Connective Tissue Disorder Flashcards

1
Q

What is Sjogren’s Syndrome?

A
  • Chronic autoimmune disorder affecting exocrine glands resulting in dry mucosal surfaces.
  • Diminished lacrimal and salivary gland secretion
  • May be primary (PSS) or secondary to rheumatoid arthritis or other connective tissue disorders (10 years after the initial onset).
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2
Q

What are associations with Sjorgren’s Syndrome?

A
  • Sjogren’s syndrome is much more common in females (ratio 8:2)
  • There is a marked increased risk of lymphoid malignancy (40-60 fold)
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3
Q

What are common symptoms of Sjorgren’s?

A

MADFRED

  • Myalgia
  • Arthralgia
  • Dry mouth
  • Fatigue
  • Raynaud’s
  • Enlarged Parotids (parotitis recurrent)
  • Dry eyes: keratoconjunctivitis sicca
  • Vaginal dryness, Sensory polyneuropathy, Renal tubular acidosis (usually subclinical)
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4
Q

What is the investigations for Sjorgren’s?

A
  • Schirmer’s test: filter paper near conjunctival sac to measure tear formation
  • BLOOD TEST
    • Anti-Ro (SSA) and Anti-La (SSB) antibodies in 30% of patients with PSS
    • Rheumatoid factor (RF)+ (100%) of patients and ANA+ (70%)
    • Hypergammaglobulinaemia
    • Low C4
  • Histology: focal lymphocytic infiltration
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5
Q

How is Sjorgrens managed?

A
  • Avoid dry or smoky atmospheres
  • Dry eyes: artificial tears
  • Dry mouth: artificial saliva, sugar free gum/pastilles, pilocarpine may stimulate saliva production
  • Skin emollients
  • Vaginal lubricants
  • Immunosuppressants/steroids rarely needed
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6
Q

What is Dermatomyosistis and Polymyosistis?

A
  • Rare idiopathic muscle disease that are characterized by inflammation of striated muscle. Proximal muscles affected symmetrically
    • Polymyositis is a variant of the disease where skin manifestations are not prominent
  • Can also be due to underlying malignancy (typically ovarian, breast and lung cancer) and connective tissue disorders
  • M:F similar and peak age of onset is 40-50 years
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7
Q

What are signs and symptoms of Dermatomyositis and Polymyositis?

A
  • Skin features
    • Photosensitive – can lead to hyper or hypo-pigmentation
    • Macular rash over back and shoulder
    • Heliotrope rash in the periorbital region
    • Gottron’s papules - roughened red papules over extensor surfaces of fingers
    • Nail fold capillary dilatation
  • Other features
    • Proximal muscle weakness +/- tenderness
    • Raynaud’s
    • Respiratory muscle weakness – diaphragm
    • Interstitial lung disease: e.g. Fibrosing alveolitis or organising pneumonia
    • Dysphagia, dysphonia – upper oesophagus has striated muscle which can be affected and can lead to aspiration pneumonia
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8
Q

What are diagnostic criteria for Dermatomyositis and Polymyositis?

A
  • PM if >= 3 of the first 4 and DM if rash and >= 2 of the first 3 of below criteria:
    • Symmetrical proximal muscle weakness
    • Raised serum muscle enzyme levels e.g.
    • Typical electromyographic (EMG) changes
    • Biopsy evidence of myositis
    • Typical rash of dermatomyositis
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9
Q

What are investigations for Dermatomyositis and Polymyositis?

A
  • Raised inflammatory mrkers
  • Raised ALT
  • Majority of patients are ANA positive, with around 25% anti-Mi-2 positive
    • anti-jo
  • Well demonstrated on MRI
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10
Q

How is Dermatomyositis and Polymyositis managed?

A
  • High dose corticosteroids are the mainstay in the first few weeks.
  • Monitoring disease activity can be difficult but the inflammatory markers and CK are often used. Repeat EMG studies/MRI or biopsy may be needed.
  • Long term control is with MTX or AZA. Rituximab can be effective. Intravenous immunoglobulin is also effective.
  • In DM, sun-protection is important and HCQ may also reduce the rash.
  • For most patients, DM and PM are long-term condition
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11
Q

What is Systemic Sclerosis?

A
  • Multisystem autoimmune disease due by increased fibroblast activity resulting in abnormal growth of connective tissue which leads to vascular damage and fibrosis
  • Characterised by hardened, sclerotic skin and other connective tissues.
  • It is four times more common in females
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12
Q

What are types of Systemic Sclerosis?

A
  • Limited cutaneous systemic sclerosis
  • Diffuse cutaneous systemic sclerosis
  • Scleroderma (without internal organ involvement)
    • tightening and fibrosis of skin
    • may be manifest as plaques (morphoea
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13
Q

What are signs and symptoms of Limited cutaneous systemic sclerosis?

A
  • Raynaud’s may be first sign
  • Scleroderma affects face and distal limbs predominately
  • Associated with anti-centromere antibodies
  • PA hypertension after a mean of 10 years of symptoms
  • CREST syndrome
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14
Q

What are signs and symptoms of Diffuse cutaneous systemic sclerosis?

A
  • Scleroderma affects trunk and proximal limbs predominately
  • Associated with scl-70 antibodies
  • Hypertension, lung fibrosis and renal involvement seen
    • Accelerated hypertension can lead to renal failure in diffuse SSC (mostly)
  • Less common but poorer prognosis
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15
Q

What are investigations and findings for Systemic Sclerosis?

A
  • Inflammatory markers usually normal
  • X-ray hand: calcinosis
  • CXR, HRCT, PFT: pulmonary disease
  • ECG & ECHO: PA hypertension, heart failure, myocarditis, arrhythmias
  • Antibodies
    • ANA positive in 90%
    • anti-scl-70 antibodies = diffuse cutaneous systemic sclerosis
    • anti-centromere antibodies = limited cutaneous systemic sclerosis
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16
Q

What is the management of Systemic Sclerosis?

A
  • No cure
  • Psychological support may be needed
  • Calcium antagonist/sildenafil/iloprost infusion for Raynaud’s symptoms
  • Methotrexate and Mycophenolate mofetil may reduce skin thickening
  • ACE-i prevent hypertensive crisis & reduce mortality from renal failure
  • Short courses of prednisolone for flares
  • Proton pump inhibitors for GI symptoms
17
Q

What is Fibromyalgia?

A
  • Chronic widespread pain throughout the body with tender points at specific anatomical sites. Appears in all 4 quadrants.
  • Allodynia, heightened and painful reponse to innocuous stimuli often present
  • Women are around 9 times more likely to be affected
  • Typically presents between 30-50 years old
18
Q

What is the pathogenesis of Fibromyalgia?

A
  • Can be induced by deliberate sleep deprivation.
    • EEG studies show reduced REM sleep and delta wave sleep.
  • This causes hyper-activation in response to noxious stimulation, and neural activation in brain regions associated with pain perception in response to non-painful stimuli.
19
Q

What are the symptoms and signs of Fibromyalgia?

A
  • Chronic pain: at multiple site, sometimes ‘pain all over’
  • Joint/muscle stiffness
  • Profound fatigue
  • Unrefreshed sleep
  • Numbness
  • Headaches
  • Irritable bowel/bladder syndrome
  • Depression and anxiety
  • Poor concentration and memory “fibrofog”
    • On examination, there should be no physical abnormalities to the MSK or neurological systems.
20
Q

What is the diagnostic criteria for Fibromyalgia?

A
  • Diagnosis is clinical and sometimes refers to the American College of Rheumatology classification criteria which lists 9 pairs of tender points on the body.
  • If a patient is tender in at least 11 of these 18 points it makes a diagnosis of fibromyalgia more likely
21
Q

How is Fibromyalgia managed?

A
  • Explanation
  • Aerobic exercise: has the strongest evidence base
  • Cognitive behavioural therapy
  • Medication: pregabalin, duloxetine, amitriptyline