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BPT Rheumatology > Systemic Sclerosis and Inflammatory Myopathies > Flashcards

Systemic Sclerosis and Inflammatory Myopathies Flashcards

1
Q

What are the two main subsets of scleroderma?

A

limited cutaneous and diffuse cutaneous

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2
Q

What genes are involved in scleroderma?

A

HLA-DRB1, STAT4, interferon

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3
Q

What environmental factors are associated with scleroderma?

A

silica, organic solvents, vinyl chloride

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4
Q

What are the three pathologic pillars of systemic sclerosis?

A

inflammation (synovitis, myositis), vasculopathy (raynaud’s, abnormal capillaries, ischaemic changes) and interstitial fibrosis (in skin = scleroderma, but also happens in other organs e.g. lung, gut, renal)

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5
Q

What is the difference between diffuse cutaneous and limited cutaenous?

A

diffuse has a more rapid onset, has earlier internal organ involvement, more constitutional symptoms
in limited cutaneous skin changes are limited to arms and face

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6
Q

Which antibodies are associated with diffuse cutaneous scleroderma?

A

Scl-70 antibodies, anti-RNA polymerase I, III, ANA with nucleolar pattern

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7
Q

What is CREST syndrome?

A

limited cutaneous scleroderma: Calcinosis, Raynaud’s, oEsophageal dysmotility, Skin changes, Telangiectasia

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8
Q

What antibodies are associated with limited cutaneous scleroderma?

A

anti-centromere antibodies

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9
Q

Is pulmonary arterial hypertension more common in diffuse or limited?

A

equal 10% in both

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10
Q

Is ILD more common in diffuse or limited?

A

diffuse

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11
Q

Is scleroderma renal crisis more common in diffuse or limited?

A

diffuse

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12
Q

What organ involvement is associated with anti Scl-70 antibody?

A

intestitial lung disease

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13
Q

What organ involvement is associated with centromere antibodies?

A

protection from ILD and renal disease

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14
Q

What organ involvement is associated with anti RNA polymerase III antibodies?

A

renal, skin, malignancy

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15
Q

What is the leading cause of death in scleroderma?

A

cardiopulmonary manifestations - pulmonary fibrosis, pulmonary hypertension

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16
Q

What is the treatment for scleroderma?

A

no overall disease modifying therapy - organ specific treatment

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17
Q

How do you diagnose interstitial lung disease?

A

high resolution CT shows non specific interstitial pneumonitis

18
Q

Which patients with ILD should be treated?

A

patients with early diffuse scleroderma with positive anti-Scl70 or elevated CRP, or based on the grade of disease on HRCT, FVC% or DLCO % less than lower limit of normal or clinically meaningful decline, desaturating on 6MWT, symptoms from ILD

19
Q

How often should patients get spirometry?

A

for every 3-4 months for the first 5 years and then yearly

20
Q

What conservative measures can be used to treat ILD?

A

treat GORD, quit smoking, vaccinations, supplemental O2

21
Q

What is first line therapy for ILD?

A

mycophenolate 3g/day

22
Q

What are other reasons patients with scleroderma may have declining DLCO other than ILD?

A

neuromusclar dysfunction (myositis), thoracic restriction (scleroderma), aspiriation (GORD), deconditioning, pulmonary hypertension

23
Q

How do you diagnose pulmonary hypertension?

A

with right heart catheter

24
Q

What screening should be done for scleroderma patients for PAH?

A

TTE and RFTs OR pro-BNP and RFTs

25
Q

What is the therapy for scleroderma associated PAH?

A

endothelial receptor antagonist or PDE-5 inhibitor or riociguat

26
Q

What are the features of scleroderma renal crisis?

A

abrupt onset of moderate to severe hypertension, normal urine sediment, progressive renal failure

27
Q

How common is scleroderma renal crisis in diffuse scleroderma?

A

10-20%

28
Q

What is a trigger for scleroderma renal crisis?

A

corticosteroids

29
Q

What is the treatment for scleroderma renal crisis?

A

effective and prompt blood pressure control with an ACE inhibitor

30
Q

What is the treatment for raynaud’s phenomenon?

A

dihydropyridine calcium channel blockers, PDE5 inhibitors, topical nitrates

31
Q

What are the different types of gut involvement in scleroderma?

A

rigidity of facial muscles/tongue, reduced saliva production, incoordination of swallowing, GORD, bloating, distension, bacterial overgorwth, intussucception, pneumatosis intestinalis, faecal incontinence

32
Q

What are the 4 classes of idiopathic inflammatory myopathies?

A

polymyositis
dermatomyositis
inclusion body myositis
immune mediated necrotising myopathy

33
Q

What are the clinical features of dermatomyositis and polymyositis?

A

symmetrical proximal muscle weakness

34
Q

What are the features of a dermatomyositis rash?

A

gottron’s papules (on extensor surface MCP and PIP joints), shawl sign, heliotrope rash (around eyes), periungal erythema

35
Q

What are the laboratory findings for DM and PM?

A

elevated muscle enzymes (CK, transaminases, LDH, aldolase), antinuclear antibodies

36
Q

What non laboratory investigations are useful in DM and PM?

A

EMG and MRI

37
Q

What screening should be done for malignancy in patients with inflammatory myopathies?

A

medical history, physical examination of breast, rectal and pelvic examination, age appropriate screening (mammography, colonoscopy)

38
Q

Which malignancies are most commonly associated with dermatomyositis?

A

cervix, lung, ovaries, pancreas, bladder, stomach

39
Q

What investigations should be done for non malignant complications of inflammatory polymyopathies?

A

cardiac (TTE and ECG)
pulmonary (HRCT, RFTs)
oesophageal (oesophageal motility studies)

40
Q

What is the treatment for inflammatory polymyopathies?

A

high dose glucocorticoids (taper over a year), steroid sparing agents (azathioprine, methotrexate, IVIG, rituximab, plasma exchange)

41
Q

What are the clinical features of inclusion body myositis?

A

insidious onset of assymetric, proximal leg weakness for up to 5 years
weakness of distal finger flexor muscles
muscle atrophy
dysphagia

BPT Rheumatology (8 decks)

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