Rheumatology Flashcards

1
Q

What is polymyositis

A

inflammation of the muscles

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2
Q

What is dermatomyositis

A

connective tissue disorder where there is chronic inflammation in the skin and muscles

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3
Q

Key diagnostic factor for dermatomyositis

A

creatine kinase

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4
Q

Presentation of dermatomyositis

A
  • Muscle pain, fatigue and muscle weakness
  • Occurs bilaterally and typically affects proximal muscles
  • Mostly affects the shoulder and pelvic girdle
  • Develops over weeks
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5
Q

Dermatomyositis skin features

A
  • Gottron lesions (scaly erythematous patches) on the knuckles, elbows and knees
  • Photosensitive malar rash on the back, shoulders and neck
  • Purple rash on eyelids and face
  • Periorbital oedema (swelling around the eyes)
  • Subcutaneous calcinosis (calcium deposits in the subcut tissue)
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6
Q

Autoantibody associated with polymyositis

A

Anti-Jo-1 antibodies

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7
Q

Autoantibodies associated with dermatomyositis

A

anti-m2 antibody
ANA antibody

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8
Q

How to diagnose dermatomyositis

A

clinical presentation
elevated CK
autoantibodies
EMG
Muscle biopsy for definitive diagnosis

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9
Q

Management of dermatomyositis

A

Guided by rheumatologist
corticosteroid - first line
Other: azathioprine, IV immunoglobulins, infliximab

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10
Q

Pathophysiology of SLE

A

characterised by ANA

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11
Q

Presentation of SLE

A

vague and non specific
Photosensitive malar rash - butterfly shaped rash over nose and cheekbones that gets worse with sunlight exposure

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12
Q

Investigations for SLE

A

autoantibodies
FBC - normocytic anaemia of chronic disease
C3 and C4 levels - decreased in active disease
CRP and ESR
Immunoglobulins - raised due to activation of B cells with inflammation
Urinalysis and protein:creatine ratio for proteinuria in lupus nephritis
renal biopsy to investigation lupus nephritis

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13
Q

Autoantibodies for SLE

A
  • ANA (anti nuclear antibodies)
    • Initial investigation for someone presenting with SLE
    • Can be positive in healthy patients or patients with other autoimmune conditions
    • Need to be taken in context of presenting symptoms
  • Anti-dsDNA (anti double stranded DNA)
    • More specific to SLE
    • Levels vary with disease activity so can be used for disease monitoring and response to treatment
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14
Q

How to diagnose SLE

A

SLICC criteria
ACR criteria

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15
Q

First line treatment for SLE

A

NSAIDs
Steroids - prednisolone
Hydroxyhlorquine (mild SLE)

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16
Q

What is rheumatoid arthritis

A

Autoimmune condition that causes chronic inflammation of the synovial lining of the joints, tendon sheaths and bursa. It is an inflammatory arthritis and symmetrical polyarthritis

17
Q

Genetic associations of RA

A
  • HLA DR4 - often present in RF positive patients
  • HLA DR1 - occasionally positive in RA patients
18
Q

Specific antibody for RA

19
Q

What is palindromic rheumatism

A
  • involves self-limiting short episodes of inflammatory arthritis with joint pain, stiffness and swelling typically only affecting a few joints
  • Episodes only last 1-2 days and then completely resolve
  • Having positive antibodies indicates that it will probably progress to RA
20
Q

Are DIP joints affected in RA

21
Q

RA signs in the hands

A
  • Z shaped deformity to the thumb
  • Swan neck deformity (hyperextended PIP with flexed DIP)
  • Boutonnieres deformity (hyperextended DIP with flexed PIP)
  • Ulnar deviation of the fingers at the knuckle (MCP joints
22
Q

Investigations for RA

A
  • Z shaped deformity to the thumb
  • Swan neck deformity (hyperextended PIP with flexed DIP)
  • Boutonnieres deformity (hyperextended DIP with flexed PIP)
  • Ulnar deviation of the fingers at the knuckle (MCP joints
23
Q

Xray changes in RA

A
  • Joint destruction and deformity
  • Soft tissue swelling
  • Periarticular osteopenia
  • Boney erosions
24
Q

When do you refer for RA

A
  • Recommended for any adult with persistent synovitis even if negative RF, anti-ccp, and inflammatory markers
  • Should be urgent referral if involves small joints of hands or feet , multiple joints or symptoms have been present for over 3 months
25
How to diagnose RA
- Scored based on 1. joints involved (more and smaller joints score higher) 2. Serology (RF and anti-ccp) 3. Inflammatory markers (ESR and CRP) 4. Duration of symptoms (more or less than 6 weeks) - Scores are added up and greater than 6 = diagnosis of RA
26
DAS28 score
disease activity score for RA - Based on assessment of 28 joints - Swollen joints - tender joints - ESR/CRP result - Useful in monitoring disease activity and response to treatment
27
Worse prognostic factors for RA
- Younger onset - Male - More joints and organs affected - Presence of RF and anti-ccp - Erosions seen on X-rays
28
Management of RA
- Short course of steroids can be given at first presentation and during flare -ups to settle disease - NSAIDs/COX 2 inhibitors are often effective and given in conjunction with PPI - DMARDs - First line - mono therapy - ***methotrexate***, ***leflunomide*** or ***sulfasalazine*** - Hydroxychloroquine - can be given in mild disease - Second line - two of the above in combination - Third line - methotrexate PLUS biologic therapy (such as TNF inhibitor) - Fourth line - methotrexate PLUS Rituximab - Biological therapies - Anti-TNF (adalimumab, infliximab, etanercept, golimumab and certolizumab pegol) - Anti-CD20 (rituximab) - Anti-IL6 (sarilumab) - Anti-IL6 receptor (tocilizumab) - JAK inhibitors (tofacitinib and baricitinib)
29
What is methotrexate
- Works by interfering with the metabolism of folate and suppressing certain aspects of the immune system - Taken by injection or tablet once weekly - Folic acid 5mg is also prescribed to be taken once weekly on a different day to methotrexate - Notable side effects include - Mouth ulcers - Liver toxicity - Bone marrow suppression and leukopenia - Teratogenic
30
What is taken alongside methotrexate
folic acid 5mg