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MBChB3 > Connective Tissue Disorders > Flashcards

Connective Tissue Disorders Flashcards

1
Q

Describe the clinical presentation of SLE

A
  • variable (mild - severe disease)
  • constitutional symptoms
  • cutaneous manifestations (individually does not = SLE)
  • arthralgia and arthritis
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2
Q

What is discoid lupus?

A

Lupus with cutaneous manifestations without any affect on internal organs

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3
Q

List the MSK manifestations of SLE

A
  • avascular necrosis
  • fibromyalgia
  • osteoporosis
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4
Q

List the renal manifestations of SLE and how to monitor this

A
  • end-stage renal failure (within 10y)
  • lupus nephritis
  • monitor with urinalysis, Us and Es and BP
  • monitor anti-ds-DNA antibodies (can predict flare up)
  • renal biopsy can help diagnosis, prognosis and treatment choice
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5
Q

List the pulmonary manifestations of SLE

A
  • pleurisy (inflammation of pleura)
  • pleural effusions
  • acute pneumonitis
  • diffuse alveolar haemorrhage
  • pulmonary hypertension
  • shrinking lung syndrome
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6
Q

List the cardiovascular manifestations of SLE

A
  • pericarditis +/- effusion
  • myocarditis
  • valvular abnormalities
  • coronary heart disease
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7
Q

List the neuropsychiatric manifestations of SLE and how you would monitor this

A
  • headache
  • anxiety and mood disorder
  • seizure
  • demyelination
  • Gullain-Barre Syndrome
  • mononeuritis
  • EEG, MRI, LP, psychiatric evaluation
  • measure anti-ribosomal P levels (associated with mood disorders)
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8
Q

List the GI manifestations of SLE

A

Not as common
- dysphagia
- reduced peristalsis
- peritonitis
- pancreatitis
- pseudo-obstruction
- lupus hepatitis (biopsy required)

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9
Q

List the haematological manifestations of SLE

A
  • anaemia of chronic disease
  • autoimmune haemolytic anaemia
  • thrombotic thrombocytopenia purpura (TTP)
  • leukopenia
  • associated lymphadenopathy and splenomegaly
  • thrombocytopenia
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10
Q

Describe the role of ANA in SLE

A
  • autoantibodies that attack self-proteins leading to activation of innate and adaptive immunity
  • sensitive but not specific for SLE
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11
Q

What is an ENA panel?

A
  • done when ANA tests positive
  • tests for the presence of 1 or more autoantibodies that react with proteins in the cells nucleus
  • knowing which antigens are affected can narrow down the diagnosis
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12
Q

Which ENA are specific for lupus?

A
  • Ro/La (SLE)
  • Ds/DNA (SLE)
  • Sm (SLE)
  • histone (drug induced lupus)
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13
Q

Which ENA are specific for systemic scleroderma?

A
  • centromere (limited)
  • Scl-70 (diffuse)
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14
Q

Describe the effect of SLE on complement

A
  • active disease = complement
  • C3 more specific
  • C4 can be chronically low
  • keep an eye on the trend more than the number itself
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15
Q

List the 3 licensed medications for SLE in the UK

A
  • steroids
  • hydroxychloroquine
  • belimumab
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16
Q

Describe the pharmacological management of SLE

A
  • NSAIDs and hydroxychloroquine for mild disease alone ± short courses of corticosteroids for flares
  • more organ involvement may require long-term corticosteroids + a DMARD as a ‘steroid sparing agent’ to reduce the steroid dose
  • severe flares causing serious renal, neurological or haematological effects need high dose corticosteroids + immunosuppressants (cyclophosphamide)
17
Q

What self-management should be employed for SLE?

A
  • sun protection
  • vaccination
  • exercise
  • avoid smoking
  • optimise body weight, blood pressure, lipids, glucose
18
Q

Describe scleroderma and differentials

A
  • characterised by skin thickening, progressive fibrosis and vascular disease
  • systemic = skin thickening of fingers extending proximal to the MCP joints
  • differentials = diabetic chieropathy, generalised morphia, eosinophilic fasciitis
19
Q

What are the other diagnostic features of systemic scleroderma?

A
  • skin changes
  • finger-tip lesions
  • telangiectasia
  • abnormal nail fold capillaries
  • pulmonary arterial hypertension and/or interstitial lung disease
  • Raynaud’s
  • related autoantibodies
20
Q

List the features of limited variant systemic scleroderma

A
  • distal skin involvement
  • skin calcification
  • telangiectasia
  • Raynaud’s
  • anti-centromere positive
  • pulmonary arterial hypertension
21
Q

List the features of diffuse variant systemic scleroderma

A
  • proximal skin involvement and trunk
  • Raynaud’s
  • early organ involvement (interstitial lung disease and pulmonary arterial hypertension, renal failure, myocardial disease, GI)
22
Q

List the cutaneous manifestations of systemic scleroderma

A
  • skin thickening
  • non-pitting oedema of hands and feet -> skin tightness
  • sclerodactyl
  • contractures
  • calcinosis
  • telangiectasia
23
Q

List the MSK manifestations of systemic scleroderma

A
  • arthralgia
  • myalgia
  • inflammatory arthritis and inflammatory myositis (less common)
  • tendon friction rubs
24
Q

List the vascular manifestations of systemic scleroderma

A
  • Raynaud’s
  • severe disease = digital ulcers with auto-amputations
25
Q

List the GI manifestations of systemic scleroderma

A

Common
- oesophageal dysmotility
- GORD
- small bowel hypoobility with bacterial overgrowth
- hypo mobility of large bowel + constipation
- pancreatic insufficiency

26
Q

List the respiratory manifestions of systemic scleroderma

A
  • interstitial lung disease
  • organising pneumonia
  • pulmonary hypertension
27
Q

List the renal manifestations of systemic scleroderma

A
  • renal crisis associated with corticosteroid use (avoid high and prolonged doses)
  • renal crisis associated with hypertension, progressive renal failure, microangiopathic haemolytic anaemia, seizures and encephalopathy
28
Q

List the cardiac manifestations of systemic scleroderma

A
  • arrhythmias
  • pericardial effusions
  • myocardial fibrosis
29
Q

List the treatment of systemic scleroderma

A
  • MSK: moisturiser, MTX, laser therapy for telangiectasia, analgesia
  • Raynaud’s: CCB, ACEi/fluoxetine, sildenafil, iloprost
  • pulmonary: mycophenolate mofetil, cyclophosphamide, lung transplant, stem cell transplant
  • GI: pro kinetics, PPI, H2 blockers, cyclical antibiotics, laxatives
  • cardiac: immunosuppressive, permanent pacemaker
  • renal: ACEi
30
Q

List the subtypes of idiopathic inflammatory myositis

A
  • polymyositis
  • dermatomyositis
  • overlap syndromes
  • juvenile PM/DM
  • drug induced
  • inclusion body myositis
31
Q

Describe the clinical manifestations of idiopathic inflammatory myositis

A
  • symmetrical, proximal muscle weakness
  • myalgia (<50%)
  • respiratory/diaphragm involvement
  • oesophageal involvement
  • face and neck involvement (rare)
  • distal disease (in IBM)
32
Q

List the cutaneous manifestations of idiopathic inflammatory myositis (dermatomyositis)

A
  • can precede muscle involvement
  • Gottrons papules (red/purple papule over MCP and PIP joints)
  • heliotrope rash + periorbital oedema
  • macular eruption (shawl sign, V sign)
  • calcinosi (children)
33
Q

List the systemic manifestations of idiopathic inflammatory myositis

A
  • overlap syndromes: CTD, anti-synthetase syndromes
  • malignancy: associated with dermatomyositis
  • non-specific symptoms: weight-loss, fever, fatigue
34
Q

What investigations would you do if you suspect idiopathic inflammatory myositis?

A
  • creatinine kinase levels
  • EMG (electromyography)
  • MRI of muscles
  • muscle biopsy
  • myositis specific antibodies
  • tumour screening if symptoms/red flags
35
Q

Describe the treatment of idiopathic inflammatory myositis

A
  • corticosteroids
  • immunosuppression (methotrexate, tacrolimus, azathioprine, mycophenolate mofetil)
  • IV immunoglobulin therapy
  • resistant disease = rituximab, cyclophosphamide

MBChB3 (100 decks)

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