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Musculoskeletal > Connective Tissue Disease > Flashcards

Connective Tissue Disease Flashcards

1
Q

What is the ratio of females:males in Lupus

A

9:1

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2
Q

In what ethnic groups is the prevalence of lupus higher?

A

Asians
Afro-Americans
Afro-Caribbeans

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3
Q

What factors contribute to the aetiology of lupus?

A

Genetic factors:

  • monozygotic twins
  • increased incidence amongst relatives

Hormonal factors:

  • higher oestrogen exposure
    (e. g. on oestrogen contraceptives or HRT)
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4
Q

Describe the pathogenesis of Lupus

A

1) Increased and defective apoptosis
2) Necrotic cells release nuclear material which act as auto-antigens

3) Exposure to nuclear and intra-cellular auto-antigens
= B and T cells are stimulated

4) Auto-antibodies produced

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5
Q

What is responsible for the renal disease in lupus?

A

Deposition of immune complexes in mesangium

Cytokine release = inflammation which causes necrosis and scarring in kidneys

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6
Q

What systems are included in the SLE classification?

A 
Mucocutaneous
Musculoskeletal
(Serositis)
Renal
Neurological
Haematological
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7
Q

What constitutional symptoms are often present in lupus

A 
Fever
Malaise
Poor appetite
Weight loss
Fatigue
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8
Q

What symptoms can show mucocutaneous involvement in lupus?

A 
Photosensitivity
Malar rash 
Discoid lupus erythematosus
Subacute cutaneous lupus
Mouth ulcers
Alopecia
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9
Q

Give examples of the musculoskeletal features of Lupus

A
  • Non-deforming polyarthritis/polyarthralgia
  • Deforming arthropathy - Jaccoud’s arthritis
  • Myopathy - weakness, myalgia and myositis
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10
Q

What features of lupus are classified as serositis?

A

Pericarditis
Pleurisy
Pleural effusion
Pericardial effusion

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11
Q

What two things should be noted on urinanalysis to determine if there is renal involvement in lupus?

A

Protein

Blood (red cell casts seen on microscopy)

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12
Q

What level of protein in the urine would indicate lupus?

A

Proteinuria of >500mg in 24 hours

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13
Q

What neurological symptoms can be present in lupus

A 
Depression/psychosis
Migrainous headache
Seizures
Cranial or peripheral neuropathy
Mononeuritis multiplex
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14
Q

What haematogical disorders are associated with lupus?

A 
Lymphadenopathy (enlarged nodes)
Leucopenia (low leukocytes)
Lymphopenia (low lymphocytes)
Haemolytic anaemia
Thrombocytopenia (low platelets)
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15
Q

What are the features of anti-phospholipid syndrome

A
  • Venous and arterial thrombosis
  • Recurrent miscarriage
  • Thrombocytopenia
  • Prolonged APTT

Association with other autoimmune conditions especially SLE

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16
Q

What factors make antiphospholipid patients susceptible to infection?

A

Intrinsic factors:

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

Extrinsic factors

  • Steroids
  • Other immunosuppressive drugs
  • Nephrotic syndrome
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17
Q

Is anti-nuclear antibody specific to Lupus?

A

No

it can be positive in low titres (1:160) in up to 20% of the population

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18
Q

Other than ANA, what other auto-antibodies are associated with lupus?

A

Anti-dsDNA
Anti-Sm
Anti-Ro
Anti-RNP

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19
Q

What antibody is most specific to lupus?

A

Anti-dsDNA

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20
Q

The titre of Anti-dsDNA decreases when disease activity increases TRUE/FALSE

A

Titre correlates with overall disease activity

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21
Q

What features can Anti-Ro antibody cause?

A
  • Cutaneous manifestations
  • Secondary Sjogren’s features
  • Congenital heart block and neonatal Lupus
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22
Q

What auto-antibodies are associated with antiphospholipid syndrome

A
  • Anti-cardiolipin antibody
  • Lupus anticoagulant
  • Anti-beta 2 glycoprotein
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23
Q

How is a diagnosis of antiphospholipid made?

A

auto-antibodies positive on 2 occasions 12 weeks apart

24
Q

What other investigations would you consider carrying out if suspicious of SLE or antiphospholipid syndrome?

A 
CXR
Pulmonary function tests
CT chest
Urine protein quantification
Renal biopsy
Echocardiogram
Nerve conduction studies
MRI brain
25
Q

C3/4 levels decrease with increased disease activity in Lupus TRUE/FALSE

A

TRUE they negatively correlate with activity

26
Q

How is SLE activity monitored?

A 
clinical assessment
Anti-dsDNA level
C3/C4 levels 
Urine examination including protein, cells and casts
Full blood count
Blood biochemistry
27
Q

What are the non-medical treatments for SLE?

A

Counselling
Regular monitoring
Avoid excessive sun-exposure
Pregnancy issues

28
Q

What pharmacological methods are used to treat SLE?

A

NSAIDs and simple analgesia

Anti-malarials – hydroxychloroquine

29
Q

When are steroids used in SLE?

A

SMALL doses - skin rashes, arthritis, serositis

MODERATE doses - resistant serositis, haematologic abnormalities

HIGH doses - severe/resistant haematologic changes, renal disease and major organ involvement

30
Q

What immunosuppressives are commonly used in treatment of SLE?

A

Azathioprine
Cyclophosphamide
Methotrexate
Mycophenolate mofetil

31
Q

What biologic drugs have been licensed for use in SLE?

A

Anti-CD20 (Rituximab)
Anti-Blys (Belimumab)

suppress B cell antibody production

32
Q

Describe the differences in treatment from mild to severe SLE

A

Mild disease

  • HCQ
  • topical steroids
  • NSAIDs

Moderate disease

  • Oral steroids
  • Azathioprine
  • Methotrexate

Severe disease

  • IV steroids
  • Cyclophosphamide
  • Rituximab
  • Belimumab
33
Q

Give examples of rheumatological autoimmune diseases

A 
SLE
Sjogren`s syndrome
Systemic sclerosis
Mixed connective tissue disease
Inflammatory myositis(Polymyositis/Dermatomyositis)
Anti-phospholipid antibody syndrome
34
Q

What clinical symptoms would make you consider an autoimmune diagnosis in rheumatology?

A 
Arthralgia/arthritis
Muscle pain/weakness
Photosensitivity
Raynaud`s
Sicca symptoms (dry eyes/mouth/throat/vagina) 
Dysphagia
SOB
Neurological symptoms.
Recurrent miscarriage/pregnancy complications
35
Q

What auto-antibody is used as general screening for all auto-immune conditions?

A

Anti-nuclear antibody (ANA)

**a higher titre = more significant
<1:160 = not significant
1:640 = HIGH

36
Q

What is Raynaud’s?

A

Vasospasm reaction to cold weather
blood supply to digits is lost = ischaemia (WHITE)

capillaries and venules dilate => cyanosis (BLUE)

relaxation of vasospasm = Rubor (RED)

37
Q

What two groups usually get Raynaud’s?

A

Primary = teenagers, no underlying autoimmune disease, benign

Secondary = older age group, underlying autoimmune disease

38
Q

What symptoms do patients usually present with in secondary Raynaud’s?

A

ulcers

gangrene

39
Q

What treatments are used in Raynaud’s?

A

KEEP WARM!!

Vasodilators

  • Ca channel blockers
  • PDE5 inhibitors such as sildenafil

Digital ulcers

  • Prostacyclin analogues eg Iloprost.
  • Botox injections.
  • Endothelin receptor antagonists-e.g.Bosentan
40
Q

What is systemic sclerosis?

A

Used to be called scleroderma(Skin thickening)

2 TYPES:
Diffuse cutaneous systemic sclerosis (dsCC)
Limited cutaneous systemic sclerosis (lsCC)

41
Q

Are men or women more likely to develop systemic sclerosis?

A

Women

42
Q

What percentage of patients with systemic sclerosis present with Raynaud’s?

A

90%

43
Q

How are limited and diffuse systemic sclerosis differentiated?

A 
Limited = only face, legs and arms are affected
Diffuse = anywhere can be affected
44
Q

What are the symptoms of Limited systemic sclerosis?

A 
CREST
C- calcium deposits in skin
R- raynaud's
E- esophageal spasm
S- sclerodactyly
T- telangiectasia
45
Q

What organ involvement is commonly seen in Diffuse systemic sclerosis?

A 
Pulmonary fibrosis (LUNGS)
Pulmonary Hypertension (LUNGS and HEART)
Renal Failure
46
Q

What auto-antibodies are associated with limited and diffuse systemic sclerosis?

A

Limited = anti-centromere

Diffuse = antitopoisomerase

47
Q

How is systemic sclerosis managed?

A

Yearly ECHO and PFTs

Tx Raynaud`s and digital ulcers.
Tx reflux with PPi +/- ranitidine+/- Gaviscon .

Immunosuppression if:

  • Pulmonary fibrosis
  • Rapidly progressive skin involvement

Pulmonary HT -

  • Prostacyclin analogues
  • endothelin receptor antagonists
  • DE5 inhibitors.
48
Q

What features are characteristic of Sjogren’s syndrome?

A

lymphocytic infiltration of exocrine glands

Keratoconjunctivitis sicca

49
Q

What questions should you ask when taking a history suspicious of Sjogren’s?

A

“Have you had gritty eyes for over 12 weeks?”

“Have you woken up most nights to drink water in the last 12 weeks?”

“Have you found it difficult to swallow bread/crackers or have to drink water to help swallow in the last weeks?”

“Any vaginal dryness( in pre-menopausal women)?”

50
Q

What other symptoms are associated with Sjogren’s?

A 
Blepharitis.
Salivary gland inflammation.
Tooth decay.
Lymphoma.
Dry cough.
Multisystem involvement.
51
Q

What auto-antibodies are associated with Sjogrens?

A

Usually ANA positive.

Anti Ro and Anti La-more specific than sensitive.

52
Q

If auto-antibodies are negative, what investigations would you do?

A

salivary gland ultrasound and biopsy.

53
Q

What do blood tests in sjogrens patients usually show?

A

Usually high ESR/plasma viscosity.

Raised IgG.

Cytopaenias.

54
Q

How is Sjogrens treated?

A

Artificial tears and salivary supplements,vaginal lubricants.

strong fluoride toothpaste-e.g.Duraphat.

Hydroxychloroquine for fatigue and arthralgia.

Immunosuppression for major organ involvement.

55
Q

What percentage of Lupus patients have antiphospholipid syndrome?

A

20%

56
Q

What is used to treat antiphospholipid antibody syndrome?

A

Lifelong anticoagulation if thrombosis (usually warfarin)

Pre-pregnancy - LMWH (Fragmin) and aspirin continue right into the post partum period

Musculoskeletal (27 decks)

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