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Connective tissue diseases Flashcards

1
Q

What is systemic lupus erythamatous?

A

Systemic autoimmune disease where the immune system attacks the bodies own cells and tissues resulting in inflammation.
The antibody-immune complexes precipitate and cause a further immune response.

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2
Q

Epidemiology of SLE

A

Occurs in females more than males.

Afro-caribbeans more than europeans etc.

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3
Q

What causes SLE?

A

Combination of genetic factors- increased incident among relatives
Hormonal factors-incidence increased with those with higher oestrogen
Environmental factors-virus’s, UV light, silica dust
Immunological factors

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4
Q

Pathogenesis of SLE

A

Primarily due to loss of immune regulation. Increased (and defective) apoptosis is caused. The necrotic cells then release nuclear material that can act as auto-antigens.
Autoimmunity probably results from extended exposure to these.
B and T cells are stimulated
Autoantibodies are produced.

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5
Q

Renal disease associated with SLE pathogenesis.

A

Immune complexes deposit in the mesangium (structure associated with capillaries in the glomerulus). Complexes consist of nuclear antigens and anti-nuclear antibodies. Complexes then form in the circulation then they are deposited. This activates complement which attracts leukocytes (WBC) and they release cytokines.
Cytokine release perpetuates inflammation and eventually can cause necrosis and scarring.

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6
Q

Mucocutaneous features of SLE (region of the body where mucus transitions to skin)

A

Photosensitivity
Malar rash (that may or may not be associated with sun exposure)- however spares the nasolabial folds.
Discoid lupus erythamatous (may scar)- disc like plaques on scalp
Subacute cutaneous lupus (skin plaques
Painless mouth ulcers
Alopecia (non scarring)

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7
Q

Musculoskeletal features of SLE

A

Non-deforming polyarthritis/polyarthralgia_ has a rheumatoid arthritis distribution but no radiological changes.
Deforming arthropathy (deforming disease of the joint)
Erosive arthritis- rare
Myopathy- weakness, myalgia, myositis

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8
Q

Serositis (inflammation of the serous membrane) features of SLE

A

Pericarditis
Pleurisy
Pleural and pericardial effusions

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9
Q

Renal features of SLE

A

Proteinurea of greater than 500mg in 24 hours.

Red cell cast

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10
Q

Neurological features of SLE

A 
Depression/ psychosis
Migrainous headache
Siezures
Cranial or peripheral neuropathy
Mononeuritis multiplex- form of damage to one or more peripheral nerves
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11
Q

Haematological features of SLE

A

Lymphadenopathy
Leucopenia- reduction in number of white cells
Lymphopenia- reduction in number of lymphocytes
Haemolytic anaemia- reduction in RBC
Thrombocytopenia

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12
Q

Antiphospholipid syndrome

A

Disorder of the immune system that causes blood clots.
Venous and arterial thrombosis
Livido reticularis- lace like purplish discolouration of the skin
Associated with other autoimmune conditions especially SLE
Thrombocytopenia- deficiency of platelets in the blood.

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13
Q

Susceptibility to infection in relation to SLE

A

Intrinsic factors- low complements

  • Impaired cell mediated immunity
  • defective phagocytosis
  • poor antibody response to certain antigens

Extrinsic factors-
steroids
Other immunosuppresive drugs

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14
Q

Investigations into SLE

A

Antinuclear antibodies
Anti-double stranded DNA
Anti- ENA
Antiphospholipid antibodies

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15
Q

Antinuclear antibodies (ANA)

A

95% positive in SLE patients

Also found in conditions such as RA, HIV and hep B

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16
Q

When should a positive ANA test be taken seriously?

A

When other antinuclear antibodies are also positive such as Anti-dsDNA, Anti-Sm, Anti-Ro, Anti-RNP

When the patient presents with CTD features

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17
Q

Anti-double stranded DNA

A

Occurs in approximately 60% of patients with SLE
Highly specific to SLA
Titre correlates with overall disease activity
May be associated with lupus nephritis

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18
Q

Anti- ENA

A 
Anti- extractable nuclear antigens- group of over 100 types of which 
-Anti-Ro makes up- 60%
-Usually associated with anti- La
Associated with cutaneous manifestations
Secondary sjogens features
Congenital heart block and neonatal LE

Anti-Sm (10-20%) is highly specific-probably association with neurological involvement
Anti-RNP (30%)- overlap features- raynauds phenomonom, sclerodermatous skin lesions, low grade myositis

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19
Q

Antiphospholipid antibodies

A

Anti-cardiolipin antibodies
Lupus anticoagulant
Anti- beta 2 cytokine
Must be positive on two occasions 12 weeks apart.

20
Q

Once a diagnosis of SLE is established, what should you do?

A

Screen for organ involvement

21
Q

Investigations of SLE for organ involvement

A 
CXR
Pulmonary function tests
CT chest
Urine protein quantification
Renal biopsy
Echocardiogram
Nerve conduction studies
MRI brain
22
Q

How do you monitor SLE activity?

A

Thorough clinical examination and BP
Anti-dsDNA correlates with disease intensity
C3/C4 levels negatively correlate
Urine examination including protein cells and casts
FBC and blood biochemistry

23
Q

General management of SLE

A

Counselling family, patient etc.
Regular monitoring
Avoiding excessive sunlight exposure
Pregnancy issues

24
Q

Drug treatment for SLE

A 
NSAIDS and simple analgesia
Steroids
Antimalarials
Immunosupressive therapy
Biologics (in severe disease)
25
Q

Anti-malarials in the treatment of SLE

A

Useful for arthritis, cutaneous manifestations and constitutional symptoms
May reduce systemic complications

26
Q

Steroids in the treatment of SLE

A 
Useful but may cause unwanted side-effects
Various doses are needed for different manifestations. 
Low dose e.g. prednisolone (<15mg/day) for skin rashes, arthritis and serositis
Medium dose e.g. (0.5mg per kg/d) for resistant serositis, haemotological abnormalities etc. 
High dose (1mg per kg/d) for severe haematological changes, major organ damage.
27
Q

Immunosupressive therapy for treatment of SLE

A

Azathioprine
Cyclophosphamide
Methotrexate
Mycophenolate mofetil

28
Q

Side effects of immunosupressive therapy for treatment of SLE

A

Can cause bone marrow suppression
Can cause increased susceptibility to infection
Potentially teratogenic (disturb the development of the embryo)

29
Q

Biologics in the treatment of SLE

A

Anti-CD20 (Rituximab)

Anti-Blys (Belimumab)

30
Q

Treatment of mild SLE

A

Topical steroids, NSAIDs and HQC

31
Q

Treatment of moderate SLE

A

Oral steroids
Azathioprine
Methotrexate

32
Q

Treatment of severe SLE

A

IV steroids
Cyclophosphamide
Rituximab

33
Q

Define a connective tissue disease

A

Characterised by the presence of spontaneous overactivity of the immune system.
Often associated with specific auto-antibodies which can help define the condition.

34
Q

How do connective tissue diseases related to systemic lupus erythamatous

A

They can all be features of SLE. E.g. antiphospholipid syndrome could manifest on its own or could be part of SLE.

35
Q

Antiphospholipid syndrome diagnosis

A 
Positive anticardiolipin antibodies
AND/OR 
Lupus anticoagulant activity
AND/OR
Anti- beta2 glycoprotein
36
Q

Affects of antiphospholipid syndrome on pregnant women?

A

Responsible for 15% of recurrent foetal loss
Or eclampsia
Or placental insufficiency

37
Q

Other features of antiphospholipid syndrome

A

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

38
Q

Treatment of antiphospholipid syndrome

A

For the thrombosis- life long anticoagulation
Pregnancy loss- aspirin and heparin during pregnancy to prevent eclampsia.
Attention to vascular risk factors.

39
Q

Sjogrens syndrome

A

Autoimmune condition
Lymphocyte infiltration of exocrine glands causing xerostomia (stress in the mouth caused by change in composition of saliva) and keratoconjunctivitis sicca (dryness of the conjunctiva)
May be primary or secondary to other autoimmune conditions

40
Q

Classification of primary sjogrens syndrome

A

Subjective ocular symptoms(daily for > 3 months)
Subjective oral symptoms (daily for > 3 months)
Objective evidence of ocular dryness
Objective evidence of salivary gland involvement
Immunology – either Ro, La or both
Biopsy evidence of lymphocytic infiltrate

Need 4 of 6 and must include either immunology or biopsy evidence

41
Q

Other manifestations of sjogrens syndrome

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)
42
Q

Primary sjogrens disease

A

Prevalence of 1-3%
Most people remain undiagnosed
Serious complications are very rare.

43
Q

Treatment of sjogrens disease

A 
Eye drops, punctal plugs
Saliva replacement
Pilocarpine
Hydroxychloroquine
Steroids and immunosuppression 
Attention to cardiovascular risk factors
44
Q

Systemic sclerosis

A

Autoimmune
Vasculopathy (Raynaud’s Syndrome)
Inflammation
Fibrosis – excess deposition of collagen in skin and internal organs

CREST-Calconosis, Raynaud’s, Esophageal dysmotilty, Sclerodactyly, Telangiectasia. Can also feature pulmonary hypertension in ~30%

45
Q

Organ manifestations of systemic sclerosis

A

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

Respiratory-
Interstitial lung disease
Pulmonary hypertension
Chest wall restriction

Renal
Hypertensive renal crisis
Ischaemic

Cardiovascular
Raynaud’s with digital ulceration
Atherosclerotic disease
Hypertensive cardiomyopathy

46
Q

Treatments of systemic sclerosis

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

Musculoskeletal (36 decks)

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