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MSK > Connective tissue disorders > Flashcards

Connective tissue disorders Flashcards

1
Q

What is the pathophysiology of SLE?

A

Anti-nuclear antibodies (ANA) target proteins within the patient’s own nucleus, generating an inflammatory response and leading to SLE

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2
Q

What general features are present in SLE?

A

Fatigue
Fever
Mouth ulcers
Lymphadenopathy

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3
Q

What skin conditions can occur in SLE?

A 
Malar rash
Discoid rash - scaly, erythematous, well-distinguished rash in sun-exposed areas
Photosensitivity
Raynaud's phenomenon
Livedo reticularis
Non-scarring alopecia
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4
Q

What MSK conditions can occur in SLE?

A

Arthralgia

Non-erosive arthritis

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5
Q

What cardiovascular conditions can occur in SLE?

A

Pericarditis

Myocarditis

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6
Q

What respiratory conditions can occur in SLE?

A

Pleurisy

Fibrosing alveolitis

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7
Q

What renal conditions can occur in SLE?

A

Proteinuria

Glomerulonephritis

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8
Q

What neuropsychiatric conditions can occur in SLE?

A

Anxiety and depression
Psychosis
Seizures

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9
Q

What investigations are used to detect and monitor SLE?

A 
Antibodies:
99% are ANA +ve
20% are RF +ve
Anti-dsDNA
Anti-Smith
Others include anti-U1 RNP, anti-Ro and anti-La

Monitoring:
Inflammatory markers - ESR and CRP
Complement levels - C3, C4
Anti-dsDNA titres used for disease monitor

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10
Q

What treatment is given in SLE?

A

1st line = NSAIDs, prednisolone, hydroxycholorquine and suncream with sun avoidance for photosensitive malar rash

If resistant or severe SLE:
Methotrexate
Mycophenolate mofetil
Azathioprine
Tacrolimus
Leflunomide
Ciclosporin
Biological therapies
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11
Q

What is the WHO classification for lupus nephritis?

A

Class I = normal kidney
Class II = mesangial glomerulonephritis
Class III = focal and segmental proliferative glomerulonephritis
Class IV = diffuse proliferative glomerulonephritis
Class V = diffuse membranous glomerulonephritis
Class VI = sclerosing glomerulonephritis

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12
Q

What is the most common type of lupus nephritis?

A

Diffuse proliferative glomerulonephritis

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13
Q

How is lupus nephritis treated?

A

Treat hypertension
Corticosteroid if clinical evidence of disease
Immunosuppressants e.g. azathioprine/cyclophosphamide

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14
Q

What is the relevance of Ro (SSA) and La (SSB) +vity in pregnancy?

A

Anti-Ro and Anti-La can cross the placenta and cause neonatal lupus.
This can include a lupus rash, complete heart block and blood abnormalities such as cytopenias

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15
Q

What serious side effect can occur with hydroxychloroquine?

A

Bull’s eye retinopathy:

May cause severe/permanent visual loss

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16
Q

What acquired disorder, that predisposes patients to venous and arterial thromboses, commonly occurs secondary to SLE?

A

Antiphospholipid syndrome

17
Q

What are the features of antiphospholipid syndrome?

A

Venous/arterial thrombosis
Recurrent fetal loss
Livedo reticularis
Thrombocytopenia
Prolonged APTT - due to reaction of lupus anticoagulant antibodies with phospholipids involved in the coagulation cascade
Elevated antiphospholipid (AP) antibodies

May also have:
Pre-eclampsia
Pulmonary hypertension

18
Q

What is the management for antiphospholipid syndrome?

A

Primary thromboprohylaxis:
Low-dose aspirin

Secondary thromboprophylaxis:
Initial VTE event - lifelong warfarin with target INR of 2-3
Recurrent VTE event - lifelong warfarin; if occurred whilst on warfarin then add low-dose aspirin and increase target INR to 3-4
Arterial thrombosis - lifelong warfarin with target INR of 2-3

19
Q

What features may be present in drug-induced lupus?

A

Arthralgia
Myalgia
Skin changes (e.g. Malar rash) and lung involvement (e.g. pleurisy) are common

20
Q

What investigations can be carried out in drug-induced lupus?

A

ANA +ve in 100%
Anti-dsDNA -ve
Anti-histone antibodies found in 80-90%
Anti-Ro and Anti-Smith +ve in 5%

21
Q

What drugs can cause drug-induced lupus?

A 
Procainamide
Hydralazine
Isoniazid
Minocycline
Phenytoin
22
Q

What antibody should you look for if suspected antiphospholipid syndrome?

A

Anti-cardiolipin antibody

23
Q

What are the features of fibromyalgia?

A

Usually affects women and presents between 30-50 years old

Chronic pain at multiple sites - 'pain all over' 
Lethargy 
Cognitive impairment - 'fibro fog' 
Sleep disturbance 
Headaches 
Dizziness
24
Q

How is fibromyalgia diagnosed?

A

Diagnosis is clinical and refers to the American College of Rheumatology (ACR) that lists 9 pairs of tender points on the body. If 11/18 points are tender, then diagnosis of fibromyalgia is more likely

25
Q

What management is given in fibromyalgia?

A

Explanation
Exercise
CBT
Medication - pregablin, duloxetine, amitriptyline, tramadol

26
Q

What are the different patterns of systemic sclerosis?

A

Limited cutaneous systemic sclerosis
Diffuse cutaneous systemic sclerosis
Scleroderma - tightening and fibrosis of skin without internal organ involvement

27
Q

What are the features of limited cutaneous systemic sclerosis?
What antibody is this disease associated with?

A 
Scleroderma of face and distal limbs
CREST syndrome:
Calcinosis
Raynauds
oEsophageal dysmotility
Sclerodactyly
Telangiectasia

Associated with anti-centromere antibodies

28
Q

What are the features of diffuse cutaneous systemic sclerosis?
What antibody is this disease associated with?

A

Scleroderma of trunk and proximal limbs
ILD and pulmonary hypertension - most common cause of death in this disease
Renal disease
Hypertension

Associated with scl-70 antibodies

29
Q

What antibodies are present in systemic sclerosis?

A

ANA +ve in 90%
RF +ve in 30%
Anti-centromere in limited cutaneous
Anti-scl-70 in diffuse cutaneous

30
Q

What condition is at a greater risk of occurring in Sjogren’s syndrome?

A

Lymphoid malignancy

31
Q

What are the features of Sjogren’s syndrome?

A 
Dry eyes
Dry mouth
Vaginal dryness
Arthralgia
Raynauds
Myalgia
32
Q

What investigations can be used to diagnose Sjogren’s syndrome?

A 
Antibodies:
ANA +ve in 70%
RF +ve in 50%
Anti-Ro antibodies
Anti-La antibodies

Schirmer’s test

Histology - may find focal lymphocytic infiltration

33
Q

What management is given in Sjogren’s syndrome?

A

Artificial saliva and tears

Pilocarpine may stimulate saliva production

MSK (21 decks)

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