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Musculoskeletal > Connective Tissue Disease > Flashcards

Connective Tissue Disease Flashcards

1
Q

Constitutional Symptoms of SLE

A 
Fever
Malaise
Poor appetite
Weight loss
Fatigue
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2
Q

SLE Environmental Factors

A

Environmental factors:
Viruses eg Epstein-Barr Virus
UV light may stimulate skin cells to secrete cytokines stimulating B-cells
Silica dust (found in cleaning powders, cigarette smoke and cement) may increase risk of developing SLE

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3
Q

SLE Mucocutaneous Features

A 
Photosensitivity
Malar rash 
may or may not be associated with sun exposure
Discoid lupus erythematosus
Subacute cutaneous lupus
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4
Q

SLE Musculoskeletal Features

A

Non-deforming polyarthritis/polyarthralgia
RA distribution but no radiological erosion
Deforming arthropathy - Jaccoud’s arthritis
Erosive arthritis - rare
Myopathy - weakness, myalgia & myositis

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5
Q

Swan neck deformities

A

These result from recurrent synovitis and inflammation of the joint capsule, tendons and ligaments

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6
Q

SLE Pulmonary Features

A 
Pleurisy
Infections
Diffuse lung infiltration and fibrosis
Pulmonary hypertension
Pulmonary infarct
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7
Q

SLE cardiac features

A

Pericarditis
Cardiomyopathy
Pulmonary hypertension
Libman Sach endocarditis (non-bacterial endocarditis)

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8
Q

SLE Glomerulonephritis Presentation

A 
Proteinuria
Urine sediments
Urine RBC and casts
Hypertension
Acute renal failure
Chronic renal failure
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9
Q

What are casts?

A

Cylindrical structures produced by the kidneys, present in some disease states

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10
Q

SLE Neurological Features

A 
Depression/psychosis
Not always related to disease activity
Migranous headache
Cerebral ischaemia
TIAs or stroke
Cranial or peripheral neuropathy
Cerebellar ataxia
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11
Q

SLE Haematological Features

A

Lymphadenopathy
~25% of all patients during their course of illness
Leucopenia (low white cells)
Anaemia (haemolytic, normochromic normocytic)
Thrombocytopenia (low platelets)

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12
Q

Screening Tests for Suspected SLE

A 
Full blood count 
Renal function tests including urine examination
Anti-nuclear antibody
Anti-double stranded DNA antibodies
ENA
Complement levels
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13
Q

Conditions that ANA is found in

A

SLE, RA, hepatitis C, HIV, other autoimmune conditions

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14
Q

Anti-SM and what it is likely to be involved with?

A

SLE

Neurological association

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15
Q

Anti-La

A

Sjogrens

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16
Q

Anti-Ro

A

Sjogrens

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17
Q

Anti-Scl70

A

Scleroderma

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18
Q

Anti-RNP

A

Mixed Connective Tissue disease

Overlap features – sclerodermatous skin lesions, Raynaud’s phenomenon, low grade myositis

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19
Q

In SLE, what titre correlates with overall disease activity?

A

Anti-double stranded DNA antibody

May be associated with lupus nephritis

20
Q

What antibody may be associated with lupus nephritis in SLE?

A

Anti-double stranded DNA antibody

21
Q

In SLE, what is anti-Ro associated with?

A

Cutaneous manifestations

22
Q

What could happen with anti-Ro and a foetus?

A

Neonatal heart block, neonatal LE

23
Q

Other investigations for SLE

A 
Depend on symptoms
CXR
Pulmonary function tests
CT chest
Urine protein quantification
Renal biopsy
Echocardiogram
Nerve conduction studies
MRI brain
24
Q

What could you measure that negatively correlated with SLE activity?

A

Complement C3/C4

anti-double stranded DNA antibody correlates WITH activity

25
Q

Monitoring SLE activity

A

Thorough clinical assessment including BP
Anti-dsDNA level positively correlates with activity
C3/C4 levels negatively correlate with activity
Urine examination including protein, cells and casts
Full blood count
Blood biochemistry

26
Q

SLE general management

A

Counselling - patients, spouse and relatives
Regular monitoring
Avoid excessive sun-exposure
Pregnancy issues

27
Q

SLE drug treatment

A

NSAID and simple analgesia
Anti-malarials – chloroquine and hydroxychloroquine
Useful for arthritis, cutaneous manifestations and constitutional symptoms
May reduce systemic complications

28
Q

SLE - Biologics

A

Anti-CD20 (Rituximab)

Anti-Blys (Belimumab)

29
Q

SLE - Immunosuppressives

A 
Azathioprine
Cyclophosphamide
Methotrexate
Mycophenolate mofetil
(All can cause bone marrow suppression
All can cause increased susceptibility to infection
Potentially teratogenic)
30
Q

What do SLE patients die of?

A

Infection important cause of death in the early / medium stage of disease
Cardiovascular disease the major the cause of death in the late stages

31
Q

What is often the first sign of systemic sclerosis?

A

Raynauds

32
Q

Treatment for sjogrens syndrome

A
  • eye drops/ punctal plugs
  • Pilocarpine
  • artificial saliva
  • steroids & immunosuppression
  • hydrochloroquinine
  • ATTENTION TO CARDIOVASCULAR RISK FACTORS
33
Q

Name some CVS risks associated with sjogrens

A

Heart attack
Stroke
Hypertryglyceridemia
Hypertension

(this is because of acute inflammatory state in sjogrens)

34
Q

The older name for limited sceloderma

A

The older term for limited scleroderma is CREST syndrome (= Calcinosis, Raynaud’s disease, (O)Esophageal dysmotility, Sclerodactyly, Telangiectasia).
PULMONARY HYPERTENSION IN 30%

35
Q

Which sjogrens antibody is associated with neonatal complete heart block?

A

Anti-Ro

36
Q

Which antibodies are associated with limited scleroderma?

A

Anti-centromere

37
Q

Which antibodies are associated with diffuse scleroderma?

A

Anti-Scl-70

38
Q

How quickly do skin changes occur in diffuse systemic sclerosis?

A

Within one year of raynauds

39
Q

Which organs are commonly involved in diffuse systemic sclerosis?

A

Kidneys, lungs, gut, muscle, joints, heart

40
Q

What is juvenile sclerosis?

A

When patient under 16 presents with 1 major and at leas two of the 20 minor criteria of systemic sclerosis

41
Q

Respiratory organ involvement in systemic sclerosis

A

ILD
Pumonary hypertension
CHEST WALL RESTRICTION

42
Q

GI organ involvement in systemic sclerosis

A

Oesophageal hypomobility
Small bowel hypomobility, bacterial overgrowth
Pancreatic insufficiency
Rectal hypomobility

43
Q

Name a medication used to treat pulmonary arterial hypertension

A

Bosentan, sildenifil

44
Q

Treatment of systemic sclerosis

A 
Calcium channel blockers
Prostacyclin (Iloprost)
ACE inhibitors
Prednisolone
Immunosuppression
Bosentan, Sildenafil
45
Q

Trigeminal neuralgia

A

Extreme severe facial pain that tends to come and go in sudden shock like attacks
-like an electric shock

Musculoskeletal (44 decks)

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