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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
Monitoring SLE activity
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
SLE general management
Counselling - patients, spouse and relatives Regular monitoring Avoid excessive sun-exposure Pregnancy issues
27
SLE drug treatment
NSAID and simple analgesia Anti-malarials – chloroquine and hydroxychloroquine Useful for arthritis, cutaneous manifestations and constitutional symptoms May reduce systemic complications
28
SLE - Biologics
Anti-CD20 (Rituximab) | Anti-Blys (Belimumab)
29
SLE - Immunosuppressives
``` Azathioprine Cyclophosphamide Methotrexate Mycophenolate mofetil (All can cause bone marrow suppression All can cause increased susceptibility to infection Potentially teratogenic) ```
30
What do SLE patients die of?
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
What is often the first sign of systemic sclerosis?
Raynauds
32
Treatment for sjogrens syndrome
- eye drops/ punctal plugs - Pilocarpine - artificial saliva - steroids & immunosuppression - hydrochloroquinine - ATTENTION TO CARDIOVASCULAR RISK FACTORS
33
Name some CVS risks associated with sjogrens
Heart attack Stroke Hypertryglyceridemia Hypertension (this is because of acute inflammatory state in sjogrens)
34
The older name for limited sceloderma
The older term for limited scleroderma is CREST syndrome (= Calcinosis, Raynaud's disease, (O)Esophageal dysmotility, Sclerodactyly, Telangiectasia). PULMONARY HYPERTENSION IN 30%
35
Which sjogrens antibody is associated with neonatal complete heart block?
Anti-Ro
36
Which antibodies are associated with limited scleroderma?
Anti-centromere
37
Which antibodies are associated with diffuse scleroderma?
Anti-Scl-70
38
How quickly do skin changes occur in diffuse systemic sclerosis?
Within one year of raynauds
39
Which organs are commonly involved in diffuse systemic sclerosis?
Kidneys, lungs, gut, muscle, joints, heart
40
What is juvenile sclerosis?
When patient under 16 presents with 1 major and at leas two of the 20 minor criteria of systemic sclerosis
41
Respiratory organ involvement in systemic sclerosis
ILD Pumonary hypertension CHEST WALL RESTRICTION
42
GI organ involvement in systemic sclerosis
Oesophageal hypomobility Small bowel hypomobility, bacterial overgrowth Pancreatic insufficiency Rectal hypomobility
43
Name a medication used to treat pulmonary arterial hypertension
Bosentan, sildenifil
44
Treatment of systemic sclerosis
``` Calcium channel blockers Prostacyclin (Iloprost) ACE inhibitors Prednisolone Immunosuppression Bosentan, Sildenafil ```
45
Trigeminal neuralgia
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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