SCE > ILD - CTD > Flashcards

ILD - CTD Flashcards

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1
Q

Prevalence of ILD in RA

A

5% symptomatic disease

but found in up to 60%

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2
Q

Which RA patients get ILD

A

Multi system disease
Nodules
Seropositive

Men
smokers

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3
Q

What pattern of ILD do you get in RA

A

UIP most commonly

can also get NSIP

can also get acute pneumonitis which is more steroid response

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4
Q

Rx RA ILD

A

Steroids/immunosuppressants

Nintedanib

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5
Q

Diagnostic criteria SLE

A

Malar rash
Discoid eczema
Photosensitivity
Oral ulcers
Non erosive gastritis
Pleuritis/pericarditis

Renal disorder
Neuro disorder (seizure, psychosis)
Haem (haemolytic anaemia, leucopaenia, lymphopaenia, thrombocytopaenia)

Anti Ds-DNA, Anti Sm, APS
ANA

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6
Q

What % SLE develop significant ILD

A

5%

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7
Q

What pattern is SLE CTD

A

Usually NSIP

Rarely progressive/severe

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8
Q

What is acute lupus pneumonitis

A

<2%

mortality >50%

tx with steroids and cytotoxics

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9
Q

Which Idiopathic Inflammatory Myopathies cause ILD

A

Dermatomyositis
Polymyositis
Anti-synthetase syndrome

ILD can occur before myositis

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10
Q

Diagnostic criteria Idiopathic Inflammatory Myopathies

A

Muscle weakness
Symmetrical proximal muscle weakness
Skin changes
Heliotropic rash
Gottron papules
Dysphagia/esophageal dysmotility
Elevated muscle enzymes CK, LDH, AST, ALT
Typical auto antibody findings
Typical muscle biopsy findings

MRI commonly used

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11
Q

What % of inflammatory myopathies get ILD

A

20-30%

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12
Q

Which inflammatory myopathy and which antibodies more commonly get ILD

A

Anti synthetase

Anti MDA5
Anti-PML-SCL

less common if malignancy related

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13
Q

HRCT findings in inflammatory myopathy ILD

A

Patchy consolidation
reticular pattern

UIP/NSIP/COP

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14
Q

Rx inflammatory myopathy ILD

A

Steroids
cyclophosphamide
rituximab

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15
Q

Complications inflammatory myopathy ILD

A

Organising pneumonia
Pulmonary vasculitis
Aspiration pneumonia
Ventilatory failure
Spontaneous pneumomediastinum

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16
Q

Features anti synthetase syndrome

A

ILD

Polyarthritis
Fever
Raynauds
Mechanics hands
Dysphagia
Pericarditis

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17
Q

Which are the antibodies for antisynthetase syndrome

A

aminoacyl-transfer RNA synthetases

Jo-1 (20-30%, most common)
PL-7
PL-12
OJ
EJ
KS

Myositis blot

Anti-MDA5 ax with rapidly progressive ILD

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18
Q

Which anti synthetase antibodies are ax with PHTN

A

Jo
PL7
PL12

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19
Q

Rx anti synthetase

A

1st line:
MMF + steroid

2nd:
cyclophosphamide

3rd:
rituximab

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20
Q

Who gets systemic sclerosis

A

F:M 4:!
50s

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21
Q

Features limited cutaneous sclerosis/CREST

A

Raynauds
Thick shiny skin
Face/hand telangectasia
Calcinosis
Oesophageal dysmotility

Oesophageal
Pulmonary fibrosis 25%
PHTN
Cardiac

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22
Q

Features Diffuse Cutaneous Systemic Sclerosis

A

Abrupt onset

Widespread itchy painful swellings limbs and face
Constitutional sx

Raynauds

Skin thickening trunk and arms

Pulmonary fibrosis 41%

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23
Q

Most common cause of death systemic sclerosis

A

Pulmonary complications

inc PHTN, fibrosis, chest wall limitations, aspiration, bronchiectasis

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24
Q

Antibodies systemic sclerosis

A

ANA +ve 60%
Scl-70 +ve commonly get lung involvement

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25
HRCT pattern systemic sclerosis
Usually NSIP Can be UIP
26
Tx Systemic sclerosis
Steroids Cyclophosphomide MMF Nintedanib
27
What features of Limited Cutaneous Systemic Sclerosis are particularly linked to PHTN
Cutaneous telangiectasia Anti centromere
28
Pathophysiology Sjorgens syndrome
Inflammation and lymphocytic infiltration of lacrimal and salivary glands causing keratoconjunctivitis sicca, xerostomia
29
Classical features sicca syndrome (2)
Dry eyes and mouth Parotid and salivary enlargement
30
What % sicca syndromes get lung involvement
25%
31
Lung manifestations of sicca syndromes
Pleuritic chest psin Airways disease Dry cough Diffuse lung disease (NSIP, LIP, UIP) Lymphoma Pleural thickening/effusion Organising pneumonia
32
What group get ank spond
90% causcasians
33
Gene for ank spond
HLA-B27
34
What % Ank Spond get pulmonary fibrosis
5-15%
35
What type of pulmonary fibrosis do ank spond get
Bilateral Upper lobes Can get cysts/cavities which can get colonised with aspergillus
36
What % SLE are ANA +ve
99%
37
What % chronic active hepatitis are ANA +Ve
75%
38
What % Sjorgens are ANA +Ve
68%
39
What % RA are ANA +ve
32%
40
What are the antibodies for SLE (4)
ANA Anti Ds-DNA Anti-SM Anti-RNP
41
What are the antibodies for scleroderma (3)
Anti centromere Anti Scl-70 (particularly get lung fibrosis) Anti RNP
42
What the antibodies for Sjogrens
Anti-Ro
43
What's the antibodies for myositis
Anti-Jo
44
What is Anti-RNP ax with (5)
SLE Scleroderma Myositis RA Mixed
45
What's the antibody for GPA
PR3 cANCA
46
What's the antibody for microscopic polyangiitis
MPO pANCA
47
What % RA are RF +Ve
70-80%
48
What % Sjorgens are RF +ve
<100%
49
What % Feltys are RF +ve
<100%
50
What % Systemic Sclerosis are RF +ve
30%
51
What % endocarditis are RF +Ve
<50%
52
What % of SLE are RF +ve
<40%
53
Whats the 1st line treatment options for CTDs
MMF Azathioprine (or toci for MMF) Rituximab Additional options: - cyclophosphamide Steroids APART FROM SYSTEMIC SCLEROSIS
54
Which CTD do they strongly advise against steroids
Systemic sclerosis
55
Rx of rapidly progressive ILD
IV glucocorticoids + Rituximab Cyclophosphamide IVIG MMF Calcineurin JAKI Use 1 of addition therapies, or 2 if suspicion MDAS or high severity
56
What % get lung fibrosis before RA sx appear
10-20%
57
When do resp manifestations tend to occur in RA
Within 1st 5 years
58
Which pattern of ILD has worst outcomes in RA
UIP
59
Risk factors for developing ILD in RA
MUC5B gene (telomere surfactant gene) Smoking, silica Seropositive RA disease activity Male Age onset >55 Telomere length shortening
60
What % RA pt's have ILD
60% on imaging But on 10% clinically signficant
61
Survival time after RA-ILD diagnosis
3-7 years
62
Which Systemic Sclerosis pt's tend to get ILD
More common in diffuse (but can be either) Anti topoisomerase +ve (or any anitbody) Male african americans Nail changes/digital ulcers/PHTN
63
What % systemic sclerosis pt's get ILD
80% on scan but only 30-40% clinically significant Is leading cause of death
64
10 year Mortality SSc-ILD
40%
65
When to SSc pt's get ILD
Within 5 years of 1st non Raynauds presentation Rarely occurs after 15 years Presentation <3 years ax with aggressive clinical course
66
What are some of the features on the systemic sclerosis score
Main criteria is skin thickening both hands
67
What's the non ILD cause of restrictive spirometry in SSc
High bound chest ie extra thoracic restriction
68
What other HRCT features might suggest SSc
patulous eosophagus Chronic aspiration PHTN
69
Features ax with worse prognosis SSc
Older age at presentation Male Smoking
70
Ix negative predictive outcomes in SSc-ILD
FVC decrease >10% Fibrosis extent >20% DCLO decrease >15%
71
What pred dose should you be wary of in SSc as risk renal crisis
>15mg/day
72
Whats the criteria for no evidence of disease progression in SSc-ILD
FVC decline <5% or FVC 5-9% with DCLO decline <15%
73
What's the criteria for disease progression in SSc-ILD
FVC decline >10% or FVC decline 5-9% with DCLO decline >= 15%
74
What ILD screening should all SSc patients have (5)
Chest auscultation PFTs with DCLO HRCT Hx PHTN screening if sx not explained by ILD
75
Treatment criteria for SSc-ILD
- FVC <80% and any ILD/sx - >20% lung involvement HRCT - >10% involvement HRCT with PFTs - high risk (ie early cutaneous disease with mild ILD <10% - worsening HRCT or PFTs - exertional desat
76
Tx for SSc-ILD
MMF 2000-3000mg/day Consider adding nintedanib (or use alone if MMF CI)
77
When to use toci in SSc-ILD
Elevated acute phase reactants Can't tolerate other antifibrotics
78
How to monitor SSc-ILD if ILD present
PFTs 3-6 monthly if stable after 4-5 years, 6 monthly PFTs if not additional Ix eg HRCT
79
How to monitor SSc-ILD if ILD *NOT* present
PFTs annually if not stable additional Ix eg HRCT
80
When do IIM pt's tend to get ILD
Can happen any time, usually around 18 months
81
Are cancer ax myositis ax with increased ILD risk
No
82
Features ax with Anti-MDA5 IIM
Asian Amyopathic Dermatosis (CADM) Deep ulcers and skin necrosis Rapidly progressive ILD
83
What are anti-Pu and anti-Ku ax with
myositis overlap syndromes SSc and SLE
84
What is the other non ILD cause of worsening spiro in anti synthetase
Resp muscle involvement
85
How to screen for diaphragmatic weakness
Sitting and supine spiro MIP/MEP
86
Which more responsive to steroids dermatomyositis and polymyositis
PM Give steroids in acute or rapidly progressing disease
87
What's considered mild IIM-ILD
<10% CT involvement Preserved PFTs No O2 req
88
What's considered moderate IIM-ILD
Abnormal PFTs >10% CT involvement give steroids and if no response, then immunosuppression
89
5 years survival Sjorgens ILD
90%
90
What is mixed connective tissue disease
sx of Systemic sclerosis polymyositis SLE and Anti-RNP +ve
91
Lung conditions SLE
ILE PE DAD Shrinking lung syndrome Pulmonary haemorrhage Pneumonitis
92
How often does ILD occur in SLE
2-4% Duration of disease is risk factor
93
5 year survival SLE ILD
93%
94
What % of interstitial pneumonia with autoimmune features progress to ILD
10-20%
95
Difference between treating IPAF-UIP and IPAF-COP/NSIP
IPAF-UIP acts more like UIP so can treat more with antifibrotics IPAF-COPD/NSIP acts more like CTD-ILD so more steroid/immunotherapy responsive
96
Indications for starting pirfenidone
IPF with FVC between 50-80% predicted
97
Indications for stopping pirfenidone
FVC decline of 10%+ in 12 month period
98
Dosage pirfenidone
Titrated over 28 days to 801mg TDS max (2403mg daily dose) If medication missed for 14+ days, re-titrate
99
What medication should you avoid with pirfenidone
Cipro If really needed, half pirfenidone dose
100
SEs pirfenidone (5)
GI Upset - take with food and anti dyspepsia meds, can reduce dose or pause for 7 days Photosensitivity Hepatic impairment Tiredness Headache Often suggest reduce dose to address SEs
101
Counselling for photosensitivity pirfenidone
Factor 50 Avoid other drugs causing photosensitiity Reduce dose to 1 TDS If rash persisting more than 7 days, stop for 15 days and retitrate If severe reaction, stop
102
Contraindications to pirfenidone (4)
Hypersensitivty to pirfenidone/analgesia Fluvoxamine Severe heptic or renal disease CrCl <30 Smoking Caution of CYPA enzyme drugs
103
LFT monitoring in pirfenidone and nintedanib
every 3 months AST - 3-5x ULN -> contact ILD team <5 with sx and hyperbilirubinaemia -> discontinue - >5x ULN, discontinue
104
Indications for nintedanib
IPF with FVC <50% Can be used for other progressive non IPF ILDs as per INBUILD trial
105
Benefit of nintedanib
FVC decline reduced by 150ml/yr Improved survival
106
When to stop nintedanib
FVC decline 10%+ in 12 months
107
INBULID Criteria
Decline in FVC 10%+ in past 24/12 compared to pre screening Decline in FVC 5-10% w worsening sx/fibrotic changes CT in past 24/12 compared to pre screening Worsening resp sx or increasing fibrotic change past 24/12
108
Dosing nintedanib
150mg BD Reduce to 100mg BD if higher dose not tolerated If missed, start at next available dose
109
SEs nintedanib
Diarrhoea 62% High liver enzymes Abdo pain Bleeding GI perf Cautions: liver impairment anticoag
110
Contraindications to nintedanib
Sensitivity peanuts or soya Hepatic impairment Child Pugh B or C MI past 6 months or unstable angina past month Increased bleeding risk Thrombotic event past 12/12 or inherited predisposition Pregnancy/breastfeeding
111
Interactions nintedanib
Nintedanib is susbtrate of P glycoprotein Inhibitor P-gp can increase levels - ciclosporin - erythromycin -ketoconazole Induces can reduce levels - St Johns wort - rifampicin - carbemazepine - phenytoin
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