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RB Y2 MSK 02 - Rheumatology > Connective Tissue Diseases: SLE > Flashcards

Connective Tissue Diseases: SLE Flashcards

1
Q

what are connective tissue diseases characterised by

A

spontaneous over activity of immune system

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2
Q

SLE

A

chronic autoimmune disease, the presentation and course can be highly varied

immune system attacks body’s cells and tissues, leading to inflammation and tissue damage

antiboidy immune complexes precipitate and cause a further immune response

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3
Q

epidemiology

A

onset usually after puberty, 20s and 30s

females>males

prevalence high in black persons in UK and US, although rarely in blacks in Africa

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4
Q

what happens when males get the disease

A

get a more severe form

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5
Q

genetic factors

A

genetic predisposition:

concordance in monozygotic twins, inc incidence among relatives

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6
Q

hormonal factors

A

higher oestrogen - early menarche and oestrogen containing contraceptives

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7
Q

environmental factors

A
  • viruses eg EBV
  • UV light may stimulate skin cells to secrete cytokines stimulating B cells eg UVA/B therapy
  • silica dust may inc risk
  • toxins eg cigarette smoke
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8
Q

development of autoantibodies

A

defect in apoptosis causes increased cell death and a disturbance in immune tolerance

defective clearance of apoptotic cell debris causes extended exposure to antigen and immune complex formation (autoantibodies)

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9
Q

what type of hypersensitivity reaction

A

III

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10
Q

what are many of the clinical manifestations of SLE due to

A

deposition of immune complexes that form with antigens in various tissues

form in the small blood vessels causing an inflammatory reaction

are desposited on BM of skin and kidneys

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11
Q

renal disease

A

asymptomatic and very severe - RENAL BIOPSY if urinalysis suggests appropriate

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12
Q

what may mild disese involve

A

only joint pain,alopecia and skin rash

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13
Q

name some constitutional symptoms

A

fever, malaise, poor appetite, weight loss and fatigue

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14
Q

muco cutaenous symptoms

A

malar rash

photosensitivity

discoid lupus

subacute cutaenous lupus

alopecia

oral/nasal ulceration

Rayndaus phenomemon

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15
Q

discoid lupus

A
  • Benign variant of lupus in which only the skin is involved. The rash is characteristic and appears on the scalp, face and ears as well-defined erythematous plaques that progress to scarring and pigmentation
  • There is often hair loss
  • Most patients will just have skin involvement, up to 10% of people will eventually develop SLE
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16
Q

photosensitivity

A

prolonged exposure to sunlight can lead to exacerbations of the disease

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17
Q

what can non scarring alopecia lead to

A

irreversible bald patches

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18
Q

describe the rash that is seen

A

butterfly pattern

spares the naso-labial folds

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19
Q

what mucocutaneous feature can precede the development of other clinical signs by years

A

Raynaud’s phenomenon

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20
Q

MSK symptoms

A

polyarthralgia and poyarthritis

myopathy

inflammatory arthrits

arthropathy - rarely deforming or erosive

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21
Q

what deforming arthropathy can occur

A
  • Jaccoud’s arthritis is a chronic non-reversible joint disorder that may occur after repeated bouts of arthritis
  • Causes ulnar deviation through MCP subluxation
  • Can occur in lots of rheumatology things
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22
Q

what is there an increased prevalence of in the femoral head

A

avascular nerosis - may relate to steroids use

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23
Q

what is the most common respiratory manifestation

A
  • pleurisy (2/3 patients) with/out effusion - effusions are small and bilateral
  • also PE, pumonary hypertension and ILD
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24
Q

what are some other respiratory manifestations

A

Basal pneumonitis is often present, perhaps as a result of poor movement of the diaphragm, or restriction of chest movements because of pleural pain.

Also, PE, pulmonary hypertension and interstitial lung disease.

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25
Q

renal symptoms

A

lupus nephritis - severe and often asymptomatic

  • 1/3 patients will have evidence of renal damage
  • the most frequently observed abnormality is proteinuria, causes nephrotic syndrome
  • renal biopsy is required
  • early treatment needed
  • The most common and severe form is the diffuse proliferative GN (class IV), ‘wire-loop’ appearance
26
Q

management of proteinuria

A
  • Proteinuria >3mg treated with steroids
  • Step up to steroids and cyclophosphamide ± immunosuppression
27
Q

neurological symptoms

A
  • Occurs in up to 60% cases and symptoms often fluctuate. There may be a mild depression, but occasionally more severe psychiatric disturbances.
  • Depression/psychosis, migrainous headache, seizures, cranial or peripheral neuropathy, mononeuritis multiplex (individual peripheral nerves affected – foot or wrist drop).
28
Q

what happens if mononeuritis multiplex onyl affects one nerve

A

foot or wrist drop

29
Q

haematological symptoms

A

lymphadenopathy

leukopaenia

anaemia - haemolytic

thrombocytopaenia

30
Q

what haematological symptom occurs in a quarter of patients

A

lymphadenopathy

31
Q

cardiac symptoms

A

pericarditis, pericardial effusion, hypertension

32
Q

which drugs can cause SLE, and what are the associated antibodies

A

associated with isoniazid, anti-histone antibodies

33
Q

anti-phospholipid syndrome symptoms

A

thrombocytopaenia (low platelet count) causes venous AND arterial thrombosis

livido reticularis (skin)

late spontaenous, or reccurent early foetal loss

migraines common

34
Q

what is the classic presentation of someone with APS

A

child bearing age, with recurrent miscarriage and eg DVT

recurrent PE and thrombosis can also lead to pulmonary hypertension

35
Q

is APS primary or secondary

A

both

36
Q

why are patinets with APS more susceptible to infection

A

intrinsic: low complement, imparied cell mediated immunity, defective phagocytosis etc
extrinsic: steroids, IS drugs, nephrotic syndrome

37
Q

what are the anti-phospholipid antibodies

A
  • anti-cardiolipin
  • anti-beta 2 glycoprotein
  • lupus anticoagulant

must be positive on 2 occasions less than 12 weeks apart

38
Q

APS treatment

A

lifelong coagulation

hydroxychloroquine helps with systemic symptoms

39
Q

anticoagulation for APS

A
  • lifelong warfarin - however this is teratogenic
  • in pregnancy - aspirin/heparin
40
Q

diagnosis of APS

A
41
Q

what are the aims of investigation

A

confirm/establish diagnosis and determine organ involvement

wide range of investigations that depend on symptoms

42
Q

what investigations would always be done

A

immunology

FBC - may show thrombocytopaenia

urinalysis/renal biopsy

43
Q

immunology

A

ANA

anti-dsDNA antibody

anti-Sm

C3/4 levels

44
Q

ANA

A
  • sensitive but not remotely specific
  • good screening test
  • can be positive in all autoimmune diseases, RA, HIV etc
  • ≥1:160 in 90% of patients withbSLE
45
Q

what ANA level is seen in the general population

A

1:160

46
Q

anti-dsDNA antibody

A
  • the most specific and varies with disease activity
  • levels often relfect lupus nephritis activity
47
Q

anti-SM

A

the most specific but low sensitivity

48
Q

other immunology

A

Anti Ro, anti-La and anti-RNP – may be seen in SLE but also may be seen in other conditions.

49
Q

C3/4 levels

A

negatively correlate with disease activity

50
Q

what must be done once the diagnosis is established

A
  • urinalysis to look fo evidence of GN
  • screen for organ involvement - CT, MRI, ECHO
51
Q

management - non pharmacological

A

counselling, regular monitoring and advice on pregnancy issues is essential

avoid sun exposure (photosensitivity)

52
Q

what is given to all patients with SLE

A

hydroxychloroquine (anti-malarial)

may reduce systemic complications

53
Q

side effects of hydroxychloroquine

A
  • irreversible retinopathy - blindness
  • corneal deposits
54
Q

what drugs are commonly used

A

NSAIDs and analgesia

55
Q

what are steroids used for

A
  • Used in small doses for skin rashes, arthritis and serositis
  • Moderate does for haematological abnormalities and persistent serosits
  • High doses for severe haematological changes and major organ involvement
56
Q

when is IS used

A
  • used when there is inflammatory arthritis or some types or organ involvement
  • (e.g. azathioprine, cyclophosphamide, methotrexate, mycophenolate mofetil)
57
Q

what are the negative effects of IS drugs

A

possibly teratogenic

bone marrow suppression and inc suscpetibility to infection

58
Q

treatment of severe organ disease eg lupus nephritis

A

IV steroids and cyclophosphamide

59
Q

treatment of unresponsive cases

A

other therapies such as IV immunoglobulin and biologics may be necessary.

  • Rituximab (anti-CD20)
  • Belimumab (anti-byls)
60
Q

monitoring

A

anti dsDNA antibodies are checked regularly, as these vary wtih disease activity

check urinalysis for blood or protein

61
Q

subactue cutaneous lupus

A
62
Q

raynaud’s phenomenon

A

RB Y2 MSK 02 - Rheumatology (33 decks)

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