Brainscape's Knowledge GenomeTM

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


See full index

Week 11- Musculoskeletal > Connective tissue disorder > Flashcards

Connective tissue disorder Flashcards

1
Q

When is the most common onset of SLE?

A

During reproductive years

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

Which races is SLE more common in?

A
  • Afro-caribbean
  • South Asian
  • Mexican
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Which sex is SLE more common in?

A

Females

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

What is the name of the classification of SLE?

A

•EULAR/ACR

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

What is the clinical presentation of lupus?

A
  • Constitutional symptoms: weight loss, headache, pain, fatigue, loss of appetite, night sweats, and malaise
  • Cutaneous manifestations
  • Arthralgia (joint stiffness)
  • Arthritis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the musculoskeletal signs of lupus?

A
  • Synovitis
  • Jaccoud’s arthropathy
  • AVN, fibromyalgia and osteoporosis are all associated
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Explain renal component of SLE

A
  • 1/3-1/2 of patient’s will have renal involvement
  • 20% will develop ESRF in 10 years
  • Typically within first year or two
  • Urinalysis, UEs, BP monitored at the clinic
  • dsDNA- rise in titre can predict flares
  • Renal biopsy may be helpful for the diagnosis, prognosis and determining treatment
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Explain the pulmonary manifestations of SLE

A
  • Pleurisy - pleuritic chest pain
  • Pleural effusions
  • Acute pneumonitis
  • Diffuse alveolar haemorrhage
  • Pulmonary hypertension
  • Shrinking lung syndrome
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Explain the cardiovascular manifestations of SLE

A
  • Pericarditis +/- effusion
  • Myocarditis
  • Valvular abnormalities
  • Coronary heart disease - high risk of morbidity and mortality long term
  • Risk of MI 50x
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Explain the neuropsychiatric manifestations of SLE

A
  • 19 different manifestations
  • Headache
  • Anxiety and mood disorder
  • Seizure
  • demyelination
  • GBS
  • Mononeuritis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Explain the investigation of the manifestations of SLE

A
  • EEG if seizures
  • MRI
  • LP to look for inflammatory cells
  • Psychiatric evaluation
  • Anti-ribosomal P - associated with mood disorders
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the gastrointestinal manifestations of SLE?

A
  • Uncommon, more likely to be effect due to medication
  • Dysphagia
  • Reduced peristalsis
  • Peritonitis
  • Pancreatitis
  • Pseudo-obstruction
  • Lupus hepatitis- biopsy is required
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are the haematological manifestations of SLE?

A
  • Anaemia of chronic disease
  • Autoimmune haemolytic anaemia
  • Thrombotic thrombocytopenia purpura
  • Leukopenia
  • Can have an associated lymphadenopathy and splenomegaly
  • Thrombocytopenia - mild or ITP
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is ANA and what is its significance in lupus?

A
  • Anti-nuclear antibodies
  • SLE is due to activation of invasive and adaptive immunity
  • Interaction of self antigens on or released by apoptotic cells
  • In SLE there is a breakdown in the ability to process debris from apoptotic cells, nuclear elements of cells are exposed and you can develop self antigens
  • it a sensitive but not a specific test
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

If ANA is positive, what is it helpful to know?

A

Which of the antigens is affected

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

Ro/La

A

SLE, sjogrens

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

Ds-DNA

A

SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Sm

A

SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

RNP

A

Mixed CTD

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

Centromere

A

Limited SScl

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Scl-70

A

diffuse SScl

22
Q

Histone

A

Drug induced lupus

23
Q

Explain complement in SLE

A
  • Complement consumption in active disease
  • C3 more specific
  • C4 can be chronically low
24
Q

What is the treatment of SLE?

A
  • Steroids
  • Hydroxychloroquine - 1st line for all severities
  • Belimumab - B cell therapy
25
Q

What are the adjuvant therapies for SLE?

A
  • Topical lubricants for sicca symptoms
  • Fatigue management groups
  • Calcium channel blockers for raynauds
  • Treatment of co-existent fibromyalgia
  • CVS risk
  • Osteoporosis
  • If coexisting APLS use anticoagulants in confirmed thromboembolic disease
26
Q

Who does scleroderma mainly affect?

A

•Women aged 30-50

27
Q

What is scleroderma characterised by?

A
  • Skin thickening

* Progressive fibrosis and vascular diseae

28
Q

What are the disease mimics of scleroderma?

A
  • Diabetic cheiropathy
  • Generalised morphed
  • Eosinophilic fasciitis
29
Q

What is sufficient for a patient to be diagnosed with scleroderma?

A

Skin thickening of the fingers extending proximal to the metacarpophalangeal joint

30
Q

What is the abbreviation for scleroderma?

A

SScl

31
Q

If skin thickening if the fingers extending proximal to the metacarpophalageal joint is not present, what is needed for a diagnosis of scleroderma

A
  • Score of >9 is in keeping with SScl
  • Skin changes
  • Fingertip lesions
  • Telangiectasia
  • Abnormal nail fold capillaries
  • Pulmonary arterial hypertension and/or ILD
  • Raynaud’s phenomenon
  • SScl-related autoantibodies
32
Q

What are the sub categories of scleroderma?

A

•Localised scleroderma

  • morphed
  • linear scleroderma

•Systemic scleroderma

  • limited
  • diffuse
33
Q

Describe limited variant scleroderma

A
  • Distal skin involvement
  • Skin calcification
  • Telangectasia
  • Raynauds
  • Anti-centromere positive
  • Late incidence of PAH
34
Q

What is diffuse variant scleroderma

A 
•Proximal skin involvement and trunk 
•Raynauds
•Early organ involvement 
 - ILD, PAH
 - Renal failure 
 - myocardial disease 
 - GI involvement
35
Q

What are the cutaneous manifestations of scleroderma?

A
  • skin thickening
  • Initially appears as non-pitting oedema of hands and feet
  • Progresses to skin tightness (puffiness recedes)
  • sclerodactyl involvement of the fingers
  • Contractures
  • Calcinosis
  • Telangectasia
36
Q

What are the musculoskeletal manifestations of scleroderma?

A
  • Arthralgia
  • Myalgia
  • Inflammatory arthritis and inflammatory myositis is less common
  • Tendon friction rubs
37
Q

What are the vascular manifestations of scleroderma?

A
  • Raynauds

* severe disease with digital ulcers and auto amputation (gangrene)

38
Q

What are the gastrointestinal manifestations of scleroderma?

A

•Oesophageal dysmotility
•GORD
•Small bowel hypo mobility with bacterial overgrowth
•Hypomobility of large bowel with constipation
*pancreatic insufficiency

39
Q

What are the respiratory manifestations of scleroderma?

A
  • Interstitial lung disease
  • Organising pneumonia
  • Pulmonary hypertension
40
Q

What are the renal manifestations of scelroderma?

A
  • Mortality rates have improved ocnsideranly with ACEi

* Crisis usually occurs within 5 years of disease onset , hypertension and progressive renal failure

41
Q

What are the cardiac manifestations of scleroderma?

A
  • Arrhythmia
  • Peicardial effusion
  • Myocardial fibrosis
42
Q

What is the treatment for the cutaneous manifestations of scleroderma?

A
  • Moistureiser
  • Methotrexate
  • Laster therapy for telangiectasia
  • Rarely for excision
43
Q

What is the treatment for musculoskeletal manifestations of scleroderma

A
  • Analgesia

* methotrexate

44
Q

What is the treatment for raynauds?

A
  • CCB first line
  • ACEi/Fluoxetine
  • Sildenafil
  • Iloprost
45
Q

What are the subtypes of idiopathic inflmamatory myositis?

A
  • Polymyositis
  • Dermatomyositis
  • Overlap syndromes
  • Juvenile PM/DM
  • Drug induced
  • Inclusion body myositis
46
Q

What are the clinical manifestations of idiopathic inflammatory myopathies?

A
  • Symmetrical, proximal muscle weakness
  • Myalgia in <50%
  • Respiratory/diaphragm involvement
  • Oesophageal involvement
  • Rarely there is face and neck involvement
  • Distal disease is unusual
47
Q

Explain the cutaneous manifestation of idiopathic inflammatory myopathies

A

•Doesn’t occur in Polymyositis
•Dermatimyositis can precede muscle involvement
- grottrons papule (red/purple papule over MCP/PIPJ)
- heliotrope rash often with per orbital oedema
- macular eruption - shawl sign, V sign
- calcinosis, more common in children

48
Q

Explain the systemic manifestations of idiopathic inflammatory myopathies

A

•Overlap syndromes:

  • Connective Tissue
  • antisynthetase syndromes

•Malignancy
- association with dermatomyositis>polymyositis

•Non specific symptoms:

  • weight loss
  • fatigue
49
Q

What are the investigations for idiopathic inflammatory myopathy?

A
  • Creatinine approx thousands (significantly higher suggests alternative causes)
  • EMG (electromyography)
  • MRI of muscles
  • Muscle biopsy
  • Myositis specific antibodies
  • Tumour screening if symptoms/red flags
50
Q

What is the treatment of idiopathic inflammatory myopathies?

A 
•Corticosteroids 
•Immunosuppression 
•IV immunoglobulins
•Resistant disease: 
 - rituximab 
 - cyclophosphamide

Week 11- Musculoskeletal (11 decks)

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