?

A

muscle and skin affected
rash (only in dermatomyositis)
muscle weakness
interstitial lung disease - SOB

40
Q

What investigations can be done for dermatomyosytis and polymyositis

A

muscle enzymes eg creatine kinase raised
electromyography - to look for abnormal muscle signal conduction
skin/muscle biopsy
screen for malignancy
CXR, PFTs, HRCT scans

41
Q

what is the management of polymyositis and dermatomyositis

A

steroids

immunosuppressive drugs

MSK (12 decks)

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