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Medicine > 11 - Muscle and Soft Tissue Rheumatology > Flashcards

11 - Muscle and Soft Tissue Rheumatology Flashcards

1
Q

What are some autoimmune connective tissue disorders?

A
  • SLE
  • Systemic sclerosis
  • Sjogren’s
  • Polymyositis and Dermatomyositis
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2
Q

If someone has a multisystem disorder with raised ESR but normal CRP, what should you consider as the diagnosis?

A

SLE

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3
Q

What is the general pathophysiology of SLE?

A

Multisystemic autoimmune disease that relapses and remits

Autoantibodies made against autoantigens which then form immune complexes

- Inadequate clearance of immune complexes cause immune responses in tissues, leading to inflammation and tissue damage

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4
Q

What are some risk factors for developing SLE?

A

- Female

  • Child-bearing age

- Afro-Caribbean or Asian

- 1st or 2nd degree relative

  • UV light
  • Drugs
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5
Q

What are some common symptoms and signs of lupus?

A

Remitting and relapsing disease that often has non specific features like malaise, fatigue, myalgia and fever

Other features: alopecia, weight loss, lymphadenopathy, Raynaud’s

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6
Q

What investigations can you do if you suspect lupus and what will they show?

A

- ESR/PV: raised

- CRP: normal

- FBC: anaemia and leukopenia

- Autoantibodies: ANA, anti-dsDNA, anti-Ro, anti-La, antiphospholipid antibodies

- C3/C4: decreased in active lupus

- Urinalysis: may have haematuria if renal disease

- Biopsy: skin and renal, can be diagnostic

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7
Q

If a patient with lupus has antiphospholipid antibodies, what can this increase the chance of?

A
  • Pregnancy loss
  • Thrombosis

HIGH CVD RISK IN SLE REGARDLESS

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8
Q

How is SLE diagnosed?

A

SLE can mimic other diseases. Take skin/renal biopsies

Need 4 or more criteria (with at least 1 clinical and 1 labatory)

OR

Biopsy proven lupus nephritis with positive ANA or anti-dsDNA

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9
Q

How can we monitor SLE activity to monitor for flares?

A

- Anti-dsDNA antibody titres

- C3/C4 levels (will be low in flares as consumption)

- ESR

- Urine for protein and erythrocyte casts

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10
Q

What are some drugs that cause drug-induced lupus?

A
    • Isoniazid*
    • TNF-a inhibitors*
  • Chlorpromazine
  • Phenytoin
    • Hydralazine*
  • Procainamide
  • Minocycline

Stop drug for resolution

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11
Q

What are some drugs that can worsen idiopathic SLE?

A
  • Oral contraceptives
  • Sulfonamides
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12
Q

What is the management for lupus? (include general measure, maintenance and flares)

A

General measures: high factor sun-block, advice on healthy lifestyle due to CVD risk, topical steroids for skin flares, screen for co-morbidities and drug toxicity

Maintenance: Hydroxychloroquine for rash and arthralgia. NSAIDs if no renal disease. Mycophenolate mofetil, Azathioprine, Rituximab are used for steroid sparing

Flares: short course of low-dose steroids such as prednisolone, if organ damage use DMARDs or mycophenolate

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13
Q

How is lupus nephritis treated differently to lupus?

A

Intesive immunosuppression to induce remission using steroids and cyclophosphamide before maintenance

BP control needed.

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14
Q

What is antiphospholipid syndrome?

A

Antiphospholipid antibodies, often associated with SLE

Coagulation defect

Livedo reticularis

Obstetric (recurrent miscarriages)

Thrombocytopenia

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15
Q

What is Raynaud’s phenomenon?

A

- Peripheral digital ischaemia due to vasospasm.

- Painful and sequence of colour changes that are bilateral over few minutes

  • Usually precipitated by cold and emotion
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16
Q

What is Raynaud’s syndrome?

A

When Raynaud’s phenomenon is idiopathic.

Common in young women. Tell them to keep warm and avoid smoking

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17
Q

What are some causes of Raynaud’s?

A

- SLE

  • Scleroderma
  • Dermatomyositis and polymyositis

- Sjogren’s

- Drug induced by beta blockers

  • Heavy vibrating tools
  • Cervical rib
  • Sticky blood
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18
Q

How is Raynaud’s treated?

A

Conservative:

  • Stop smoking
  • Keep warm e.g gloves, handwarmers

Pharmacological:

- CCB’s like nifedipine 1st line.

  • PDE-5 inhibitors like sildenafil and epoprostenol also good
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19
Q

How does the pattern of Raynaud’s vary between idiopathic and secondary causes?

A

Idiopathic: last minutes and bilateral

Secondary: can last hours and can be asymmetrical with only a few digits involved

20
Q

What are some complications of Raynaud’s?

A
  • Digital ulcers
  • Infection
  • Gangrene
21
Q

What is nail fold capillaroscopy?

A

Non-invasive way of looking at structural changes in peripheral microcirculation.

Helps with diagnosis of scleroderma and DM with Raynaud’s

22
Q

What is vasculitis?

A

Inflammatory blood vessel disorder that gets clinical features from damage of blood vessel walls with subsequent thrombosis, ischaemia, bleeding and aneurysm formation

23
Q

How is a diagnosis of vasculitis made?

A
  • History using glove sweater trunk approach
  • Physical exam
  • Urine dipstick to check for glomerulonephritis
  • Tests to exclude other diagnoses
24
Q

How is vasculitis categorised, and give some examples in each category?

A

Size of blood vessel

Small: MPA, Wegener’s granulomatosis, IgA vasculitis, Goodpasture’s

Medium: Kawasaki disease, polyarteritis nodosa

Large: GCA, Takayasu’s arteritis

25
Q

How is vasculitis managed?

A
  • Rule out infection and stop any drugs in secondary causes
  • Large: corticosteroids

- Medium/Small: immunosuppresion with biological agents or immunosuppressants

26
Q

What is dermatomyositis and polymyositis?

A
  • Autoimmune muscle diseases that have symmetrical inflammation of striated muscle leading to muscle weakness
  • Can present with shortness of breath due to muscle weakness
  • Also may have fever, arthralgia, Raynaud’s, interstitial lung fibrosis and myocardial involvement
27
Q

How does dermatomyositis present differently to polymyositis?

A

Myositis plus skin signs that are photosensitive:

  • Macular rash (shawl sign)
  • Heliotrope rash on eyelids
  • Nailfold erythema
  • Gottron’s papules (knuckles, elbows and knees)
28
Q

What is the diagnostic criteria for dermatomyositis and polymyositis?

A
  • Myositis can be demonstrated on MRI
  • Raised CK
29
Q

How is dermatomyositis and polymyositis treated?

A

- High dose corticosteroids (Prednisolone) initially

- Methotrexate or Azathioprine long term

- IVIG

  • Sun protection
  • Hydroxychloroquine can help rash
30
Q

What is systemic sclerosis and the pathophysiology of this?

A

Multisystemic autoimmune disease that has:

- Scleroderma (skin fibrosis)

- Internal organ fibrosis

- Microvascular abnormalities

Due to increased fibroblast activity

31
Q

What are the two types of scleroderma and how do they present?

A

Limited/CREST syndrome

  • More common
  • Face, hands and feet

Diffuse

  • Less common and higher mortality risk
  • Involves whole body
  • Sudden onset skin involvement
32
Q

What is CREST syndrome?

A
  • Calcinosis cutis
  • Raynaud’s phenomenen
  • Esophageal dysmotility
  • Sclerodactyly
  • Telengiectasia
33
Q

What invesitgations are done for systemic sclerosis and what do they show?

A

- Inflammatory markers: normal

- X-Ray hands: calcinosis

- CXR, HRCT, PFT: pulmonary disease

- ECG and ECHO: PA HTN, heart failure, arrhythmias

- ANA: positive

34
Q

How is systemic sclerosis managed?

A
  • No cure
  • CCB/Sildenafil for renal crisis
  • Methotrexate and MMF for skin thickening
  • ACEi to prevent hypertensive crisis
  • Prednisolone for flares
  • PPI for GI symptoms
35
Q

What is Sjogren’s syndrome?

A

Chronic autoimmune disorder that can be primary or secondary.

Characterised by diminished lacrimal and salivary gland secretion due to lymphocytic infiltration and fibrosis of exocrine glands

Usually affects women

36
Q

What are some of the common symptoms and signs of Sjogren’s?

A

MADFRED

  • Myalgia
  • Arthralgia
  • Dry mouth
  • Fatigue
  • Raynaud’s
  • Enlarged parotids
  • Dry eyes and dry mouth
37
Q

What investigations are done to diagnose Sjogren’s?

A

ANA: Anti-Ro and Anti-La usually positive, RF and anti-dsDNA can also be positive

Schirmer’s test: measures conjunctival dryness

Salivary gland biopsy: shows lymphocyte aggregation

38
Q

How is Sjogren’s treated?

A

Symptomatic treatment:

  • Artifical tears
  • Artifical saliva, drink frequently, sugar free sweetd
  • Vaginal lubricants
  • NSAIDs and HCQ can be used for arthralgia
39
Q

What other conditons is Sjogren’s associated with?

A
  • RA
  • SLE
  • Coeliac
  • Primary billiary cirrhosis
  • Autoimmune thyroid disease
40
Q

What malignancy is Sjogren’s associated with?

A

B cell lymphoma

41
Q

What can dermatomyositis be caused by?

A

Paraneoplastic syndrome due to malignancy

42
Q

What is this and what can it indicate?

A

Abnormal nailfold capillaries which is common in SLE and Sjogren’s

43
Q

What autoantibodies are found in SLE?

A

ANA most common

44
Q

What are some examination findings with SLE?

A
  • Low grade fever
  • Pallor
  • Alopecia
  • Arthritis
  • Erythematous rash over face
45
Q

What is a useful mneumonic to remember the diagnostic criteria for SLE?

A
46
Q

What are some features in SLE that might suggest active disease (a flare up)?

A
  • Low C3/C4
  • Raised inflammatory markers
  • Raised anti-dsDNA
  • Proteinuria
  • Rash, alopecia, fatigue
47
Q
A

Medicine (30 decks)

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