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MSK week 2 > Cortex rheumatology - Inflammatory arthropathies 3 > Flashcards

Cortex rheumatology - Inflammatory arthropathies 3 Flashcards

1
Q

Connective tissues diseases are seropositive inflammatory arthropathies - what are the most common ones ?

A
  • Systemic lupus erythematosus (SLE)
  • Sjogrens syndrome
  • Systemic sclerosis
  • Mixed connective tissue disease
  • Anti-phospholipid syndrome.

These tend to be multisystem disorders and cause organ pathology

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2
Q

What is SLE?

A
  • A chronic autoimmune disease in which the presentation and disease course can be highly variable
  • It mainly involves the skin, joints, kidneys, blood cells, and nervous system but can affect almost any organ
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3
Q

Who is at most risk of developing SLE?

A

Women 11:1 during child-bearing years (20s and 30s)

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4
Q

What are the key diagnostic features seen on history and examination of SLE?

A
  • Malar (butterfly) rash
  • Photosensitive rash
  • Discoid rash
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5
Q

Describe the pathogenesis of SLE

A
  • Defective apoptosis causes increased cell death.
  • The defective clearance of the apoptotic cell debris allows for the persistence of antigen and immune complex production.
  • Many of the clinical manifestations result from the circulating immune complexes forming with antigens in various tissues
  • Leading to complement activation and inflammation
  • Antibody-antigen complexes are deposited on the basement membranes of skin and kidneys.
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6
Q

What are some of the symptoms of SLE?

A

(Note SLE pretty much affects everything so try to look out for the presence of antibiody specific to it and also key features of it)

  • General - fever, fatigue, mouth ulcers & lymphadenopathy
  • Musculoskeletal: arthralgia, myalgia and inflammatory arthritis (non-erosive)
  • Skin - malar (butterfly) rash: spares nasolabial folds, discoid rash (scaly, erythematous, well demarcated rash in sun-exposed areas.), photosensitivity, Raynaud’s phenomenon, livedo reticularis, non-scarring alopecia
  • Renal: proteinuria, glomerulonephritis (diffuse proliferative glomerulonephritis is the most common type)
  • Respiratory: pleurisy & interstitial lung disease (fibrosing alveolitis)
  • Haematological: leukopenia, lymphopenia, anaemia (may be haemolytic), and thrombocytopenia
  • Neuropsychiatric: seizures, psychosis, headache, aseptic meningitis
  • Cardiac: pericarditis, myocarditis
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7
Q

What are some of the key investigations for SLE ? (note there is not one diagnostic test)

A

1st investigations to order:

  • FBC and differential
  • activated PTT
  • urea and electrolytes
  • ESR and CRP
  • antinuclear antibodies, dsDNA, Smith antigen
  • urinalysis
  • chest x-ray
  • ECG
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8
Q

What are the tests done to monitor SLE?

A
  • Urinanalysis - to look for blood and protein in the urine indicating glomerulonephritis
  • Monitoring cardiac risk factors - BP, chiolestrol etc
  • Check anti-dsDNA antibodies and complement levels regularly
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9
Q

What are the auto-antibodies associated with SLE?

A

Immunology:

  • Anti-nuclear antibody - positive in >95% of patients, not specific
  • Anti-dsDNA antibody - specific and varies with disease activity
  • Anti-Sm - specific but low sensitivity
  • Anti Ro, anti-La and anti-RNP - may be seen in SLE but may also be seen in other conditions
  • C3/4 levels - low when disease active, especially renal disease
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10
Q

What is the best auto-antibody test for SLE?

A

Anti-dsDNA

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11
Q

What is the main overall treatment of SLE?

A
  • 1st line: NSAID + lifestyle changes
  • adjunct: hydroxychloroquine
  • adjunct: corticosteroids - prednisolone
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12
Q

What is sjorgens syndrome ?

A

An auto-immune disorder characterised by the presence of dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia) as a consequence of lymphocytic infiltration into the lacrimal and salivary glands.

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13
Q

What are the key signs/symptoms of sjogrens syndrome?

A
  • Fatigue
  • Dry eyes
  • Dry mouth
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14
Q

What are some of the other (possibly less common) signs of sjorgens syndrome ?

(add in pic of parotid gland swelling)

A

Other symptoms include:

  • Arthralgia,
  • Fatigue
  • Vaginal dryness
  • Parotid gland swelling

(try think its the disease which causes dryness and swelling in the neck)

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15
Q

What are thinvestigations used to diagnose sjorgens syndrome ?

A

​1st investigatiojns to order:

  • Schirmer’s test
  • anti-60 kD (SS-A) Ro and anti-La (SS-B)
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16
Q

What is the treatment of sjorgens syndrome ?

A
  • Lubricating eyedrops are used and saliva replacement products may be tried
  • Tx for arthralgia = NSAID’s + hydroxychloroquine
17
Q

What does sjorgens increase the risk of ?

A

Non-hodgkins lymphoma

18
Q

What is systemic sclerosis ?

A
  • A systemic autoimmune connective tissue disorder.
  • Where excessive collagen deposition causes skin and internal organ changes
19
Q

Describe the presentation of systemic sclerosis and how a diagnosis is reached from an examination

A

Raynaulds is a common early finding (uncommon to not have it)

Skin becomes thickened and tight

Features are divided into major and minor:

  • Major: includes centrally located skin sclerosis that affects the arms, face (pinching of the skin of nose (‘beaking’)), and/or neck
  • Minor: includes sclerodactyly (thickened and tight skin of the fingers and toes) and bilateral lung fibrosis.
20
Q

Systemic sclerosis can affect organs what are some of the common complications occuring in systemic sclerosis due to organ involvement ?

A

Pulmonary hypertension, Pulmonary fibrosis and accelerated hypertension leading to renal crisis are important organ manifestations.

21
Q

What are the two different classifications of systemic sclerosis and how do they differ in presentation ?

A

Diffuse and limited

  • Diffuse - skin changes develop more rapidly and can involve the trunk. Early significant organ involvement
  • Limited - skin involved tends to be confined to face, hands and forearms and feet. Organ involvement tends to occur later.
22
Q

What are the investigations done to help diagnose systemic sclerosis ?

A

Autoantibody tests:

  • Diffuse - anti-scl-70 antibody
  • Limited - anti-centromere

Organ screening performed regularly also - pulmonary function testing, echo and monitoring of renal function.

23
Q

Describe the main idea of the management of systemic sclerosis

A

Management tends to be tailored to the specific issues below:

  • Raynauds/digital ulcers: calcium channel blockers (nifedipine), Iloprost, bosentan
  • Renal involvement: ACE inhibitors
  • GI involvement: proton pump inhibitors for reflux
  • Interstitial lung disease: immunosuppression, usually with cyclophosphamide.
24
Q

What is meant by mixed connective tissue disease?

A

It is a condition which features symptoms also seen in other connective tissue diseases, these include:

  • Raynauds phenomenon
  • Arthralgia/arthritis
  • Myositis
  • Sclerodactyly
  • Pulmonary hypertension
  • Interstitial lung disease (ILD)
25
Q

What investigations should be taken in patients with mixed connective tissue disease ?

A

Associated with anti-RNP antibodies

  • Risk of pulmonary hypertension so regular echocardiograms
  • Screening for ILD with pulmonary function tests
26
Q

What is the management of mixed connective tissue disease?

A

Management as with most connective tissue disease varies dependant on its presentation e.g. immunosuppression if significant muscle or lung disease, CCB for Reynolds etc

27
Q

What is Anti-phospholipid syndrome?

A

A disorder that manifests clinically as recurrent venous or arterial thrombosis and/or fetal loss.

28
Q

What are the common presenting features of antiphospholipid syndrome ?

A
  • Increased frequency of stroke or MI, especially in younger individuals.
  • Recurrent pulmonary emboli or thrombosis can lead to life-threatening pulmonary hypertension.
  • Spontaneous fetal loss prior to 34 weeks due to eclampsia, pre-eclampsia or with evidence of placental insufficiency.
  • A common cutaneous finding is of livedo reticularis (shown in the pic below)
29
Q

What are the investigations and antibodies associated with antiphospholipid syndrome ?

A

Antibodies:

  • Lupus anticoagulant
  • Anti-cardiolipin antibodies
  • Anti-beta 2 glycoprotein

There may be thrombocytopenia (low platelets) and prolongation of APTT (activated partial thromboplastin time - measures clotting time)

30
Q

What is the treatment of antiphospholipid syndrome ?

A

Mainstay is anti-coagulation:

non-pregnant with acute thrombosis:

  • 1st line:low molecular weight heparin
  • plus: warfarin following low molecular weight heparin
  • plus: management of risk factors

pregnant

with acute thrombosis

  • 1st line: low molecular weight heparin
  • plus: fetal monitoring + specialist multidisciplinary care
  • plus: warfarin post-delivery
  • plus: management of risk factors

(remember warfarin is teterogenic)

Long term Mx:

  • Life long warfarin with a target INR of 2-3

MSK week 2 (7 decks)

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