ILD Flashcards

1
Q

What will you find in BAL for Sarcoid?

A

CD4+/CD8+ ratio of more than 4

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2
Q

What is stage 2 sarcoid ?

A

LN and lung involvement

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3
Q

What is the median survival for people with IPF in the UK?

A

3 years from diagnosis (though is variable as 20% survives more than 5 years)

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4
Q

What are the clinical features of IPF?

A

Gradual onset exertional breathlessness and cough (often 9/12 prior to presentation)

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5
Q

What is estimated prevalence in the UK of IPF?

A

30,000 in UK

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6
Q

What is the purported cause of IPF?

A

Repeated alveolar epithelial injury leading to aberrant wound healing. Triggers include inhalation of metal/wood/GORD

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7
Q

What are the criteria for lung transplant in IPF?

A

TLCO <40%
FVC fall 10% over 6 months
TLCO fall 15% over 6 months
O2 desat to <88% on 6MWT

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8
Q

Should we ventilate patients with IPF?

A

Poor outcomes , do not routinely offer mechanical ventilation to patients with IPF who develop life threatening resp failure

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9
Q

What are the treatment guidelines for Pirfenidone in IPF?

A

FVC 50-80%

dose is 267mgTDS

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10
Q

What are the treatment guidelines for Nintedanib in IPF?

A

FVC > 50%

(Due to Inbuild trial can now give you FVC >80%, lower cut off still in place)

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11
Q

What are the main side effects of Pirfenidone?

A

Nausea
Photosensitive rash

(NB can cause Liver Function derangement)

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12
Q

What are the main side effects of Nintedanib

A

Diarrhoea
Weight loss
Incr bleeding risk

(NB can cause liver function derrangement)

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13
Q

How is IPF histologically characterised?

A

Temporal and special heterogeneity with areas of active fibroblastic foci (reflecting acute injury) and architectural distortion / honeycombing (reflecting chronic inflammation) interspersed with areas of normal lung. Reflecting different stages of evolution of disease process across the lung

IPF is seen as the archetypal ILD

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14
Q

What lung diseases associated with a UIP pattern

A

IPF
CTD-ILD
Chronic HP
Sarcoid
Nitrofurantoin ILD
Asbestosis

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15
Q

Who gets IPF?

A

Men
60-70 classically
Ex smokers often

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16
Q

What tests to do in IPF?

A

Bloods : ANA, ANCA, RhF, Anti CCP, Immunoglobulins, HIV, ?Avian precipitans ?Polymositis scleroderma panel (ie ruling out other causes). ESR and CRP often mildly raised , mild anaemia

NB can get positive RhF and / or ANA at low titres

Lung function
HRCT
?BAL
?Biopsy (rarely done)
TTE

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17
Q

What is Nintedanib

A

Tyrosine Kinase Inhibitor (TKI) which inhibits platelet derived growth factor (PDGFR) , fibroblast growth factor (FGFR) and vascular endothelial growth factor receptor (VEGFR)

Combined analysis of three nitedanib trials has shown that it improves all cause mortality by 30% and respiratory related mortality by 38%

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18
Q

What is Pirfenidone

A

Unclear exact mechanism but inhibits collagen synthesis and reduces fibroblast proliferation

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19
Q

What signifies a failure of treatment with anti fibrotic?

A

FVC decline >10% in 6 months / 1 year or TLCO decline >15% in 6 months/1 year

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20
Q

What are poor prognostic factors in IPF?

A

Low BMI
TLCO <40% at presentation
FVC fall > 10% in 6 months
TLCO fall > 15% in 6 months
Pulmonary hypertension
Fibroblastic foci on biopsy

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21
Q

What do patients either IPF usually die of ?

A

Respiratory failure and infection

NB increased risk of lung cancer

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22
Q

What is inpatient mortality for IPF exacerbation ?

A

60%

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23
Q

What is a UIP picture on scan?

A

Basal/subpleural preponderance of reticulation
Traction bronchiectasis
Honeycombing
Propellar blade

With ABSENCE of :
- XS ground glass
- Bronchocentricity
- Lots of interlobular septal thickening

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24
Q

What is honeycombing

A

Multilevel, walled , 3-10 mm size

Beware
Emphysema with any sort of infiltration surrounding it can look like honeycombing , traction bronchiectasis can etc

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25
Q

What are the ATS/ERS Classifications of UIP?

A

UIP Pattern
Probable UIP pattern
Indeterminate for UIP
CT findings suggestive of alternate diagnosis

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26
Q

CT findings demonstrate peribronchovascular predominant with subpleural sparing what should you consider ?

A

NSIP

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27
Q

CT findings demonstrate perilymphatic distribution what should you consider ?

A

Sarcoidosis

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28
Q

CT findings demonstrate upper or mid lung what should you consider ?

A

Fibrotic HP
CTD-ILD
Sarcoidosis

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29
Q

CT findings demonstrate subpleural sparing what should you consider ?

A

NSIP
Smoking related IP

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30
Q

CT findings demonstrate cysts what should you consider ?

A

LAN
PLCH
LIP
DIP

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31
Q

CT findings demonstrate mosaic attenuation or three density sign what should you consider ?

A

HP

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32
Q

CT findings demonstrate predominant GGO what should you consider ?

A

HP
Smoking related disease
Drug toxicity
Acute exacerbation of fibrosis

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33
Q

CT findings demonstrate profuse centrilobular micronodules what should you consider ?

A

HP
Smoking related disease

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34
Q

CT findings demonstrate nodules what should you consider ?

A

Sarcoidosis

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35
Q

CT findings demonstrate consolidation what should you consider ?

A

Organising pneumonia

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36
Q

CT findings demonstrate pleural plaques what should you consider ?

A

Asbestosis

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37
Q

CT findings demonstrate dilated oesophagus what should you consider ?

A

CTD-ILD

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38
Q

What is NSIP

A

Non Specific Interstitial Pneumonia is a histological pattern rather than a clinical entity.
Poorly understood probably encompasses several distinct clinical/radiological conditions , patients with NSIP on biopsy gave generally better prognosis and response to steroids compared with IPF

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39
Q

Who typically has NSIP

A

40-50yos
Idiopathic
CTD (NSIP may be first manifestation)
Drug related
Infection
Immunodeficiency (HIV, BMT, Chemo)

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40
Q

Describe HRCT features of NSIP

A

Groundglass change, often in a basal distribution with or without reticulation and traction bronchiectasis. Appearances usually more confluent and homogenous c/w patchy heterogenous distribution seen in IPF
Honeycombing usually absent but can occ see with fibrotic NSIP

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41
Q

What are the three types of NSIP on histology?

A

NSIP 1: Primarily inflammation , termed “cellular”
NSIP 2: Inflammation and fibrosis
NSIP 3 : Primarily fibrosis

Fx of NSIP and UIP sometimes seen on biopsies from the same individual - in such cases the diagnosis is considered to be IPF

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42
Q

How do you diagnose NSIP pattern ?

A

HRCT non specific and thus surgical biopsy often required for diagnosis - an exception is NSIP in the setting of CTD when histological confirmation is not required

Need to interpret Biopsy of NSIP along with context of clinical and radiological findings

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43
Q

How do you treat NSIP?

A

Steroids 0.5mg/kg/day
(Azathioprine / MMF useful if not responding to steroids alone)

(Cover with PPI, Bone Protection and Co-Trimoxazole prophylaxis against PCP)

Monitor with LFT

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44
Q

What is the prognosis in NSIP pattern

A

Variable, often stable on tx
Cellular pattern is associated with good prognosis , fibrotic NSIP is associated with markedly better prognosis than IPF (5year survival > 50% in fibrotic NSIP compared with 10-15% in IPF)

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45
Q

What are squeaks on auscultation?

A

Signs of active alveolitis

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46
Q

What is HSP?

A

Hypersensitivity pneumonitis is a group of lung diseases caused by inhalation of an organic antigens to which an individual has been previously sensitized

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47
Q

What do you see on histological samples for HSP?

A

Loose granulomas

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48
Q

What are the most common causes of HSP?

A

MOULD

Farmers lung — Thermophilic Actinomycete - mouldy hay, sugar cane, contaminated water

Pigeon fanciers lung —protein in the droppings

Mycobacterium avium —- hot tub lung

49
Q

Is there a questionnaire for HSP?

A

None standardised , can use UCSF

50
Q

How do we classify HSP?

A

Non fibrosing inflammatory HP

Fibrotic (Mixed inflammatory plus fibrotic or pure fibrotic)

51
Q

What is required to classify as non fibrotic inflammatory HSP?

A

One HRCT fx of parenchymal infiltration:
- Ground glass
- Mosaic attenuation

One HRCT fx of small airways disease:
- Ill defined centrilobular nodules all lobes
- Air trapping

Distribution:
- Axial
- Craniocaudal

20% will have a normal CXR

52
Q

What is required to classify a fibrotic (mixed inflammatory plus fibrotic or pure fibrotic) HSP?

A

One HRCT pattern of fibrosis:
- Reticulation
- Traction Bronchiectasis
- Honeycombing

One HRCT finding of small airways disease
- Ill defined centrilobular nodules
- Mosaic attenuation
- 3 density (nodules, GG, air trapping in same lobe)

Distribution:
- Axial
- Craniocaudal
- Mid lung
- Sparing bases

53
Q

What would you expect to see on BAL to support HSP dx ?

A

Lymphocytes > 30%; > 50% is strongly suggestive

NB in smokers probably above 20% is significant

If fibrotic HSP will likely be a normal BAL

54
Q

What conditions give you a lympocytosis on BAL?

A

HSP
CTD- ILD
Sarcoid
Infection
Drug Induced ILD
COP
NSIP
Chronic beryllium

55
Q

In a normal adult what should the cell make up of their BAL be?

A

Macrophages > 85%
Lymphocytes 10-15%
Neutrophils <3%
Epithelial <1%.

NB if epithelial cells > 5% then you have not sampled the alveolar space

56
Q

What gives you >1% eosinophils on BAL?

A

Eosinophillic Pneumonia
Drug induced pneumonia
BMT
Asthma, Bronchiolitis
Churg Strauss
ABPA
Bacterial, fungal, Helminth , Pneumocystis infection
Hodgkins

57
Q

What gives you >3% neutrophils on BAL?

A

Collagen /vascular disorders
IPF
Aspiration Pneumonia
Infection: Bacterial/ fungal
Bronchiolitis
Asbestosis
ARDS
Diffuse alveolar damage

58
Q

What is CD4+/CD8+ > 4 on BAL indicative of ?

A

Highly specific for sarcoidosis (in absence of higher proportion of other cell types)

59
Q

What is eosinophil >25% on BAL indicative of ?

A

Acute or chronic eosinophillic pneumonia

60
Q

What is neutrophil count >50% on BAL indicative of ?

A

Acute lung injury
Aspiration Pneumonia
Suppurative infection

61
Q

What is bloody fluid incr on subsequent alliquots on BAL indicative of ?

A

Pulmonary haemorrhage/ Diffuse alveolar damage

62
Q

What is milky fluid with positive periodic acid Schiff staining and amorphous debris indicative of ?

A

Pulmonary Alveolar Proteinosis

63
Q

What is the diagnostic test for HSP?

A

BAL and TBB (NB in fibrotic HSP will need to be surgical biopsy/ cryobiopsy; in non fibrotic HSP TBB and surgical biopsy )

64
Q

Are serum precipitans useful in HSP?

A

Marker of exposure , not disease , 40% of farmers will have

65
Q

How do we treat HSP?

A

Antigen avoidance !!!

If poor lung function, very symptomatic , evidence of fibrosis - Prednisolone 0.5 mg/kg 2-4 weeks and taper ( taper as per response , HRCT , lung function - no definitive guidance)

If steroids alone not improving things can add MMF /AZA

66
Q

What makes prognosis of HSP worse?

A

Intensity of exposure
Age
Clubbed
Traction bronchiectasis/ honeycombing on CT
NSIP and UIP on histology
Fibroblastic foci
Unknown antigen

67
Q

Talk through pulmonary langerhans cell histocytosis

A

Cystic lung disease strongly associated with cigarette smoking

Initially thought to be reactive but now evidence of MAPK pathway alteration in 85% and BRAVF 600 mutation in 36-50%

Sx : SOB , cough , fever

15% have PTX , 63% have recurrence. Can cause bone cysts , DI (can proceed lungs) , multi system involvement <1%

HRCT: upper and mid zone bizarrely shaped cysts, diffuse centrilobular nodules , sometimes cavitating

PFTs: mixed restrictive / obstructive

TBB- may yield dx material but often unhelpful as risk of PTX, surgical lung biopsy preferred but only if atypical ie purely cystic, purely modular

Mx : smoking cessation (rarely Cladribine) ; lung transplant if severe and pulmonary HTN

68
Q

Talk through Lymphangioleiomyomatosis (LAM)

A

Rare
Caused by abnormal proliferation of smooth muscle cells

(termed sporadic LAM if not associated with tuberous sclerosis)

Female , epilepsy, learning difficulties , associated with tuberous sclerosis

70% have PTX , recurrence 73%

Lymphatic obstruction can lead to chylothorax

Associated with abnormal angiomyolipoma

HRCT: variable sized cysts distributed throughout lung
CT Abdo - check for angiomyolipoma

PFTs: obstructive or mixed

Dx: Consider in young/Middle Aged women who have recurrent pneumothorax and cystic lung disease / obstruction / chylous pleural effusions and angiomyolipoma and tuberous sclerosis
Mx: Mx PTX, avoid oesteogen, sirolimus

69
Q

Talk through Burt Hogg Dube

A

Multi-system disorder characterized by :
- cutaneous manifestation , typically fibrofolliculomas (skin tags)
- multiple lung cysts
- PTX
- increased risk of renal tumours
- genetic mutation in the follicular gene

HRCT: Basal predominance, paramediastinal/ perifissural , irregular shaped , round and elongated adjacent to the interlobular septa and vessels

70
Q

Talk through Lymphoid Interstitial Pneumonia

A

Definition: Interstitial pneumonia characterised by lymphoid infiltration and often lymphoid hyperplasia

Causes: Idiopathic (rare), CTD (Sjogrens, SLE, RA), Immunodeficiency (HIV, CVID), Infection (PCP, Hep B), AI disease, Drugs (Phenytoin)

Clinical fx: Gradual onset breathless, fever , WL , crackles on exam

Ix:
Bloods: mild anaemia, poly/monoclonal increased immunoglobulins
HRCT : Ill defined cebtrilobular and subpleural nodules cysts , scattered and thin walled. Predominant ground glass
BAL: non clonal lymphocytosis

Treatment: Steroids

71
Q

What is anti synthetase syndrome?

A

An autoimmune disorder characterised by antibodies against an aminoacylT RNA synthetase along with one or more of the following : ILD, myositis , Raynaud’s, arthritis

Two diagnostic criteria are proposed :

  1. Presence of an anti amino acyl tRNA synthetase antibody
    AND
    One or more of the following clinical features :
    Raynauds, Arthritis , ILD, Fever (not attributable to another cause) , mechanics hands

2 major / 1 major and 2 minor

Major: ILD , polymyositis/dermatomyositis

Minor Arthritis, Raynauds, Mechanic hands

72
Q

A transbronchial biopsy specimen shows abundance of eosinophils and birbeck granules, what is the diagnosis ?

A

Pulmonary Langerhans Cell Histocytosis

73
Q

What are Birbek granules?

A

Well demarcated cytoplasmic organelles- LCH

74
Q

How do you restart Pirfenidone after interruption?

A

If interrupted for more than 14 days : Start with 267 mg TDS for 7 days , then 534 mg TDS for 7 days then 801 mg TDS

75
Q

What is the gene mutated in Birt Hogg Dube ?

A

FLCN gene

76
Q

What is the most common tracheobronchial abnormality in granulomatosis with polyangitis?

A

Subglottic stenosis

77
Q

What is Heerfordt-Waldenstrom syndrome ?

A

Rare subtype of sarcoidosis . More common in Afro-Caribbean patients and 15% of patients have relatives who suffered from it

Characterised by : enlargement of the parotid or salivary glands , facial nerve paralysis and anterior uveitis

78
Q

What are the systemic association of LCH?

A

Severe PHT - may be seen in the absence of significant parenchymal involvement, direct involvement of pulmonary vessels has been described

Systemic LCH- particularly diabetes insipidus from pituitary disease , skin involvement , lytic honey lesions , rarely cardiac/ GI

Lymphoma - may proceed, complicate or co-exist

79
Q

What other organs are affected in LAM?

A

Kidney: Angiomyolipoma (Benign ; 50%) - tumour respected or embolized

Abdomen: lymphadenopathy - due to lymphatic obstruction

Pelvis: Lymphangioleiomyoma - cystic mass that enlarges

Chylous ascites (can occur in absence of chylothorax)

Skin- cutaneous swellings

80
Q

What is cryptogenic organising pneumonia ?

A

ILD characterised by plugging of the alveolar spaces with granulomatous tissue, can expand into bronchioles

81
Q

Who gets Cryptogenic Organising Pneumonia (COP)?

A

More common in non smokers
Male = Female
55yo mean onset

82
Q

What can cause Organising pneumonia ?

A

Cryptogenic
or
Organising pneumonia secondary to :
Infection, Drug, CTD, Diffuse alveolar damage, Haematological malignancy , IBD, Post BMT, Pulmonary infarction, Lung malignancy /obstruction

NB often get some OP surrounding a lung cancer

83
Q

What does HRCT of organising pneumonia show?

A

Consolidation with air bronchograms sometimes with associated ground glass or nodules. Basal, subpleural and peribronchial . May migrate. Reticulation may represent poor response to tx

84
Q

How do you diagnose OP and how treat ?

A

Clinical + HRCT +/- Biopsy (if needed should be surgical)

Tx: Steroids (AZT/ Cyclophosphamide if poor response)

85
Q

Outline Acute Interstitial Pneumonia (AIP) - formerly known as Hamman Rich

A

Definition: Rapidly progressive form interstitial pneumonia characterised histologically by diffuse alveolar damage

Clinical: Often proceeded by viral illness , rapid onset <3 weeks after sx onset

HRCT: Bilateral diffuse ground glass and patchy airspace consolidation , later traction bronchiectasis , cystic change and reticulation

Dx : Biopsy to exclude ARDS (practically difficult)

Tx : Nil evidence but usually high dose steroids once infection ruled out

Mortality is 50%

86
Q

What is Respiratory Bronchiolitis ILD? (RB-ILD) ?

A

Respiratory Bronchiolitis refers to the accumulation of bronchiolar pigmented macrophages in cigarette smokers and is nearly always asymptomatic

Minority of smokers develop ILD , exact relationship between DIP and RB-ILD is unclear

87
Q

What does HRCT of RB-ILD show?

A

Poorly defined centrilobular nodules , ground glass change , thick walled airways often associated with centrilobular emphysema

88
Q

What is Desquamative Interstitial Pneumonia (DIP)?

A

ILD in smokers associated with pathological finding of pigmented macrophages located diffuse throughout alveolar space (more extensive form of RB-ILD)

Very rare

89
Q

What does HRCT with DIP demonstrate?

A

Ground glass change, lower zone , peripheral predominant , Reticulation and honeycombing may be evident but are mild

90
Q

Tx for RB-ILD and DIP?

A

Smoking cessation!

DIP often steroids also RB-ILD steroids occasionally

91
Q

What are the baseline cardiac tests for sarcoid ?

A

ECG and echo

If abnormalities in ECG or echo suggestive of sarcoid then should get Cardiac MRI or PET

92
Q

What is Lofgren’s Syndrome?

A

Bilateral Hilar Lymphadenopathy
Erythema Nodosum
Arthralgia

Occurs in 30% patients and remits 70-80% within 2 years ; often steroids needed for severe arthralgia but usually shorter courses with high doses 30-40mg for 1-2 weeks and tapering

Can be discharged once resolution confirmed

93
Q

What is the first line agent and regimen ins sarcoid tx ?

A

Steroids, if acute disease 20-40mg for 4-6 weeks and then wean by 5mg every 2 weeks to maintenance of 5 or 10mg and attempt withdrawal at 6-12 months

94
Q

What is the second line treatment in Sarcoid?

A

Methotrexate or Azathioprine

95
Q

Outline issues related to Vitamin D supplementation and Sarcoid

A

Patients with sarcoid may have low 25-OH-Cholecalciferol (Vitamin D3) , Serum 1,25 (OH) 2 Cholecalciferol may be elevated due to granuloma macrophages over expressing 1 alpha hydroxylase which can lead to hypercalcemia or hypercalcurea. So if giving Vitamin D supplementation should be done carefully

96
Q

What is the Scadding Staging of Sarcoid ?

A

0- Normal (5-15%)
1- Enlarged nodes only (45-65%)
2- Enlarged nodes and parenchymal changes (30-40%)
3- Parenchymal changes without enlarged nodes / fibrosis (10-15%)
4- Fibrosis (5%)

97
Q

What % of patients will have radiological and clinical resolution without treatment (by stage) ?

A

0: n/a
1: 50-90%
2: 30-70%
3: 10-20%
4: 0%

In total around 40% of patients with sarcoid will remit within 6 months but it’s not clear which ones

98
Q

What are the positive signs for cardiac sarcoid on investigations ?

A

CMR: late gadolinium enhancement
PET: significant FDG uptake

(NB on ECG - advanced atrioventricular block, ventricular tachycardia; Echo- LV systolic/ diastolic impairment)

99
Q

What are findings associated with pulmonary htn in cardiac sarcoid ?

A

Reduced DLCO in setting of restrictive
Increased PA diameter (≥29mm)
Increase in PA diameters /ascending aorta diameter ratio (≥1)
O2 sats < 90% on 6MWT
Need for LTOT

100
Q

What are the CT findings in sarcoid ?

A

LN enlargement
- Hilar +/- mediastinal
- Bilateral and symmetrical
- Well defined homogenous

LN classification
- Icing sugar
- Eggshell

Nodules:
- well defined 2-5mm
- paraseptal predominant
- fissural beading / peribronchovascular nodularity
- Colaescence

Fibrosis
- Reticular opacity
- Volume loss / architectural distortion
- Traction bronchiectasis
- Peribronchovascular air trapping / mosaic pattern

101
Q

What are the 2 situations in sarcoid where you don’t require tissue biopsy (bar obviously patient choice etc) ?

A

Lofgren’s Syndrome
Long standing Pulmonary disease

102
Q

What do you expect to see /sample on BAL for sarcoid?

A

Nodules 2-3mm waxy yellow mucosal lesions with mucosal cobble stone , biopsy these

BAL - lymphocytes 15-25% provide support for granulomatous disease (but seen in others)
- CD4:CD8 > 4 (in the absence of increased proportion of other inflammatory cells is highly specific

103
Q

When do we treat sarcoid ?

A

If potential danger of fatal outcome or permanent disability

Or

Unacceptable QOL

104
Q

What are the spirometric cut offs loosely applied for sarcoid for when to treat?

A

DLCO <65%
Spirometric <70%
FVC 10% drop
TLCO 15% drop

Caveat would be in the presence of inactive fibrotic disease which may result in irreversible but non progressive lung function loss

105
Q

What are the indications for 2nd line agents ?

A
  1. Progression of pulmonary disease or an unacceptable symptom burden despite steroid treatment
  2. Intolerable side effects from steroids
  3. Inability to taper to below 10-15mg
  4. Presence of major comorbidities likely to be adversely affected by steroids
  5. Strong patient aversion
106
Q

What are the second line options for Sarcoidosis?

A

MTX or AZT
MMF
Leflunomide

107
Q

What is methotrexate induced pneumonitis?

A

Productive cough, dyspnoea and fever can occur acutely within days to weeks after initiation or can be insidious
On CT will get new ground glass change , particularly suggestive if changes differ from pre-existing. Poorly formed granulomas on biopsy but fibrosis is not a feature

108
Q

In what settings within sarcoid is Hydroxychloroquine useful?

A

Mainly for fatigue, joint and skin sarcoid

109
Q

What is the third line treatment in sarcoid?

A

Infliximab + AZT/MTX

110
Q

Do ACE/ BAL lymphocytosis/Gallium scanning correlate with disease progression in sarcoid?

A

No

111
Q

What are the fx of steroid withdrawal in presence of normal hypothalamic - pituitary - adrenal axis?

A

Fatigue, dizziness , headache, mood swings , athralgia , myalgia , nausea and abdo pain

112
Q

What are the cut offs for early morning cortisol?

A

<100 Insufficient
100-450 Stimulation test, d/w Endo
>450 Sufficient

113
Q

What is the GAP score ?

A

GAP score can be used to predict mortality in the case of Idiopathic Pulmonary Fibrosis (IPF). It uses four parameters: gender, age, and two physiological parameters of FVC and diffusion capacity of carbon monoxide (DLCO). On the basis of GAP score, patients are grouped into 3 stages with each successive stage predicting poorer prognosis.

114
Q

What effect does Ciprofloxacin have on Pirfenidone ?

A

Ciprofloxacin enhances Pirfenidone so need to reduce dose of Pirfenidone , ideally avoid Cipro

115
Q

What are the contraindications to Pirfenidone ?

A

eGFR < 30
Child Pugh C
Smoking
Pregnant

116
Q

What are the contraindications to Nintedanib?

A

MI last 6/12
Unstable angina
Pregnant
Hypersensitivity to peanut /soy

117
Q

What is the median survival following an exacerbation of IPF?

A

3 months

118
Q

What are some common drugs associated with drug induced organising pneumonias ?

A

Amiodarone
Interferons
Minocycline
Rituximab
Statins

Drug withdrawal usually leads to improvement