ILD Flashcards

Question

Primary ciliary dyskinesia

Answer 1

Abnormal cilia so abnormal MC clearance so micro colonisation Chronic infection Bronchiectasis Ar Kids OM situs inversus Resp distress Adult Bronchiectasis clubbing infertility Saccharin test Reduced feno Abx PT Vaccine Haemorrhage management

Answer 2

Lung injury so increased vascular permeability Fluid and protein in alveolar space + surfactant damage Reduced compliance VQ mismatch and increased LtoR shunt Hypoxia PHN Dx Within a week of insult Bilateral infiltrates on CT Echo no RHF Pa02 over fio2 less than 300 Treat. Cause Permissive Hypercapnia To less than 6ml per kg Increase peep Plateau pressure less than 30 Paralyse Negative fb Ecmo

Answer 3

Environmental trigger Cd4 activated release il2 and ifn gamma so attract macrophages Release ace Granuloma fibroblast Non caseating granuloma Lofgrens EN BHL arthralgia — severe arthralgia steroid— 80% remits 2y Chronic infiltrative lung disease Stages lung O nil 1 BHL —90% resolve 2 BHL and parenchymal change —70% resolve 3 parenchymal change —20% resolve 4 fibrosis —0% resolve Other raised ace or ca Lung function obstructive CT Micro nodules perifissural GGO BHL Fibrosis late Ebus gold standard 93% sensitivity Not needed typical lung or Lofgrens Ebus Hx and afb Bal cd4: cd8 over 4. Lymphocytes over 25% Mx Symptomatic stage 2 onwards pred then wean. Higher dose pred cardio and renal Second line Mtx or aza or hq or MMf : indication progress/ SE pred/ not able taper below 10mg/ com/ pt wishes 3rd inflix + aza or Mtx S4 lung tx Pred indication Severe symptom life threatening Extrathoracic eg hyperca When to treat lung DLCO less than 65 Fev1 less than 70 Fvc drops 10% and TLCO drops 15%

Answer 4

CNS CN or neurosarcoid CSF ace and raised wcc Git cirrhosis Calcium raised Granuloma converts d3 to active vitamin d + macrophages produce calcitriol more ca absorbed Fluids and low dose pred Skin en or lupus pernio Topical pred then po Eyes uveitis Topical steroid then po pred or iss Cardiac 5% Cardiomyopathy with chb or pericarditis Echo and ecg Then holter with cmri Steroid amio to ppm to heart transplant Kidney Hyperca or GN Steroid then hq

Answer 5

Black Over 40 Neuropathy Lupus pernio Nephrocalcinosis Lofgrens good prognosis

Answer 6

Dendritic cell proliferation so Histocyte infiltrate lung and multi organ MAPK pathway 85% BRAVF 600 mutation 50% Unknown antigen BIRKBECK granules Smokers Pneumothorax 15% Systemic langerhans rib lesion PH DI Skin lesion HypoT Lymphoma Association. Phtn DI lymphoma lung cancer CT Nodules with cavity spare cpa Upper and mid zones Apical Thick walled cysts irregular Normal lung in between Bal pigmented macrophages Hx birkbeck granules Mixed defect pft Mx Don’t smoke Pred not evidence based Cladrabine Transplant if PH Pg variable

Answer 7

Abnormal proliferation smooth muscle and form cysts TSC gene Young female Non smoker Ass epilepsy, LD, TS and kidney tumour angiomyolipoma Cysts to Ptx Chylous effusion Obs spirometry or mixed CT Cyst various size with Normal lung between BUT MORE OVAL Lower lobes Include CPA Pleural effusion Other Lipomata kidney LN abdominal Skin swelling Chylous ascites Stop smoking Avoid oestrogen Sirolimus mTor inhx (IND poor lft) Pleural pleurectomy or pleurodesis or thoracic duct ligation Bad OCP increases symptoms

Answer 8

Defect follicular gene Present cystic lung disease, skin tags, Ptx, renal tumour Cystic lung disease Lower zone Oval lentiform septated cyst next to interlobular septa and vessel No GGO Other Fibrofolliculoma Kidney tumour AD FLCN gene

Answer 9

Cd4: cd8 High sarcoid Low hsp Neut (* more than 50%) ARDS ** Dah Aspiration pneumonia ** Bacterial infection ** IPF Asbestosis Lymph >25% hsp sarcoid Chronic berylliosis Cellular NSIP Drug reaction LIP COP Lymphocytes more than 50% HSP Cellular NSIP Eo Acute or chronic Eo pneumonia more than 25% Drug induced Asthma Egpa ABPA PJP Lymphoma Specific Proteinacious PAP Haemosidderin haemorrhage Macrophage foamy amiodatone

Answer 10

Scleroderma and silicosis CT Upper lobe nodules Fibrocalcinosis

Answer 11

Auto ir myositis Anti jo positive Anti rna synthetase Raynauds Arthritis Ild Fever PM or DM Mechanics hands CT OP NSIP

Answer 12

Cough and sob Rf for Ptx Ct Apical caps UL volume loss Traction bre

Answer 13

EGPA Dx Asthma Eo more than 10% peripheral Pulmonary infiltrates Sinus disease Mononeuritis Bx eosinophils PANCA MPO ————————————————— Werner’s GPA 90% lung involvement Dx Subglottic stenosis, nasal ulcer Nodules lung, pulmonary haemorrhage Renal failure GN CANCA positive PR3 Mx End organ issue eg pulm haemorrhage or Renal failure : methylpred then cyclophos then ritux/aza Plex Dialysis

Answer 14

Rare 100 per million Female middle aged GI skin raynaud calcinosis telengiectasia microstomia Anti scl 70 Pattern ILD NSIP then UIP Mx A) Lower dose pred 10mg used —Methylpred contra indicated risk Renal crisis B) MMF or Cyclophosphamide/aza C) Nintedanib in fibrotic NSIP no FVC cutoff. Reduce rate decline fvc — not for ritux or aza

Answer 15

Cyst Thin walled Normal lung LAM lCH LIP Birt Hogg dube Secondary UIP thick walled honeycomb PJP prox UL bilateral HSP

Answer 16

Rheumatoid Sle Systemic sclerosis PM/DM Pred then cyclophos or MMf or aza or ritux. Avoid high dose MP risk renal crisis esp Syst sclerosis Indication more than 20% lung or symptoms or reduced fvc Nintedanib indication - Worse FVC 10% 24m - Reduce FVC 5-10% with worse fibrosis and symptoms - Worsening symptoms and fibrosis Prognosis best for CTD ild then unclassified then ipf

Answer 17

Gord Wood Metal REPEATED ALVEOLAR EPITHELIAL INJURY ABERRANT WOUND HEALING

Answer 18

IPF CTD ILD esp RA CHRONIC HP SARCOID NITROFURANTOIN ILD ASBESTOSIS

Answer 19

Low bmi TLCO less than 40% TLCO decline 15% or fvc 10% in 6-12m PH Fibroblasts on Bx ——————————- Age Gender TLCO Desat 6wt to less than 88% TLCO less than 40% TLCO fall 15% in a year Fvc fall 10% in a year Other bmi low, ph

Answer 20

UIP Probable Indeterminate Alternate diagnosis

Answer 21

Fibrotic HP Sarcoid CTD ILD

Answer 22

HP Sarcoid LCH

Answer 23

Asbestosis

Answer 24

Systemic sclerosis or ct Ild

Answer 25

85% mc 10% lymphocytes 3% neutrophil 1% epithelial

Answer 26

Older Clubbed More exposure Fibrotic foci NSIP and UIP on histology Unknown antigen

Answer 27

Subtype sarcoidosis In Afro caribbeans Enlarged parotid and salivary gland. Facial nerve paralysis. Anterior uveitis

Answer 28

Reduced DLCO and restrictive Increased PA Sats less than 90% Need for LTOT

Answer 29

LN bilateral symmetrical homogenous icing sugar or eggshell Nodules paraseptal with perifissural beading that coalesce Large parenchyma UZ nodular infiltrates Fibrosis Reticular opacity Volume loss Traction Bronchiectasis Air trapping

Answer 30

Lofgrens Long standing pulmonary disease

Answer 31

Fatigue Joint swelling Skin

Answer 32

PDL1 antagonist Cough sob chest pain Ct NSIP w GGO / hsp /op Bal exclude infection Grade one mild dose reduce Grade two hold and pred Grade three ARDS - hold itu methylpred w Abx then post infection screen cyclo or mtx

Answer 33

Clubbing Inspiration creps

Answer 34

Ana Anca RHF anti ccp IG HIV Avian prec Myosotis panel

Answer 35

Thick walled 3-10mm

Answer 36

Fev1 falls 10% 6m DLCO falls 15% 6m DLCO <40% Desat on 6WT Sats < 88% on 8L Po2<8 on 5L Urgent Po2 less than 8 despite 10L Refractory RHf despite medication

Answer 37

TERT is short telomere syndrome CP grey prem, blood abnormality, nail dystrophy, OP, liver disease Muc5 is mutation in surfactant cells

Answer 38

ILD by disease RA 11% systemic sclerosis 50% Myosotis 40% Lupus 6% Mixed connective 60%

Answer 39

Older male smoker Increased duration disease Severe disease Positive serology

Answer 40

Reduces risk

Answer 41

UIP Then NSIP

Answer 42

White lines on CT

Answer 43

Ritux As good cyclophosphamide Better se profile Not licensed Toci Reduce fvc decline

Answer 44

Young women Anti ccp RhF CT UIP most common NSIP better outcome OP LIP or DIP rare Mx - Continue methotrexate - Pred no evidence base - MMF or cyclophos/aza - right thing in UIP RA retrospective study safe - Nintedanib safe w biologic Other Biological cause progress stop Ritux better Pg Worse UIP, severe, lower FVC decline 10% baseline

Answer 45

Rare form dermatomyositis 50-60 Female 10% risk cancers MDA5 NSIP OP Pred MMF then aza/mtx or ritux Bad prognosis

Answer 46

Ro and La AB LIP most common NSIP

Answer 47

Small vessel vasculitis MPO positive CT UIP

Answer 48

HRCT with expiratory hold

Answer 49

BAL and TBB diagnostic in non fibrotic Fibrotic lung biopsy Loose bronchocentric granulomatosis

Answer 50

IgG to precipins Exposure

Answer 51

HP upper lobe NSIP basal

Answer 52

Lymphocytic infiltration Sicca 25% Resp pleuritic chest pain due to thickening / dry cough/ LIP commonest then NSIP more than UIP or OP/ NHL 40x risk / pH rare

Answer 53

15% affects the lung UL fibrosis Pleural involvement Cyst or cavity Aspiration Restrictive defect

Answer 54

CP Oral or genital ulcer Skin lesion Arthritis Uveitis Resp Thrombosis Pulmonary aneurysm Effusion Eo pneumonia

Answer 55

20% lung manifestation Pleuritic pain Effusion Exudative or pseudochylo Drain and steroid Fibrosis Sob and cough Clubbing and crackles UIP more than NSIP Restrictive Raised ana Pred Poor prognosis Acute pneumonitis Pred Nodule Subpleural CX Ptx Pet less avid OP Fever cough sob Ct Bx Pred OB Proliferation terminal bronchioles Sob and cough. Squeak Obs pft Diagnosis BX Pred Vasculitis Association cryogenic/ arthritis/ Bronchiectasis/ Sjogrens/ Kaplan

Answer 56

Pleural Bilateral Exudative Raised lymph or neut Bx non specific Mx drain nsaid pred Atelectasis Ild 70% NSIP, 5% UIP Restrictive Good pg Other DAH OP Acute pneumonitis 50% mortality Non specific alveolar injury on Bx MP then cyclophos PH 50% 5y mortality Mx as PH 30% have antiphos Shrinking lung Sob and diaphragm weak Ct Normal parenchyma Restrictive with reduced TLCO Pred

Answer 57

Ild Sob cough arthralgia fever Fine basal crackles NSIP mostly UIP OP dAH Association Anti synthetase - polya fever mechanic hands myositis - ANA neg/ RNA synthase JO post MDA5 Anti pm scl DM Pred then ritux Poor prognosis OP or DAH pH Pharyngeal weakness and aspiration - restrictive - t2 rf Spontaneous pneumomed

Answer 58

Limited Crest Raynaud PF or PH in 25% Diffuse cutaneous Rauynaud PF or PH in 20% Overlap Systemic sclerosis ——————————- Pulmonary fibrosis Sob and raynauds Crest and crackles chest 89% ANA, anti scl Restrictive with reduced TLCO NSIP then rarer OP Pred then MMf or cyclophos Prognosis better than IPF PH G1 or G3 Sob CX RHF Prostacyclin iv Chest wall restriction due to skin thickening Aspiration pneumonia Bre

Answer 59

Tricuspid annular plane systolic excursion Displacement of lateral tricuspid annulus towards apex during systole measures RV function

Answer 60

Seropositive RA and Low exposure pneumoconiosis

Answer 61

Pause Rash goes Reintroduce lower dose comes back switch

Answer 62

More macrophages More eo

Answer 63

HP LCH RB ILD PCP Sarcoid

Answer 64

RBILD Ddx HSP

Answer 65

Resp mechanics reduced compliance and increased elastic loading so stretch Impaired gas exchange destruction of alveolar capillary membrane PH Peripheral muscle destruction

Answer 66

ANA and anca ENA has Scl70 diffuse SSc or Jo PM or Ro/La Sjogrens and Sle DsDNA SLE Ccp RA pANCA MPO or cANCA PR3

Answer 67

Cause - vasculitis eg GPA or goodpastures - bleeding DO - diffuse alveolar damage eg ARDS or drugs or infection or OP or AIP Respiratory failure Only 60% have haemoptysis Ix Septic screen inc atypical Viral serology Bronch 3x50 lovage progressively more bloody Mx ITU with lung protective measures Ecmo unclear due to AC High dose dex Correct coagulopathy Abx