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
What are the ATS/ERS Classifications of UIP?
UIP Pattern Probable UIP pattern Indeterminate for UIP CT findings suggestive of alternate diagnosis
26
CT findings demonstrate peribronchovascular predominant with subpleural sparing what should you consider ?
NSIP
27
CT findings demonstrate perilymphatic distribution what should you consider ?
Sarcoidosis
28
CT findings demonstrate upper or mid lung what should you consider ?
Fibrotic HP CTD-ILD Sarcoidosis
29
CT findings demonstrate subpleural sparing what should you consider ?
NSIP Smoking related IP
30
CT findings demonstrate cysts what should you consider ?
LAN PLCH LIP DIP
31
CT findings demonstrate mosaic attenuation or three density sign what should you consider ?
HP
32
CT findings demonstrate predominant GGO what should you consider ?
HP Smoking related disease Drug toxicity Acute exacerbation of fibrosis
33
CT findings demonstrate profuse centrilobular micronodules what should you consider ?
HP Smoking related disease
34
CT findings demonstrate nodules what should you consider ?
Sarcoidosis
35
CT findings demonstrate consolidation what should you consider ?
Organising pneumonia
36
CT findings demonstrate pleural plaques what should you consider ?
Asbestosis
37
CT findings demonstrate dilated oesophagus what should you consider ?
CTD-ILD
38
What is NSIP
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
39
Who typically has NSIP
40-50yos Idiopathic CTD (NSIP may be first manifestation) Drug related Infection Immunodeficiency (HIV, BMT, Chemo)
40
Describe HRCT features of NSIP
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
41
What are the three types of NSIP on histology?
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
42
How do you diagnose NSIP pattern ?
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
43
How do you treat NSIP?
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
44
What is the prognosis in NSIP pattern
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)
45
What are squeaks on auscultation?
Signs of active alveolitis
46
What is HSP?
Hypersensitivity pneumonitis is a group of lung diseases caused by inhalation of an organic antigens to which an individual has been previously sensitized
47
What do you see on histological samples for HSP?
Loose granulomas
48
What are the most common causes of HSP?
MOULD Farmers lung — Thermophilic Actinomycete - mouldy hay, sugar cane, contaminated water Pigeon fanciers lung —protein in the droppings Mycobacterium avium —- hot tub lung
49
Is there a questionnaire for HSP?
None standardised , can use UCSF
50
How do we classify HSP?
Non fibrosing inflammatory HP Fibrotic (Mixed inflammatory plus fibrotic or pure fibrotic)
51
What is required to classify as non fibrotic inflammatory HSP?
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
What is required to classify a fibrotic (mixed inflammatory plus fibrotic or pure fibrotic) HSP?
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
What would you expect to see on BAL to support HSP dx ?
Lymphocytes > 30%; > 50% is strongly suggestive NB in smokers probably above 20% is significant If fibrotic HSP will likely be a normal BAL
54
What conditions give you a lympocytosis on BAL?
HSP CTD- ILD Sarcoid Infection Drug Induced ILD COP NSIP Chronic beryllium
55
In a normal adult what should the cell make up of their BAL be?
Macrophages > 85% Lymphocytes 10-15% Neutrophils <3% Epithelial <1%. NB if epithelial cells > 5% then you have not sampled the alveolar space
56
What gives you >1% eosinophils on BAL?
Eosinophillic Pneumonia Drug induced pneumonia BMT Asthma, Bronchiolitis Churg Strauss ABPA Bacterial, fungal, Helminth , Pneumocystis infection Hodgkins
57
What gives you >3% neutrophils on BAL?
Collagen /vascular disorders IPF Aspiration Pneumonia Infection: Bacterial/ fungal Bronchiolitis Asbestosis ARDS Diffuse alveolar damage
58
What is CD4+/CD8+ > 4 on BAL indicative of ?
Highly specific for sarcoidosis (in absence of higher proportion of other cell types)
59
What is eosinophil >25% on BAL indicative of ?
Acute or chronic eosinophillic pneumonia
60
What is neutrophil count >50% on BAL indicative of ?
Acute lung injury Aspiration Pneumonia Suppurative infection
61
What is bloody fluid incr on subsequent alliquots on BAL indicative of ?
Pulmonary haemorrhage/ Diffuse alveolar damage
62
What is milky fluid with positive periodic acid Schiff staining and amorphous debris indicative of ?
Pulmonary Alveolar Proteinosis
63
What is the diagnostic test for HSP?
BAL and TBB (NB in fibrotic HSP will need to be surgical biopsy/ cryobiopsy; in non fibrotic HSP TBB and surgical biopsy )
64
Are serum precipitans useful in HSP?
Marker of exposure , not disease , 40% of farmers will have
65
How do we treat HSP?
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
What makes prognosis of HSP worse?
Intensity of exposure Age Clubbed Traction bronchiectasis/ honeycombing on CT NSIP and UIP on histology Fibroblastic foci Unknown antigen
67
Talk through pulmonary langerhans cell histocytosis
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
Talk through Lymphangioleiomyomatosis (LAM)
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
Talk through Burt Hogg Dube
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
Talk through Lymphoid Interstitial Pneumonia
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
What is anti synthetase syndrome?
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
A transbronchial biopsy specimen shows abundance of eosinophils and birbeck granules, what is the diagnosis ?
Pulmonary Langerhans Cell Histocytosis
73
What are Birbek granules?
Well demarcated cytoplasmic organelles- LCH
74
How do you restart Pirfenidone after interruption?
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
What is the gene mutated in Birt Hogg Dube ?
FLCN gene
76
What is the most common tracheobronchial abnormality in granulomatosis with polyangitis?
Subglottic stenosis
77
What is Heerfordt-Waldenstrom syndrome ?
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
What are the systemic association of LCH?
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
What other organs are affected in LAM?
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
What is cryptogenic organising pneumonia ?
ILD characterised by plugging of the alveolar spaces with granulomatous tissue, can expand into bronchioles
81
Who gets Cryptogenic Organising Pneumonia (COP)?
More common in non smokers Male = Female 55yo mean onset
82
What can cause Organising pneumonia ?
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
What does HRCT of organising pneumonia show?
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
How do you diagnose OP and how treat ?
Clinical + HRCT +/- Biopsy (if needed should be surgical) Tx: Steroids (AZT/ Cyclophosphamide if poor response)
85
Outline Acute Interstitial Pneumonia (AIP) - formerly known as Hamman Rich
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
What is Respiratory Bronchiolitis ILD? (RB-ILD) ?
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
What does HRCT of RB-ILD show?
Poorly defined centrilobular nodules , ground glass change , thick walled airways often associated with centrilobular emphysema
88
What is Desquamative Interstitial Pneumonia (DIP)?
ILD in smokers associated with pathological finding of pigmented macrophages located diffuse throughout alveolar space (more extensive form of RB-ILD) Very rare
89
What does HRCT with DIP demonstrate?
Ground glass change, lower zone , peripheral predominant , Reticulation and honeycombing may be evident but are mild
90
Tx for RB-ILD and DIP?
Smoking cessation! DIP often steroids also RB-ILD steroids occasionally
91
What are the baseline cardiac tests for sarcoid ?
ECG and echo If abnormalities in ECG or echo suggestive of sarcoid then should get Cardiac MRI or PET
92
What is Lofgren’s Syndrome?
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
What is the first line agent and regimen ins sarcoid tx ?
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
What is the second line treatment in Sarcoid?
Methotrexate or Azathioprine
95
Outline issues related to Vitamin D supplementation and Sarcoid
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
What is the Scadding Staging of Sarcoid ?
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
What % of patients will have radiological and clinical resolution without treatment (by stage) ?
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
What are the positive signs for cardiac sarcoid on investigations ?
CMR: late gadolinium enhancement PET: significant FDG uptake (NB on ECG - advanced atrioventricular block, ventricular tachycardia; Echo- LV systolic/ diastolic impairment)
99
What are findings associated with pulmonary htn in cardiac sarcoid ?
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
What are the CT findings in sarcoid ?
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
What are the 2 situations in sarcoid where you don’t require tissue biopsy (bar obviously patient choice etc) ?
Lofgren’s Syndrome Long standing Pulmonary disease
102
What do you expect to see /sample on BAL for sarcoid?
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
When do we treat sarcoid ?
If potential danger of fatal outcome or permanent disability Or Unacceptable QOL
104
What are the spirometric cut offs loosely applied for sarcoid for when to treat?
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
What are the indications for 2nd line agents ?
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
What are the second line options for Sarcoidosis?
MTX or AZT MMF Leflunomide
107
What is methotrexate induced pneumonitis?
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
In what settings within sarcoid is Hydroxychloroquine useful?
Mainly for fatigue, joint and skin sarcoid
109
What is the third line treatment in sarcoid?
Infliximab + AZT/MTX
110
Do ACE/ BAL lymphocytosis/Gallium scanning correlate with disease progression in sarcoid?
No
111
What are the fx of steroid withdrawal in presence of normal hypothalamic - pituitary - adrenal axis?
Fatigue, dizziness , headache, mood swings , athralgia , myalgia , nausea and abdo pain
112
What are the cut offs for early morning cortisol?
<100 Insufficient 100-450 Stimulation test, d/w Endo >450 Sufficient
113
What is the GAP score ?
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
What effect does Ciprofloxacin have on Pirfenidone ?
Ciprofloxacin enhances Pirfenidone so need to reduce dose of Pirfenidone , ideally avoid Cipro
115
What are the contraindications to Pirfenidone ?
eGFR < 30 Child Pugh C Smoking Pregnant
116
What are the contraindications to Nintedanib?
MI last 6/12 Unstable angina Pregnant Hypersensitivity to peanut /soy
117
What is the median survival following an exacerbation of IPF?
3 months
118
What are some common drugs associated with drug induced organising pneumonias ?
Amiodarone Interferons Minocycline Rituximab Statins Drug withdrawal usually leads to improvement