CASE PRESENTATION
CASE PRESENTATION2010-07-09R3
Right lung upper and mid portion peripheral portion irregular
patchy consolidation RUL more prominent. right lung irregular
ground glass opacity(GGO) LLL focal consolidation GGO . Both lung
subpleural portion intralobular interlobular septum thickening
reticular density traction bronchiectasis, honeycombimg cyst .
lower lung prominent. Both lung paraseptal emphysema centrilobular
emphysema BUL prominent . BUL interstitium fibrosis paraseaptal
emphysema centrilobular emphysema wall prominent . Major vessel
scanty amount pneumomediastinum . Rigth paratracheal portion
subcarina portion short diameter 10-13mm several lymph node . LAD
atherosclerotic calcification .
1. Pneumonia in right lung and R/O LLL, more severe in RUL.2.
UIP(usual interstitial pneumonia) in both lungs. 3. Paraseptal and
centrilobular emphysema, more prominent both upper lungs, 4. Small
amount of pneumonediastinum. 5. Several enlargement of mediastinal
lymph node, reactive hyperplasia rather than pathologic condition.
6. Atherosclerotic calcification at LAD.
1
CASE10297654 67/MC.C: cough, sputum, fever (onset:20
days)Smoking : 3 , 1ppd x 30yrs
2
Suggestive of both lung fibrosis, such as UIP (usual
interstitial pneumonia).
--------------------------------[Reading] Both lung diffuse
reticular density BML BLL zone homeycomb cyst diffuse . 3
UIP and EmphysemaHRCT scan A number of patients with IPF(30%)
Combination of emphysematous lesions(upper) and pulmonary
fibrosis(lower) combined pulmonary fibrosis & emphysema
Pathophysiology : unknownBut may be related to common environmental
trigger or genetic susceptibility factor - ex> Smoking
initially reported to becoincidental2, but has now been proposed
as a distinct syndrome7
Combined pulmonary fibrosis & emphysemaDisease entity
distinct from UIPCharacteristic imaging featuresRelatively well
preserved lung volumesStrongly impaired CO transfer, PaO2 on
exerciseHigh prevalence of pulmonary hypertensionPoor survival
Clinical featureMen, smokers or ex-smokers (>40
pack-years)Mean age: 65 yearsExertional dyspnea, basal crackles on
auscultation.
PFT: RV and RF: normal or subnormal, but CO transfer: reduced,
exercise hypoxemia
The diagnosis is based on findings on high resolution
computedtomography (HRCT) of the chest, which show either
centrilobular emphysemaor upper-zone bullous emphysema, associated
in 90% of cases with verysuggestive paraseptal emphysema and
diffuse infiltrating fibrosing lungdisease at the bases (subpleural
reticular opacities, honeycomb images,traction bronchiectasis),
with more frequent ground glass opacities thanin IPF (Figure 1).
Pulmonary hypertension is present at diagnosis in almosthalf of all
patients and represents the principal negative prognostic factorfor
this condition, which has a median survival of 25 months.8
Complication of UIPPulmonary infection: m/c cxTraction
bronchiectasis, poor clearance of mucusLung cancerIncreased risk of
bronchogenic carcinomaFrequency: 4-48%Pulmonary HTN: poor
pxRelatively common(30-50%)Vasoconstriction & loss of pul.
capillaries Acute exacerbationCoronary artery disease, DVT,
pulmonary embolism, GERD, pneumothorax
acute onset of dyspnea (< 1 month) with worsening hypoxia and
progressive infiltrates seen in the absence of heart failure or
infection. New ground-glass infiltrates are seen on chest CT scans
with diffuse alveolar damage superimposed on a background of usual
interstitial pneumonia that is evident on histopathology. In many
patients with idiopathic pulmonary fibrosis, death is triggered by
a complicating illness, mainlyTraction bronchiectasis, poor
clearance of mucus, and perhaps an increased incidenceof
gastroesophageal reflux predispose patients with idiopathic
pulmonary fibrosis to lower respiratorytract infections.Even in the
absence of a complicating disease, themedian survival after the
diagnosis of biopsy-confirmedidiopathic pulmonary fibrosis is less
than threeyears.rupture of the subpleural honeycombing ...
9
Clinical analysis of the acute exacerbation in patients with
idiopathic pulmonary fibrosis
From 1994 to 2004 112 pts with IPF, 56 pts died.Due to Aex
(42.9%), lung cancer (21.4%), ch. respiratory failure (14.3%),
lower respiratory infections (8.9%).Aex of IPF : 25.0%, Death of
Aex: 85.7%
Nihon Kokyuki Gakkai Zasshi. 2006 May;44(5):359-67.
10
REFERENCEIdiopathic Pulmonary Fibrosis and Emphysema Chest
2009;136;10-15IDIOPATHIC PULMONARY FIBROSIS NEJM 2001;345(7);
517-525 Combined pulmonary fibrosis andemphysema: a distinct
underrecognised entity Eur Respir J 2005; 26: 586593
