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MSK SM > Connective Tissue Diseases > Flashcards

Connective Tissue Diseases Flashcards

1
Q

What are connective tissue diseases

A

They are characterised as a group by the presence of spontaneous over activity of the immune system

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2
Q

What are connective tissue diseases often associated with

A

Specific auto-antibodies which can help define the diagnosis

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3
Q

What is systemic lupus erythematosis

A

Systemic autoimmune disease that can affect any part of the body

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4
Q

What is the difficulty for clinicians and SLE

A

It is often misdiagnosed as it mimics or is mistake n for other illnesses

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5
Q

What populations are more likely to develop Lupus

A

Women of Afro-Caribbean Afro-Americans and Hispanic Americans or Asian population
Uncommon in African Blacks

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6
Q

What are some of the factors leading to lupus

A 
Oestrogen exposure (hormonal)
Genetic factors
Environmental factors - silica dust 
Immunological Factors 
Infection - particularly viral infections (ebstein barr)
UV light
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7
Q

Describe the pathogenesis in a normal cell

A

You get a foreign antigen (often a virus) and a WBC will pick it up and show it to the T cells. They then release cytokines which provoke an inflammatory reaction which then stimulates the B cells to start producing antibodies against the foreign body

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8
Q

Describe the pathogenesis in autoimmunity

A

In autoimmune, these antigens are not foreign – they are part of the body and they are cell proteins – all cells break down and die over time. Whats in the cell will continue to float around for a short time – antigen presenting cells pick these up and see this as an external threat – B cells begin to produce antibodies against the normal cellular protein (autoantibodies)

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9
Q

Name some of the constitutional symptoms of SLE

A 
Fever
Malaise
Poor appetite
Weight loss
Fatigue
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10
Q

What are some of the mucocutaneous features of SLE

A

photosensitivity
Malar rash
Discoid lupus erythematosis
Subacute cutaneous lipus

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11
Q

What are some of the musculoskeletal features

A

Non-deforming polyarthritis/ polyarthralgia
Deforming arhtropathy
Erosive arthritis (rare)
Myopathy - weakness, myalgia and myositis

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12
Q

What are some of the pulmonary features of SLE

A 
Pleurisy 
Infections 
Diffuse lung infiltration and fibrosis 
Pulmonary hypertension
Pulmonary infarct
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13
Q

What are some of the cardiac features of SLE

A

Pericarditis
Cariomyopathy
Pulmonary hypertension
libman -Sachs endocarditis (no bacteria often)

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14
Q

What are the findings in Glomerulonephritis SLE

A 
Proteinuria 
Urine sediments
Urine RBC and casts 
Hypertension
Acute renal failure 
Chronic renal failure
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15
Q

What are some of the neurological features of SLE

A 
Depression/ psychosis 
Migranous headache 
Cerebral ischaemia 
Cranial or peripheral neuropathy 
Cerebellar ataxia
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16
Q

What are some of the haematological features of SLE

A

Lymphadenopathy
Leucopenia
Anaemia
Thrombocytopenia

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17
Q

What are the aims of investigations for suspected SLE

A

To confirm the diagnosis

To determine the degree of organ involvement

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18
Q

How do we screen for suspected SLE

A 
FBC 
Renal function tests (Urine examination)
Anti-nuclear antibody
Anti-Double stranded DNA antibodies 
ENA 
Complement levels
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19
Q

In what conditions is Anti-nuclear antibody found (ANA)

A

Rheumatoid arthritis
HIV
Hep C

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20
Q

What would be the finding in the majority of SLE positive patients in terms of ANA

A

Positive in titre of 1:160 or greater

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21
Q

When should a positive ANA test be taken seriously

A 
If other antinuclear antibodies are also positive 
ANti-dsDNA
anti-Sm
Anti-Rs
Anti-RNP

When the patient presents with CTD features

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22
Q

Anti-Double stranded DNA antibody is not highly specific for SLE. True or False.

A

False - it is highly specific and occurs in 60% of patients with SLE

23
Q

Anti-SM if highly specific to SLE. What is there a probably association with when this is present

A

Neurological involvement

24
Q

What is the general management involved with SLE

A

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

25
Q

What drugs should be used in SLE

A

NSAIDs and simple analgesia
Anti-malarials - hydroxychloroquine
Steroid - caution due to side effects
Immunosuppressives

26
Q

What is the treatment for Mild disease lupus

A

HCO
Topical steroids
NSAIDs

27
Q

What is the treatment for Moderate lupus

A

Oral steroids
Azathioprine
Methotrexate

28
Q

What is the treatment for Severe disease

A

IV steroids
Cyclophosphamide
Rituximab - immunosuppression

29
Q

What do patients with SLE die of

A

Complications fo SLE in the first few years - Males, late onset, lower socioeconomic class
Infection - early/ medium stage
Cardiovascular disease in the late stages

30
Q

How can you make a diagnosis of Anti-phospholipid syndrome

A

Positive anti-cardiolipin antibodies and or lipus anticoagulant activity and/ or anti-beta2-glycoprotein on 2 occasions at least 12 weeks apart

Pregnancy loss with no other explanation

31
Q

Anti-phospholipid syndrome is responsible for what 2 things

A

Recurrent foetal loss

recurrent thrombosis in young people

32
Q

Anti-phospholipid is more common in young women. True or false

A

True

33
Q

What are some of the other features of anti-phospholipid syndrome

A

Superficial thrombophlebitis and lived reticular
Neurological features - migraine, transverse myelitis
Libman-Sacks endocarditis

34
Q

Describe the appearance of Lived reticular

A

Marbling appearance

35
Q

What is the treatment for anti-phsophold syndrome

A

Thrombosis- lifelong anti-coagulation
Pregnancy loss - aspirin and heparin during pregnancy
attention to vascular risk factors

36
Q

What is Sjogren’s syndrome

A

Lymphocyte infiltration of exocrine glands causing xerostomia and keratoconjunctivitis sicca

37
Q

What test is carried out for suspected Sjogren’s syndrome

A

Schirmer test

38
Q

What are needed to diagnose Primary Sjogren’s syndrome

A

4 of 6 of the classification criteria
Ocular or oral symptoms dryness
Immunology 0 either Ro,La or both
Biopsy evidence of lymphocytic infiltrate

39
Q

What are some of the other manifestations of Sjogren’s syndrome

A 
Fatigue 
Arthralgia 
Raynauds 
Salivary swelling 
Lymphadenopathy 
Skin and vaginal dryness
Interstitial lung disease 
neuropathy 
lymphoma 
neonatal complete heart block (anti-Ro)
40
Q

What is the peak age for Primary Sjogren’s syndrome

A

40-60

41
Q

What is the treatment for Sjogren’s

A 
Eye drops, punctal plugs 
Saliva replacement 
Pilocarpine 
Hydroxychloroquine
Steroids and immunosuppression
Attention to cardiovascular risk factors
42
Q

What results in a patient with systemic sclerosis

A

Fibrosis - excess deposition of collagen in skin and internal organs

43
Q

What are some of the main features of systemic sclerosis

A 
CREST:
Calconosis
Raynaud's 
Esophageal dysmotility 
Sclerodactyly
Telangiectasia 
Pulmonary Hypertension in 30%
44
Q

What is systemic sclerosis associated with

A

Anti-centromere antibodies

45
Q

What are some of the GI manifestations of systemic sclerosis

A 
Oesophageal hypomobility 
small bowel hypo mobility 
bacterial overgrowth 
pancreatic insufficiency 
rectal hypomobility
46
Q

What are some of the respiratory manifestations of systemic sclerosis

A

Interstitial lung disease
pulmonary hypertension
Chest wall restriction

47
Q

What are some of the renal manifestations of systemic sclerosis

A

Hypertensive renal crisis

Ischaemic

48
Q

What are some of the cardiovascular manifestations of systemic sclerosis

A

Raynaud’s with digital ulceration
Atherosclerotic disease
hypertensive cardiomyopathy

49
Q

What is the common age to develop systemic sclerosis

A

25-55

50
Q

What are the treatment options for systemic sclerosis

A 
Calcium channel blockers
Prostacyclin 
ACE inhibitors 
Prednisolone
Immunosuppression
Bosentan, sildenafil
51
Q

What is mixed connective tissue disease (MCTD) defined by

A

Various combinations of major and minor criteria

52
Q

What are some of the major criteria for MCTD

A 
severe myositis 
pulmonary involvement 
Raynaud's phenomenon 
Swollen hands observed 
Sclerodactyly
53
Q

What are some of the minor criteria for MCTD

A 
Alopecia 
Leukpenia 
Anaemia
Pleuritis
Pericarditis
Arthritis 
Trigeminal neuralgia 
Malar rash 
THrombocytopenia 
Mild myositis 
History of swollen hands
54
Q

Describe the prognosis of Undifferentiated Connective tissue disorder

A

Usually better than most connective tissue disorders

MSK SM (42 decks)

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