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Aneesha Flashcards BRS 2 > Connective tissue diseases > Flashcards

Connective tissue diseases Flashcards

1
Q

Give examples for seronegative arthritis

A

Ankylosing spondylitis
Reactive Arthritis (Reiters syndrome)
Arthritis associated with psoriasis (psoriatic arthritis)
Arthritis associated with gastrointestinal inflammation (enteropathic synovitis)

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2
Q

What is SLE? What tissues does it affect and what autoantibodies is it associated with?

A

Chronic tissue inflammation in the presence of antibodies directed against self antigens
Multi-site inflammation but particularly the joints, skin and kidney
Associated with autoantibodies:
Antinuclear antibodies
Anti-double stranded DNA antibodies
Anti-phospholipid antibodies

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3
Q

What type of arthritis/ arthralgia can be seen in connective tissue disorders?

A

Non- erosive

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4
Q

Significance of serum auto-antibodies in connective tissue disorders?

A

May aid diagnosis
Correlate with disease activity
May be directly pathogenic

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5
Q

Name a common phenomenon seen in connective tissue disorders. What happens in this?
Does this phenomenon always indicate a connective tissue disorder

A

Raynaud’s phenomenon- Intermittent vasospasm of digits on exposure to cold
Typical colour changes – white to blue to red
Vasospasm leads to blanching of digit
Cyanosis as static venous blood deoxygenates
Reactive hyperaemia

Does NOT always indicated connective tissue disorder- most commonly an isolated and benign condition (‘Primary Raynaud’s phenomenon’)

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6
Q

Give examples for connective tissue disorders

A

Systemic Lupus Erythematosus (SLE)
Sjögren’s syndrome
Autoimmune inflammatory muscle disease: Polymyositis, dermatomyositis
Systemic sclerosis (scleroderma): Diffuse cutaneous, limited cutaneous
Overlap syndromes

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7
Q

What demographic is SLE commonly diagnosed in?

A

Females aged between 15-45

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8
Q

Clinical manifestations of SLE

A

Malar rash – erythema that spares the nasolabial fold
Photosensitive rash
Mouth ulcers
Hair loss
Raynaud’s phenomenon
Arthralgia and sometimes arthritis
Serositis (pericarditis, pleuritis, less commonly peritonitis)
Renal disease – glomerulonephritis (‘lupus nephritis’)- see IgG, immune complexes, inflammatory cells (monoctyes, macrophages), complement components
Cerebral disease – ‘cerebral lupus’ e.g. psychosis

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9
Q

SLE pathgenesis

A

Incompletely understood, current paradigm is as follows

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10
Q

Key auto-antibodies in osteoarthritis

A

None

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11
Q

Key auto-antibodies in reactive arthritis

A

None

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12
Q

Key auto-antibodies in gout

A

None

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13
Q

Key auto-antibodies in ankylosing spondylitis

A

None

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14
Q

Key auto-antibodies in systemic vasculitis

A

Anti nuclear cytoplasmic antibodies (ANCA)

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15
Q

Key auto-antibodies in rheumatoid arthritis

A

Rheumatoid Factor
Anti-cyclic citrullinated peptide antibody
also termed antibodies to citrullinated peptide antigens (ACPA)

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16
Q

Key auto-antibodies in SLE

A

Antinuclear antibodies (ANA)
Anti-double stranded DNA antibodies (anti-dsDNA)
Anti-phospholipid antibodies a.k.a anti-cardolipin antibodies

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17
Q

Give examples for different types of anti-nuclear autoantibodies (ANA)

A

Anti-Ro
Anti-La
Anti-centromere
Anti-Sm
Anti-RNP
Anti-ds-DNA antibodies
Anti-Scl-70

Cytoplasmic antibodies include:

Anti-tRNA synthetase antibodies
Anti-ribosomal P antibodies

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18
Q

Significance of ANA

A

Seen in all SLE cases
Not specific for SLE

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19
Q

Significance of anti-dsDNA

A

Specific for SLE
Serum level of antibody correlates with disease activity

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20
Q

Significance of anti-Sm antobodies
What is the antigen to these antobodies

A

Specific for SLE
Serum level of antibody does NOT correlates with disease activity

antigen is ribonucleoprotein

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21
Q

Significance of anti-phospholipid antibodies (a.k.a. anti-cardolipin antibodies)

A

associated with risk of arterial and venous thrombosis in SLE

may also occur in absence of SLE in what is termed the ‘primary anti-phospholipid antibody syndrome

22
Q

Significance of Anti-Ro antibodies and anti-La antibodies (antigen is ribonucleoprotein for both btw)

A

Secondary Sjögren’s syndrome
Neonatal lupus syndrome (transient rash in neonate, permanent heart block)

23
Q

Significance of Anti-ribosomal P antibodies

A

Cerebral lupus

24
Q

SLE investigations

A

Inflammation:
high ESR but C-reactive protein is typically normal unless infection or serositis/arthritis
Haematology:
Haemolytic anaemia, Lymphopenia, Thrombocytopenia
Renal:
very important to measure urine protein (most commonly urine protein:creatinine ratio [uPCR])
look at albumin
Immunological:
Antinuclear antibodies
Anti-double-stranded DNA antibodies - highly specific, correlate with disease activity
Complement consumption – e.g. low C4 and C3

Clotting – antiphospholipid antibodies
Lupus anticoagulant and anti-cardiolipin antibodies

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

25
Q

What would happen to anti-dsDNA levels and complement levels in unwell lupus patients

A

Unwell lupus patient has LOW complement C3 and C4 levels and HIGH levels of anti-ds-DNA antibodies

26
Q

What happens to eGFR, serum creatinine and urea, blood albumin level and urine protein level/ urine protein creatine ratio in SLE?

A

Fall in eGFR, leading to elevated serum urea and creatinine
Hypoalbuminuria and proteinuria/ elevated urine protein: creatine ratio in urine

27
Q

What would happen to blood hemoglobin, platelet count, lymphocyte count and reticulocyte count in SLE

A

Fall, fall, fall, increase

28
Q

What are the aims of treatment of SLE?

A

Induce remission and prevent relapse

29
Q

How is SLE treated?

A

Hydroxychloroquine is recommended in all patients with lupus
Maintenance treatment glucocorticoids should be minimised and, when possible, withdrawn.
Appropriate initiation of immunomodulatory agents (methotrexate, azathioprine, mycophenolate) can expedite the tapering/discontinuation of glucocorticoids
In persistently active or severe disease we use cyclophosphamide and B cell targeted therapies (rituximab and belimumab)

30
Q

What is Sjogren’s syndrome

A

Autoimmune exocrinopathy
lymphocytic infiltration of especially exocrine glands and sometimes of other organs (extra-glandular involvement)

31
Q

What symptoms does exocrine gland pathology cause in Sjogren’s syndrome

A

Dry eyes (xerophthalmia)
Dry mouth (xerostomia)
Parotid gland enlargement

32
Q

Extra-glandular manifestations of Sjogren’s sydrome

A

Commonest extra-glandular manifestations are non-erosive arthritis and Raynaud’s phenomenon

33
Q

What is secondary Sjogren’s syndrome

A

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

34
Q

What do we see on salivary gland biopsy in Sjogren’s syndrome

A

Lymphocytic infiltration predominantly CD4 helper T cells and to lesser extent B lymphocytes

35
Q

What test can be used to assess tear production?

A

Schirmer’s test – a test to assess tear production. Filter paper is placed under lower eyelid and extent of wetness measured after 5 minutes. Abnormal is <5mm after 5 minutes

36
Q

What is inflammatory muscle disease

A

Proximal muscle weakness due to autoimmune-mediated inflammation either with (dermatomyositis) or without (polymyositis) a rash

37
Q

Skin changes in dermatomyositis

A

Lilac-coloured (heliotrope) rash on eyelids, malar region and naso-labial folds
Red or purple flat or raised lesions on knuckles (Gottron’s papules)
Subcutaneous calcinosis
Mechanic’s hands (fissuring and cracking of skin over finger pads)

38
Q

Abnormal investigations in INFLAMMATORY MUSCLE DISEASE

A

Elevated CPK, abnormal electromyography, abnormal muscle biopsy (polymyositis = CD8 T cells; dermatomyositis = CD4 T cells in addition to B cells)

39
Q

What conditions are associated with inflammatory muscle disease?

A

Malignancy, pulmonary fibrosis

40
Q

What is scleroderma

A

Thickened skin with Raynaud’s phenomenon
Dermal fibrosis, cutaneous calcinosis and telangiectasia

41
Q

What are the two broad categories of scelroderma

A

Diffuse systemic sclerosis
Limited systemic sclerosis

42
Q

Symptoms of diffuse systemic sclerosis
What autoantibody is associated with it?

A

Fibrotic skin proximal to elbows or knees (excluding face and neck)
Pulmonary fibrosis, renal (thrombotic microangiopathy) involvement
Short history of Raynaud’s phenomenon
Anti-topoisomerase-1 (anti-Scl-70) antibodies

43
Q

Symptoms of limited systemic sclerosis?
What autoantibody is involved?

A

Fibrotic skin hands, forearms, feet, neck and face
Pulmonary hypertension
Long history of Raynaud’s phenomenon
Anti-centromere antibodies

44
Q

What is a subtype of limited systemic sclerosis

A

CREST
It stands for Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia

45
Q

Digital ischemia in scleroderma

A
46
Q

What is overlap syndrome

A

When features of more than 1 connective tissue disorder are present e.g. SLE and inflammatory muscle disease we can use the term overlap syndrome

47
Q

What term describes presence of incomplete features of a connective tissue disease

A

undifferentiated connective tissue disease

48
Q

What is mixed connective tissue disease and what autoantibody is associated with it?

A

a group of patients with features of seen in SLE, scleroderma, rheumatoid arthritis, and polymyositis were identified by the presence of an autoantibody:
Anti-U1-RNP antibody
….this condition was termed Mixed Connective Tissue Disease (‘MCTD’)
((U1-RNP are uridine-rich small nuclear ribonucleoproteins))

49
Q

Autoantibodies associated with Dermato-/Polymyositis

A

Anti-tRNA transferase antibodies
E.g. histidyl transferase (also termed anti-Jo-1 antibodies)

50
Q

Autoantibodies associated with Sjögren’s syndrome

A

No unique antibodies but typically see
Antinuclear antibodies - Anti-Ro and anti-La antibodies
Rheumatoid factor

Aneesha Flashcards BRS 2 (47 decks)

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