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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

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25
90% of patients with ankylosing spondylitis have what gene?
HLA-B27
26
Ankylosing spondylitis is more common in which gender?
Males
27
There is a strong genetic risk to developing Ankylosing spondylitis and the disease severity - what genes are implicated?
HLA B27 (but unclear role) ERAP1 ARTS1
28
What is HLA-B27?
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
What is the role of antibodies to Klebsiella pneumoniae in Ankylosing spondylitis?
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
How does the pathophysiology of ank spond differ from RA?
Both involve inflammation and cartilage erosion but ankylosing spondylitis involves an additional process - repair or ossification
31
What is the Calin criteria for Inflammatory Back pain?
Age <40 Back pain > 3 months Insidious onset Improves with exercise Early morning stiffness
32
In addition to inflammatory back pain, what other disease associations/manifestations may be present in patients with Ank Spond?
Uveitis Iritis Enthesitis Psoriasis Inflammatory bowel disease Dyspnoea Fatigue Family history of spondyloarthropathy Sleep disturbance
33
What are some of the key physical examination findings in patients with ankylosing spondylitis?
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
What test is diagnostic of Ankylosing spondylitis?
None
35
What is the first test you should order in a patient suspected of having ankylosing spondylitis?
Pelvic XRAY
36
Does a negative pelvic X-RAY exclude Ankylosing spondylitis?
No! If suspicious need to obtain MRI + often helpful to check if HLA-B27 positive
37
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
16-fold increased risk
38
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
16-fold increased risk
39
What is the classic MRI pelvis finding in a patient with ankylosing spondylitis?
Bone marrow oedema on a T2-weighted sagittal short-tau inversion recovery (STIR) image
40
What are some distinguishing features between Ankylosing spondylitis and Inflammatory-bowel related arthritis?
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
What are some distinguishing features between Ankylosing spondylitis and psoriatic arthritis?
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
What are some distinguishing features between Ankylosing spondylitis and psoriatic arthritis?
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
What are some distinguishing features between Ankylosing spondylitis and psoriatic arthritis?
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
What are the known
45
What are the known
46
What are the known predictors of radiographic progression of Ankylosing spondylitis?
Syndesmophytes on initial X-RAY Elevated CRP/ESR Smoking
47
What are the known predictors of radiographic progression of Ankylosing spondylitis?
Syndesmophytes on initial X-RAY Elevated CRP/ESR Smoking
48
What non-pharmacological measures are appropriate for Ankylosing spondylitis?
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
What is the first line therapy for pain and stiffness in Ankylosing spondylitis?
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
What is the role of steroids in Ankylosing spondylitis?
No role for systemic steroids Can consider intra-articulate or local-site specific steroid injections as required
51
What therapies are indicated for peripheral joint involvement in Ankylosing spondylitis?
Sulfasalazine or methotrexate - note these do not treat axial disease and evidence is weak
52
For Ankylosing spondylitis patients with axial disease refractory to NSAIDs and physiotherapy +/- steroid injections and sulfasalazine what treatment is indicated?
TNF-alpha inhibitors Eg Adalimumab, certolizumab pegol, etanercept, golimumab, and infliximab
53
Should you continue NSAIDs in patients with ankylosing spondylitis commenced on TNF-alpha inhibitors?
Yes - recommended to continue continuous NSAIDs whilst active disease / established on TNF-alpha inhibitor then can reduce to PRN use
54
Should you attempt to wean TNF-alpha inhibitor therapy for Ankylosing spondylitis once patients achieve remission/disease control?
You can but high risk of disease flare - individual decision
55
What do you give a patient with ankylosing spondylitis who relapses after initial remission on TNF-alpha inhibitor therapy?
Try another TNF-alpha inhibitor before trying an alternative bDMARD therapy
56
What do you give a patient with ankylosing spondylitis who relapses after initial remission on TNF-alpha inhibitor therapy?
Try another TNF-alpha inhibitor before trying an alternative bDMARD therapy
57
If TNF-alpha inhibitor fails in Ankylosing spondylitis, what should you give?
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
After TNF-alpha inhibitors and IL-17 inhibitors, what is the third line bDMARD therapy for refractory Ankylosing spondylitis?
JAK inhibitors eg tofacitinib, upadacitinib)
59
After TNF-alpha inhibitors and IL-17 inhibitors, what is the third line bDMARD therapy for refractory Ankylosing spondylitis?
JAK inhibitors eg tofacitinib, upadacitinib)
60
What is the mechanism of action of Hydroxychloroquine?
It is an antimalarial drug with anti-inflammatory (and possibly immunosuppressive effects) Also appears to: - have lipid lowering properties - improves glycaemic control - have antithrombotic/anticoagulant affects by inhibiting platelet aggregation and adhesion (but not increasing bleeding time/risk) Anti-inflammatory effect: - blocks constimulation of B cell antigen receptor and Toll-like receptor (TLR)-9 pathways + inhibits TLR signalling - interferes with normal function of subcellular compartments that depend on an acidic milieu as it is a weak base - so called "lysosomotrophic action" - this may interfere with various processes incl secretion of cytokines/inflammatory mediators
61
Which patients with SLE should receive hydroxychloroquine?
ALL patients unless contraindicated
62
What is the typical/initial dose of hydroxychloroquine in SLE?
5mg/kg up to max 400mg daily
63
What are the 2 most significant side effects of hydroxycholoroquine therapy?
Vision-threatening toxic retinopathy QTc prolongation
64
What monitoring is required when on hydroxychloroquine therapy?
Baseline ocular assessment/opthal review then annual opthalmology review starting after 5 years of therapy
65
What are the risk factors for vision threatening toxic retinopathy secondary to hydroxychloroquine (HCQ)?
Daily HCQ dose >5mg/kg Long duration HCQ therapy > 5 years CKD3+ Tamoxifen use Female gender Macular disease
66
What are the benefits of hydroxychloroquine therapy in SLE?
Reduces mortality Reduces risk of disease flare Reduces fatigue Reduces skin and musculoskeletal symptoms Reduces thrombotic events Reduces end-organ damage accrual Reduces cancer
67
What must you do if a patient on HCQ therapy for SLE presents with vision impairment?
Stop HCQ and obtain opthal review
68
What are the symptoms/signs of retinopathy secondary to HCQ therapy?
Early - asymptomatic; only detected by spectral domain optical coherence tomography Later - symptoms incl dropout of letters when reading, photophobia, blurred distance vision, reduced night vision, visual field defects, flashing lights; o/e - central patchy area of depigmentation of macula surrounded by concentric ring of pigmentation ("bull's eye")
69
Do you need to adjust HCQ dosing in renal impairment?
Renally cleared and tend to reduce dose to 200mg (ie half dose) in ESKD Note HCQ decreases creatinine clearance by ~10% by competitively inhibiting creatinine secretion (not a true change in kidney function)
70
Is it safe to take hydroxychloroquine in pregnancy?
Yes
71
What are some less significant side effects of HCQ therapy?
Nausea and vomiting Pruritic maculopapular rash/skin reaction Headaches Myopathy
72
What rheumatological drugs are considered relatively safe to take in pregnancy?
Hydroxychloroquine Sulfasalazine Low dose aspirin Azathiopurine 6-mercaptopurine Colchicine

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