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BPT > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

What are the main considerations re treatment of dermatomyositis?

A

acute vs chronic
skin disease vs combined skin and muscle disease

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2
Q

What is the ongoing/chronic treatment for dermatomyositis with skin and muscle disease?

A

1) high dose oral steroids eg prednisolone 0.75-1.5mg/kg/d for 2-4 weeks then taper (use lowest possible dose)
- steroids reduce morbidity, improve muscle strength and motor function

2) Photoprotection - high protection sunscreen

3) topical steroids - even if treated with systemic corticosteroids
- often help control erythema and pruritis
- otherwise consider topical antipruritics and oral antihistamines OR topical tacrolimus

4) simple moisturisers and emollients

IF underlying malignancy - treat underlying malignancy - improvement or complete resolution of DM has been reported in some cases after successful treatment of underlying malignancy

Consider
Antimalarials eg hydroxycholoroquine

2nd line =
Methotrexate (both muscle and skin)
- steroid-sparing
- more rapid onset of effect than azathioprine

Azathioprine (more muscle disease than skin)
- takes longer than MTX to have effect eg >6mths

3rd line =
Mycophenolate

Ciclosporin - skin and early ILD

4th line =
IVIG

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3
Q

How do you treat patients with dermatomyositis with skin manifestations unresponsive to topical treatments?

A

Add Hydroxychloroquine

2nd
Methotrexate
Azathioprine

3rd
Mycophenolate
Ciclosporine

recalcitrant disease
IVIG, dapsone, thalidomide

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4
Q

How do you treat acute dermatomyositis?

A

If severe muscle weakness (e.g., quadriplegia), interstitial lung disease, myocarditis, respiratory failure, severe dysphagia, or other life-threatening complications.
- IV methylpred1g daily for 3-5 days then PO steroids

+

IVIG

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5
Q

What features may predict treatment-resistance to steroid therapy for dermatomyositis and necessitate early adjunctive treatment?

A

Anti-signal recognition particle (SRP) antibodies in patients with DM are associated with acute severe, treatment-resistant necrotising myositis - IVIG is often required at an early disease stage in these patients; alternative = Rituximab

Patients diagnosed with anti-synthetase syndrome may be corticosteroid-resistant and concomitant use of immunosuppressive drugs (e.g. methotrexate, azathioprine) is required

Presence of anti-melanoma differentiation-associated gene 5 (MDA5) is specific to clinically amyopathic DM, and is strongly associated with rapidly progressive interstitial lung disease. Patients often require treatment with immunosuppressive drugs as well as high-dose systemic corticosteroids from an early disease stage

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6
Q

What are the specific risks of IVIG in dermatomyositis treatment?

A

acute renal failure
skin rashes
aseptic meningitis
stroke

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7
Q

What is the most sensitive muscle enzyme test for the diagnosis of dermatomyositis?

A

CK

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8
Q

Other than CK, what other serum markers can elevated in dermatomyositis (incl if CK is normal)?

A

Serum aldolase
ANA - not specific
Myositis specific antibodies (MSAs) and myositis associated antibodies (MAAs)
- Anti-Mi-2 antibodies
- Anti‐U1 ribonucleoprotein (RNP)
- anti‐Ku
- Anti-Jo-1 and other anti-aminoacyl-tRNA synthetase antibodies (anti-ARS antibodies; including anti‐PL‐7, anti‐PL‐12, anti‐EJ, anti‐OJ, and anti‐KS

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9
Q

What is the significance of finding Anti-transcription intermediary factor 1 gamma in a patient with dermatomyositis?

A

Strongly assoc with malignancy therefore need extensive search for underlying malignancy

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10
Q

What is the significance and association of Anti-signal recognition particle (SRP) antibodies in dermatomyositis?

A

Found almost exclusively in polymyositis and assoc with acute severe, treatment-resistant necrotising myositis - need early IVIG +/- Rituximab

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11
Q

What are the key clinical features and antibodies assoc with anti-synthetase syndrome?

A

ILD, fever, myostitis, polyarthritis, mechanic’s hands, raynaud’s phenomenon

Anti-Jo-1 and other anti-aminoacyl-tRNA synthetase antibodies (anti-ARS antibodies; including anti‐PL‐7, anti‐PL‐12, anti‐EJ, anti‐OJ, and anti‐KS

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12
Q

What is Anti-melanoma differentiation-associated gene 5 (MDA5; also known as CADM‐140) specific for in dermatomyositis?

A

Clinically amyopathic dermatomyositis (CADM)

It is strongly associated with a rapidly progressive ILD with poor prognosis (need early immunosuppressive tx)

Tender palm papules and diffuse alopecia may be present

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13
Q

Anti-NXP2 is strongly associated with what form of dermatomyositis?

A

Juvenile DM with a high proportion suffering from cutaneous calcinosis due to dystrophic calcification

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14
Q

Anti-PM-Scl antibodies are often found in patients with?

A

Overlapping myositis and scleroderma

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15
Q

Describe the ideal muscle biopsy in patients with suspected dermatomyositis?

A

In symptomatic patients the biopsy should be taken from a weak but not severely atrophied muscle. Quadriceps and deltoid are common sites

Greater yield with open surgical biopsy cf needle biopsy

Greater yield if pre-biopsy EMG or MRI to target biopsy (given possible patchy muscle involvement)

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16
Q

What pattern of atrophy on muscle biopsy is diagnostic of dermatomyositis?

A

Perifascicular atrophy is seen due to phagocytosis and necrosis of muscle fibres

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17
Q

How are patients with dermatomyositis commonly classified according to severity?

A

Severe disease - those with severe muscle weakness (e.g., quadriplegia), interstitial lung disease, myocarditis, respiratory failure, severe dysphagia, or other life-threatening complications

Non-severe disease - 4/5 muscle power + none of life threatening complications

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18
Q

How do you diagnose dermatomyositis?

A

By using the European League Against Rheumatism and American College of Rheumatology (EULAR/ACR) classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIMs)

  • based on scoring system results in definite’, ‘probable’, and ‘possible’ IIM
  • patients can be further subclassified based on classification tree
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19
Q

What is the mode of inheritance of Marfan syndrome?

A

Autosomal dominant

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20
Q

What is the most typical genetic abnormality in Marfan Syndrome?

A

FBN1 - encodes the connective tissue protein fibrillin-1

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21
Q

What is the most typical genetic abnormality in Marfan Syndrome?

A

FBN1 - encodes the connective tissue protein fibrillin-1

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22
Q

What is the clinical hallmark of ankylosing spondylitis?

A

Inflammatory back pain

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23
Q

What is the clinical hallmark of ankylosing spondylitis?

A

Inflammatory back pain

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24
Q

90% of patients with ankylosing spondylitis have what gene?

A

HLA-B27

25
Q

90% of patients with ankylosing spondylitis have what gene?

A

HLA-B27

26
Q

Ankylosing spondylitis is more common in which gender?

A

Males

27
Q

There is a strong genetic risk to developing Ankylosing spondylitis and the disease severity - what genes are implicated?

A

HLA B27 (but unclear role)
ERAP1
ARTS1

28
Q

What is HLA-B27?

A

A heterotrimeric complex with beta-2-microglobulin that presents intracellular pathogens for recognition by the T-cell receptor of CD8 T cells. HLA-B27 can also be expressed as cell surface beta2-microglobulin (beta2m)-free homodimers (B27[2])

29
Q

What is the role of antibodies to Klebsiella pneumoniae in Ankylosing spondylitis?

A

Unclear - Antibodies directed against an epitope of a Klebsiella pneumoniae-derived protein are present in the majority of patients with AS but studies have yet to elucidate role in pathogenesis of AS

30
Q

How does the pathophysiology of ank spond differ from RA?

A

Both involve inflammation and cartilage erosion but ankylosing spondylitis involves an additional process - repair or ossification

31
Q

What is the Calin criteria for Inflammatory Back pain?

A

Age <40
Back pain > 3 months
Insidious onset
Improves with exercise
Early morning stiffness

32
Q

In addition to inflammatory back pain, what other disease associations/manifestations may be present in patients with Ank Spond?

A

Uveitis
Iritis
Enthesitis
Psoriasis
Inflammatory bowel disease
Dyspnoea
Fatigue
Family history of spondyloarthropathy
Sleep disturbance

33
Q

What are some of the key physical examination findings in patients with ankylosing spondylitis?

A

Loss of lumbar lordosis and flexion
Tenderness at sacroiliac joints
Kyphosis
Peripheral joint involvement

Tragus-to-wall distance
Lumbar flexion
Cervical rotation
Lumbar side flexion
Intermalleolar distance
- these measurements taken together to form Bath Ankylosing Spondylitis Metrology Index

34
Q

What test is diagnostic of Ankylosing spondylitis?

A

None

35
Q

What is the first test you should order in a patient suspected of having ankylosing spondylitis?

A

Pelvic XRAY

36
Q

Does a negative pelvic X-RAY exclude Ankylosing spondylitis?

A

No!
If suspicious need to obtain MRI + often helpful to check if HLA-B27 positive

37
Q

What is the risk of developing AS among HLA-B27-positive first-degree relatives of HLA-B27-positive probands with AS, compared with HLA-B27-positive individuals in the population

A

16-fold increased risk

38
Q

What is the risk of developing AS among HLA-B27-positive first-degree relatives of HLA-B27-positive probands with AS, compared with HLA-B27-positive individuals in the population

A

16-fold increased risk

39
Q

What is the classic MRI pelvis finding in a patient with ankylosing spondylitis?

A

Bone marrow oedema on a T2-weighted sagittal short-tau inversion recovery (STIR) image

40
Q

What are some distinguishing features between Ankylosing spondylitis and Inflammatory-bowel related arthritis?

A

IBD-related arthritis tend to:
Have a hx of UC or Crohns
Have no sex bias (males and females equally affected)
Less likely to be HLA-B27 positive (only 30%)
More likely to have peripheral joint involvement
May have asymmetric sacroillitis
May have pyoderma gangrenosum or erythema nodosum

41
Q

What are some distinguishing features between Ankylosing spondylitis and psoriatic arthritis?

A

Patients with psoriatic arthritis tend to:
Develop disease at slightly older age (30-40s as opposed to 20s)
Have asymmetric/unilateral sacroillitis
Have a history of psoriasis
Have dactylitis
Have erosive disease on X-ray

42
Q

What are some distinguishing features between Ankylosing spondylitis and psoriatic arthritis?

A

Patients with psoriatic arthritis tend to:
Develop disease at slightly older age (30-40s as opposed to 20s)
Have asymmetric/unilateral sacroillitis
Have a history of psoriasis
Have dactylitis
Have erosive disease on X-ray

43
Q

What are some distinguishing features between Ankylosing spondylitis and psoriatic arthritis?

A

Patients with psoriatic arthritis tend to:
Develop disease at slightly older age (30-40s as opposed to 20s)
Have asymmetric/unilateral sacroillitis
Have a history of psoriasis
Have dactylitis
Have erosive disease on X-ray

44
Q

What are the known

A
45
Q

What are the known

A
46
Q

What are the known predictors of radiographic progression of Ankylosing spondylitis?

A

Syndesmophytes on initial X-RAY
Elevated CRP/ESR
Smoking

47
Q

What are the known predictors of radiographic progression of Ankylosing spondylitis?

A

Syndesmophytes on initial X-RAY
Elevated CRP/ESR
Smoking

48
Q

What non-pharmacological measures are appropriate for Ankylosing spondylitis?

A

1) Smoking cessation
2) active physio therapy
3) patient education
4) cardiovascular disease risk calculation and modification
- as px with AS have increased CVD related mortality

49
Q

What is the first line therapy for pain and stiffness in Ankylosing spondylitis?

A

NSAIDs
- conflicting evidence whether reduced disease progression but helpful for symptoms
- should trial at least 2 at highest tolerated dose before moving on to other treatments

50
Q

What is the role of steroids in Ankylosing spondylitis?

A

No role for systemic steroids
Can consider intra-articulate or local-site specific steroid injections as required

51
Q

What therapies are indicated for peripheral joint involvement in Ankylosing spondylitis?

A

Sulfasalazine or methotrexate
- note these do not treat axial disease and evidence is weak

52
Q

For Ankylosing spondylitis patients with axial disease refractory to NSAIDs and physiotherapy +/- steroid injections and sulfasalazine what treatment is indicated?

A

TNF-alpha inhibitors
Eg Adalimumab, certolizumab pegol, etanercept, golimumab, and infliximab

53
Q

Should you continue NSAIDs in patients with ankylosing spondylitis commenced on TNF-alpha inhibitors?

A

Yes - recommended to continue continuous NSAIDs whilst active disease / established on TNF-alpha inhibitor then can reduce to PRN use

54
Q

Should you attempt to wean TNF-alpha inhibitor therapy for Ankylosing spondylitis once patients achieve remission/disease control?

A

You can but high risk of disease flare - individual decision

55
Q

What do you give a patient with ankylosing spondylitis who relapses after initial remission on TNF-alpha inhibitor therapy?

A

Try another TNF-alpha inhibitor before trying an alternative bDMARD therapy

56
Q

What do you give a patient with ankylosing spondylitis who relapses after initial remission on TNF-alpha inhibitor therapy?

A

Try another TNF-alpha inhibitor before trying an alternative bDMARD therapy

57
Q

If TNF-alpha inhibitor fails in Ankylosing spondylitis, what should you give?

A

1) can try another TNF-alpha inhibitor
OR
2) IL-17 inhibitor (eg Secukinumab, Ixekizumab)
- these are more effective in TNF-alpha naive patients and in patients with psoriasis

58
Q

After TNF-alpha inhibitors and IL-17 inhibitors, what is the third line bDMARD therapy for refractory Ankylosing spondylitis?

A

JAK inhibitors eg tofacitinib, upadacitinib)

59
Q

After TNF-alpha inhibitors and IL-17 inhibitors, what is the third line bDMARD therapy for refractory Ankylosing spondylitis?

A

JAK inhibitors eg tofacitinib, upadacitinib)

BPT (11 decks)

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