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Connective Tissue Diseases Flashcards

1
Q

What are connective tissue diseases?

A

They are characterized as a group by the presence of spontaneous over activity of the immune system.
They are often associated with specific auto-antibodies which can help define the diagnosis.

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2
Q

What is SLE?

A

Systemic AI disease that can affect any part of body. Can often mimic or is mistaken for other illnesses

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3
Q

What is the epidemiology of SLE?

A

F:M 9:1. Higher in asians, afro-americans/caribbeans and hispanic americans. Commoner in asian indians than caucasians in UK.

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4
Q

What genetic factors lead to SLE?

A

High concordance in monozygotic twins-increased incidence amongst relatives, identification of gene abnormalities predisposing to lupus

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5
Q

What hormonal factors lead to SLE?

A

Incidence increased with higher oestrogen exposure-early menarche, on oestrogen containing contraceptives and HRT

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6
Q

What environmental factors lead to SLE?

A

Viruses e.g. EBV. UV light may stimulate skin cells to secrete cytokines stimulating B-cells. Silica dusk may increase risk

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7
Q

What is the pathogenesis of SLE?

A

Due to loss of immune regulation. Increased/defective apoptosis. Necrotic cells release nuclear material which acts as potential autoantigen. Autoimmunitu possibly results by extended exposure to nuclear/intracellular autoantigens. B/T cells stimulated, autoantibodies produced

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8
Q

What are the constitutional symptoms of SLE?

A

Fever, malaise, poor appetite, weight loss, fatigue

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9
Q

What are the mucocutaneous features of SLE?

A

Photosensitivity, malar rash, discoid lupus erythematosus, subacute cutaneous lupus

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10
Q

What are the MSK features of SLE?

A

Non-deforming polyarthritis/arthralgia (RA distribution- no radiological erosion), deforming arthropathy-Jacoud’s, erosive arthritis, myopathy -weakness, myalgia, myositis

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11
Q

What are the pulmonary features of SLE?

A 
Pleurisy
Infections
Diffuse lung infiltration and fibrosis
Pulmonary hypertension
Pulmonary infarct
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12
Q

What are the cardiac features of SLE?

A

Pericarditis
Cardiomyopathy
Pulmonary hypertension
Libman-Sachs endocarditis

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13
Q

What is the presentation of glomerulonephritis in SLE?

A 
Proteinuria
Urine sediments
Urine RBC and casts
Hypertension
Acute renal failure
Chronic renal failure
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14
Q

What are the neurological features of SLE?

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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15
Q

What are the haematological features of SLE?

A

Lymphadenopathy (25% patients). Leucopenia, anaemia (haemolytc, normochromic, normocytic), thrombocytopenia

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16
Q

What are the intrinsic factors making SLE patients susceptible to infection?

A

Low complements
Impaired cell mediated immunity
Defective phagocytosis
Poor antibody response to certain antigens

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17
Q

What are the extrinsic factors making SLE patients susceptible to infection?

A

Steroids
Other immunosuppressive drugs
Nephrotic syndrome

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18
Q

What are the 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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19
Q

In what conditions will ANA be found?

A

RA, other AI conditions such as SLE, HIV, Hep C

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20
Q

In what percentage of SLE patients will ANA be +ve?

A

95% 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 positive
Anti-dsDNA
Anti-Sm
Anti-Ro
Anti-RNP

When the patient presents with CTD features

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22
Q

Describe anti-double stranded DNA antibody in SLE

A

Occurs in ~60% patients. Highly specific for SLE-titre correlates with disease activity. May be associated with lupus nephritis

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23
Q

Describe Anti-ENA’s associations

A 
Anti-Ro (60%)
Usually associated with anti-La
Associated with cutaneous manifestations
Secondary Sjogren’s features
Congenital heart block and neonatal LE
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24
Q

Are Anti-Sm autoantibodies highly or not highly specific?

A

Highly (10-20% cases). Probably association with neuro involvement

25
Q

What conditions are associated with anti-RNP?

A

Sclerodermatous skin lesions, Raynaud’s, low grade myositis, SLE (30%)

26
Q

What investigations other than bloods can be used in SLE?

A 
CXR
Pulmonary function tests
CT chest
Urine protein quantification
Renal biopsy
Echocardiogram
Nerve conduction studies
MRI brain
27
Q

What is done in SLE activity monitoring?

A

Clinical assessment incl. BP. Anti-dsDNA level +vely correlates with activity. C3/4 levels -vely correlate. Urine exam incl protein, cells and casts. FBC, blood biochemistry

28
Q

What drug treatment is used in SLE?

A

NSAIDs, analgesia, anti-malarials (hydroxychloroquine), steroids, immunosuppressives (azathoprine, cyclophosphamide, methotrexate, mycophenolate mofetil), biologics (rituximab, belimumab)

29
Q

What auto-antibody does Rituximab target?

A

Anti-CD20

30
Q

What auto-antibody does Rituximab target?

A

Anti-Blys

31
Q

What clinical features or test findings are needed to make a diagnosis of anti-phospholipid syndrome?

A

1 lab + 1 clinical feature needed-+ve anti-cardiolipin (phospholipid) antibodies and/or lupus anticoagulant activity and/or anti-beta2-glycoprotein on 2 occasions at least 12 wks apart. Arterial/venous thrombosis. Pregnancy loss (no other explanation) 10-34/40 or 3 losses (no explanation)

32
Q

What group of people does anti-phospholid syndrome occur in?

A

Young women M:F 1:3.5

33
Q

What are other non-diagnostic criteria features of anti-phospholipid syndrome?

A

Superficial thrombophlebitis and livedo reticularis
Mild/moderate thrombocytopenia
Neurological features – migraine, transverse myelitis
Libman-Sacks endocarditis
Catastrophic anti-phospholipid syndrome

34
Q

What is the treatment of anti-phospholipid syndrome?

A

Thrombosis – lifelong anti-coagulation
Pregnancy loss – aspirin + LMWH during pregnancy
Attention to vascular risk factors

35
Q

What is Sjogren’s syndrome?

A

AI condition- lymphocyte infiltration of exocrine glands causing xerostomia and keratoconjunctivitis sicca. Can be 1’/2’ to other AI conditions

36
Q

What is the classification criteria for primary Sjogren’s syndrome

A

4/6-immunology or biopsy evidence needed: ocular symptoms (daily >3 months), oral symptoms (daily >3 months), ocular dryness, salivary gland involvement, immunology-either Ro, La or both, biopsy evidence of lymphocytic infiltrate

37
Q

What tests are used in diagnosing Sjogren’s?

A

Schirmer test(ocular dryness), positive anti-Ro/La and typical features on lip gland biopsy

38
Q

What other conditions can manifest in Sjogren’s?

A 
Fatigue 
Arthralgia
Raynauds
Salivary swelling
Lymphadenopathy
Skin and vaginal dryness
Interstitial lung disease
Neuropathy
Lymphoma (x40 risk)
Renal tubular acidosis
Neonatal complete heart block (anti-Ro)
39
Q

What is the M:F ratio of Sjogren’s?

A

1:9

40
Q

What is the treatment of Sjogren’s?

A 
Eye drops, punctal plugs
Saliva replacement
Pilocarpine
Hydroxychloroquine
Steroids and immunosuppression 
Attention to cardiovascular risk factors
41
Q

What is systemic sclerosis?

A

AI condition. Presents with vasculopathy- Raynaud’s Syndrome, inflammation, fibrosis causing skin thickening

42
Q

What are the 2 classes of systemic sclerosis?

A

Limited, diffuse

43
Q

What are the features of limited systemic sclerosis?

A

Previously known as CREST- Calconosis, Raynauds, Esophageal dysmotility, Sclerodactyly, Telangiectasia. Pulmonary HT in 30%. Associated with anti-centromere antibodies

44
Q

What are the features of diffuse systemic sclerosis?

A

Skin changes within 1 year of Raynaud’s
Truncal and acral skin involvement
Early significant organ involvement (kidneys, lungs, gut, muscle, joints, heart)
Anti-Scl-70 antibodies

45
Q

What are the cutaneous features of systemic sclerosis?

A

Sclerodactyly, calcinosis, ulcerated fingertips, vasculitis

46
Q

What are the GI manifestations of systemic sclerosis?

A

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

47
Q

What are the respiratory manifestations of systemic sclerosis?

A

Interstitial lung disease
Pulmonary hypertension
Chest wall restriction

48
Q

What are the renal manifestations of systemic sclerosis?

A

Hypertensive renal crisis, ischaemic

49
Q

What are the cardiovascular manifestations of systemic sclerosis?

A

Raynaud’s with digital ulceration
Atherosclerotic disease
Hypertensive cardiomyopathy

50
Q

Who does systemic sclerosis commonly present in?

A

Age 25-55, M:F 1:4

51
Q

What is the treatment for systemic sclerosis?

A 
Calcium channel blockers
Prostacyclin (Iloprost)
ACE inhibitors
Prednisolone
Immunosuppression
Bosentan, Sildenafil
52
Q

What is mixed connective tissue disease(MCTD)?

A

Mixed connective tissue disease has signs and symptoms of a combination of disorders — primarily lupus, scleroderma and polymyositis. Has major and minor criteria and can present with various combinations of them

53
Q

What is undifferentiated connective tissue disorder?

A

‘Connective tissuish’ but usually don’t fit a particular syndrome
Usually a better prognosis
Would expect some antibody positivity

54
Q

How is systemic sclerosis diagnosed?

A

Auto-antibodies associated include anti-centromere/Scl-70. Organ screening-includes PFT, echo and monitoring of renal function

55
Q

What antibodies are associated with MCTD?

A

Anti-RNP

56
Q

How is MCTD monitored?

A

Regular echocardiograms (annually) suggested due to risk of Pulmonary HT, screening for ILD with PFT should take place

57
Q

How is MCTD managed?

A

Varies according to presentation, CCBs may help Raynauds. Immunosuppression if significant muscle or lung disease

58
Q

What is anti-phospholipid syndrome (APS)?

A

A disorder than manifests clinically as recurrent venous or arterial thrombosis and/or fetal loss. Can occur in association with SLE or other rheumatic/AI condition

Musculoskeletal (16 decks)

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