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BRS MUSCULOSKELETAL > 1b// Lupus and Related Autoimmune Connective Tissue Diseases > Flashcards

1b// Lupus and Related Autoimmune Connective Tissue Diseases Flashcards

1
Q

What are the autoimmune connective tissue disorders?

A

Systemic Lupus Erythematosus (SLE)

Sjogren’s syndrome

Systemic sclerosis (scleroderma)

Autoimmune Inflammatory muscle disease

*overlap syndromes can occur, especially in children

*NB term “connective tissue disorder” not entirely accurate – often involvement of other organs ‘Multisystem autoimmune rheumatic diseases’ might be better

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2
Q

Give an overview of RA.

A
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3
Q

What is ankylosing spondylitis?

A

Chronic spinal inflammation that can result in spinal fusion and deformity

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4
Q

Where is the site of inflammation for ankylosing spondylitis? And what are the antibodies involved?

A

Site of inflammation includes the enthesis

no autoantibodies

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5
Q

What are the 4 seronegative inflammatory arthritis?

A

Ankylosing spondylitis

Reactive Arthritis

Arthritis associated with psoriasis (psoriatic arthritis)

Arthritis associated with gastrointestinal inflammation
(enteropathic arthritis)

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6
Q

What is SLE?

A

Systemic lupus erythematosus (SLE)

Autoimmune disease involving disturbance of both innate and adaptive immune systems

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7
Q

What is the v basic pathogenesis of SLE?

A

Autoantibodies (antibodies directed against self antigens)
-antibodies to nuclear components

Antibody-antigen (immune complexes) & other mechanisms -> chronic tissue inflammation:
Multi-site inflammation but particularly the joints, skin and kidney

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8
Q

What are the 3 general points of autoimmune connective tissue disorders?

A

1) Arthralgia and arthritis is typically non-erosive (unlike rheumatoid)

2) Serum autoantibodies are characteristic and…
- Useful diagnostically
- Some correlate with disease activity
- May be directly pathogenic

3) Raynaud’s phenomenon is common in these conditions

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9
Q

What is Raynaud’s phenomenon?

A

Intermittent vasospasm of digits

Usually triggered by cold exposure

Typical triphasic colour changes

WHITE -> Vasospasm leads to blanching of digit
BLUE -> Cyanosis as static venous blood deoxygenates
RED -> Reactive hyperaemia

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10
Q

What does severe Raynaud’s phenomenon lead to?

A

Severe Raynaud’s -> tissue ischaemia, ulcers and necrosis

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11
Q

What is the epidemiology of SLE?

A
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12
Q

What are the skin and mucosal clinical features of SLE?

A

Malar rash – erythema that spares the nasolabial fold Photosensitive rash
Mouth ulcers
Hair loss

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13
Q

What are the vascular clinical features of SLE?

A

Raynaud’s

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14
Q

What are the MSK clinical features of SLE?

A

Arthralgia and sometimes arthritis (usually non-erosive)

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15
Q

What are the internal organs clinical features of SLE?

A

Serositis (pericarditis, pleuritis, less commonly peritonitis) Renal disease – glomerulonephritis (‘lupus nephritis’) Cerebral disease – ‘cerebral lupus’ e.g. psychosis Myocarditis

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16
Q

What are the haematological clinical features of SLE?

A

autoimmune thrombocytopenia (low platelets) haemolytic anaemia
Lymphopenia

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17
Q

What are “other” clinical features of SLE? (2)

A

Lymphadenopathy

Fever in absence of infection

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18
Q

What antibody is a hallmark of SLE?

A

ANA anti-nuclear antibodies

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19
Q

In who is ANA found?

A

Found in all SLE patients

Negative ANA effectively rules out SLE

However, ANA is not specific for lupus;
- may be seen in other autoimmune diseases, infection or even healthy people

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20
Q

What will the lab do for ANA?

A

Lab will do further more specific autoAb tests to identify what ANA are reacting to…

  • Anti-ds-DNA antibodies
  • Anti-Ro
  • Anti-La
  • Anti-centromere
  • Anti-Smith (Sm)
  • Anti-RNP
  • Anti-Scl-70
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21
Q

Apart from ANA, what antibody can be found in SLE patients?

A

As well as ANA, some SLE patients have antibodies directed to phospholipids on cell membrane

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22
Q

What are APLs associated with risk wise?

A

APL are associated with ↑ risk of:

1) Thrombosis
arterial (e.g. stroke)
venous (e.g. deep vein thrombosis, DVT)

2) Pregnancy loss (miscarriage)

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23
Q

What does persistent presence of APL lead to?

A

Persistent presence of APL + a clinical event = “anti-phospholipid antibody syndrome”

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24
Q

When can anti-phospholipid antibody syndrome occur asw?

A

Anti-phospholipid antibody syndrome can also occur in absence of SLE (‘primary anti-phospholipid antibody syndrome’ )

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25
Q

What are the main autoantibodies for SLE?

A
26
Q

What are the autoantibodies rheumatology?

A
27
Q

What is the immunopathogenesis of SLE?

A
28
Q

How is the immune system generating a response to nuclear antigens as it should be hidden inside the cell? (the waste disposal hypothesis)

A
29
Q

Why are manifestations of lupus so varied?

A

it is systemic autoimmunity, so any organ can be affected

unlike hashimoto’s where it’s only the thyroid

30
Q

From investigations of SLE, what can be seen for inflammation?

A

high ESR but usually normal C-reactive protein unless infection or serositis/arthritis

31
Q

From investigations of SLE, what can be seen for haematology?

A

Haemolytic anaemia, Lymphopenia, Thrombocytopenia

32
Q

From investigations of SLE, what can be seen/ done for renal?

A

very important to measure urine protein (urinalysis + quantify with urine protein:creatinine ratio [uPCR])

Creatinine (part of “U&E”, measure of renal function)

look at albumin

Kidney biopsy if persistent proteinuria

33
Q

From investigations of SLE, what can be seen/ done for immunological?

A

Antinuclear antibodies

Anti-double-stranded DNA antibodies - highly specific, correlate with disease activity

Complement consumption – e.g. low C4 and C3

antiphospholipid antibodies

In treated patients SOME changes may reflect ADVERSE REACTIONS TO MEDICATION
* e.g. abnormal liver function tests (‘transaminitis’) or fall in neutrophil count (neutropenia)

34
Q

How do you measure disease activity?

A

Combination of:
-Clinical symptoms and signs
-Investigations

35
Q

What does an unwell patient with active lupus typically have (immunology wise)?

A

Low complement C3 and C4 levels

High anti-dsDNA antibodies

36
Q

What are the general principles of management of SLE?

A
  1. Treatment in SLE aims at remission or low disease activity and prevention of flares
  2. Balance of controlling disease vs avoiding iatrogenic harm (especially steroids)
  3. Specific choice of treatment will depend on disease severity and organ manifestations
37
Q

What are the possible side effects of steroids?

A

Infection
Osteoporosis
Avascular necrosis (AVN) - often affects hips: higher incidence in lupus, especially in presence of APL Abs

Appropriate initiation of immunomodulatory agents can expedite the tapering/ discontinuation of glucocorticoids

38
Q

What is recommended for all patients with lupus treatment wise?

A

Hydroxychloroquine is recommended in all patients with lupus

39
Q

What is given to treat acute flares in SLE?

A

Steroids for acute flare but aim to withdraw as soon as safe to do so

40
Q

What is given as treatment for mild disease SLE?

A

Mild disease: Hydroxychloroquine alone may be sufficient

41
Q

What is given for a more serious disease of SLE?

A

If more serious disease, immunomodulatory agents (mycophenolate, methotrexate, azathioprine)

42
Q

What is given for renal disease in SLE?

A

Mycophenolate +/- rituximab usually used for renal disease

43
Q

What is given in persistently active disease of SLE?

A

In persistently active disease: B cell targeted therapies

Rituximab = anti-CD20 monoclonal antibody: depletes B cells
Belimumab = anti-BAFF antibody (BAFF = a B cell survival factor/cytokine)

44
Q

What is given for severe or life-threatening disease of SLE?

A

Severe or life-threatening disease (e.g. myocarditis) : iv steroids + iv cyclophosphamide (+/- rituximab)

45
Q

What is given to patients with SLE and anti-phospholipid syndrome?

A

Patients with SLE and antiphospholipid antibody syndrome (i.e. episode of clot + antiphospholipid antibody positive) should be treated with anticoagulation (warfarin)

46
Q

What is an emerging therapy for SLE?

A

anifrolumab (interferon receptor blockade)

47
Q

What should be considered with pregnancy and lupus?

A
48
Q

What are the types of autoimmune inflammatory muscle disease?

A

Polymyositis Dermatomyositis

49
Q

What are the types of Systemic sclerosis (scleroderma)?

A

Diffuse cutaneous Limited cutaneous

50
Q

What is SJÖGREN’S SYNDROME?

A

Autoimmune exocrinopathy

lymphocytic infiltration of exocrine glands (lacrimal and salivary glands)

51
Q

What does the exocrine gland pathology result in in SJÖGREN’S SYNDROME?

A

Dry eyes (xerophthalmia)

Dry mouth (xerostomia)

Parotid gland enlargement

52
Q

What are extra-glandular manifestations of SJÖGREN’S SYNDROME?

A

non-erosive arthritis
Raynaud’s phenomenon pleural effusion autoimmune hepatitis

53
Q

What are autoantibodies involved with SJÖGREN’S SYNDROME?

A

Ro+, La+. RF often + but CCP -.

54
Q

What is secondary Sjögren’s syndrome?

A

Termed ‘secondary’ Sjögren’s syndrome if occurs in context of another connective tissue disorder e.g. SLE

55
Q

What is inflammatory muscle disease?

A

Autoimmune-mediated inflammation of muscle

Causes proximal muscle weakness (& sometimes pain)

56
Q

What is dermatomyositis?

A

muscle and skin inflammation

57
Q

What is polymyositis?

A

(muscle only, no rash)

58
Q

What are investigations for dermatomyositis?

A

Investigations:

1) Bloods: Elevated CK (creatine kinase)

2) Electromyography (electrical activity of the muscles)

3) Muscle biopsy (polymyositis = CD8 T cell infiltrate; dermatomyositis = CD4 T cells in addition to B cells)

NB associated with malignancy (10-15%) and pulmonary fibrosis

59
Q

What is systemic sclerosis?

A

Thickened skin with Raynaud’s phenomenon

dermal fibrosis

cutaneous calcinosis

telangiectasia

60
Q

Describe the skin changes in systemic sclerosis.

A

Skin changes may be limited or diffuse

61
Q

Describe limited systemic sclerosis.

A

Fibrotic skin limited to hands, forearms, feet, neck and face

Anti-centromere antibodies

Pulmonary hypertension

Long history of Raynaud’s phenomenon

*CREST describes a sub-type of limited systemic sclerosis.
It stands for Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia

62
Q

Describe diffuse systemic sclerosis.

A

Fibrotic skin proximal to elbows or knees (excluding face and neck)

Anti-Scl-70 antibodies

Pulmonary fibrosis, renal (thrombotic microangiopathy) involvement

Short history of Raynaud’s phenomenon

BRS MUSCULOSKELETAL (12 decks)

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