History History Admitted to Wrexham Hospital Injuries: 1) fractures of maxilla and mandible, floor of R orbit, nasal bones & soft tissues 2) fracture one rib, collapse R lung. Bilateral pneumothoraces. Onset of adult respiratory distress syndrome (ARDS) 3) ventilated for eight weeks. Transplant?
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History Admitted to Wrexham Hospital Injuries: 1)fractures of maxilla and mandible, floor of R orbit, nasal bones & soft tissues 2)fracture one rib, collapse.
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HistoryHistory
Admitted to Wrexham Hospital
Injuries:
1) fractures of maxilla and mandible, floor of R orbit, nasal bones & soft tissues
2) fracture one rib, collapse R lung. Bilateral pneumothoraces. Onset of adult respiratory distress syndrome (ARDS)
3) ventilated for eight weeks. Transplant?
Clinical ProgressClinical Progress
• Jan 1997; referred for transplant assessment. QoL good and lung disease static
• August 97; holiday in Spain!
• Sept 98; sad, breathless and no QoL Isolated
• Oct 98; transplant assessment
Pathologists and clinicians each developed their own nomenclature
Differing terminologies in different countriesDiffering terminologies in different countries
IPF (USA and ASIA) = CFA (GB)
Idiopathic BOOP (USA ) = COP (GB)
+ many synonyms for IPF/CFA
such as: Hamman-Rich syndrome
"honeycomb lung'
Osler-Charcot disease
Realisation - a worse prognosis with some patterns, e.g. UIP cf. DIP
HRCT, correlated with pathology, separated the different groups
AIP - histopathologyAIP = ARDS on histology - no identifiable aetiology in AIP
Exudative phase dev. in week 1 post - insult - interstitial &
intra-alveolar oedema, hyaline membranes, type II
pneumocytes, intra-alveolar haem. and interstitial
mononuclear inflamm.
In hrs. after insult, ?only in neutrophils in alv. cap. and
interstitial oedema
Bouros D Eur Respir J 2000; 15: 412 -18
AIP – histopathology (2)
Prolif. stage during week 2 – ↑ fibroblastic proliferation in interstitium & alveoli, ↑ type II cells & nuclear atypia - ?malignant, esp. on cytology
Combinations of patterns often seen
Bouros D Eur Respir J 2000; 15: 412 -18
NSIPNSIP
DefinitionDefinition - “an idiopathic interstitial pneumonia with a histologic patten that does not conform to the characteristic features of UIP, RB-ILD, DAD, or COP.”
Main feature is a temporarily HOMOGENEOUSHOMOGENEOUS pattern of inflammation or fibrosis
DefinitionDefinition - “UIP is a histologic pattern seen in the clinical setting of diffuse, bilateral interstitial lung disease. Changes often distributed along the subpleural and paraseptal regions”
Main feature is a PATCHY, PATCHY, TEMPORALLYTEMPORALLY HETEROGENEOUSHETEROGENEOUS fibrosis with scattered fibroblastic foci at the edge of fibrotic scars, causing lung remodeling and honeycombing
Aetiological differentialAetiological differentialdiagnosis of UIP patterndiagnosis of UIP pattern
Collagen vascular disease
Drug - induced pneumonitis
Asbestosis
Radiation pneumonitis
Hermansky - Pudlak syndrome
EAA
Idiopathic UIP
Histological patterns are distinctive but Histological patterns are distinctive but not not specificspecific
UIPUIP - collagen diseases, asbestosis
COP or idiopathic BOOPCOP or idiopathic BOOP - can be seen in a variety of conditions (Tx, infection)
NSIPNSIP - EAA, collagen diseases, end result of DAD, drugs etc
Variation in histological patterns of ILD between Variation in histological patterns of ILD between connective issue diseasesconnective issue diseases
PM/DM (13) - OP +/- NSIP (one UIP)
RA (17) - Follicular bronchiolitis +/- NSIP (two UIP)
Sjögrens (5) - Chronic bronchiolitis +/- NSIP
SLE (2) - UIP (1), other follicular bronchiolitis & minor compnt. cellular NSIP
Tansey D et al. Histopathology 2004; 44: 585-96
CTD-UIP has ↓fibroblast foci, emphysema & honeycombing, ↑
germinal centre & total inflamm. scores (Song JW Chest 2009;136: 23)
UIP + NSIP
Cases with mixed patterns (NSIP plus UIP)
64 cases of ‘CFA’ with multiple bx. showed 25 concordant cases of UIP (present in all lobes), 8 discordant (UIP + NSIP) and 31 NSIP (3 cellular and 28 fibrotic)
Patients in concordant NSIP group had significantly better survival than discordant or concordant UIP (p = 0.02 and p = 0.04, respectively)
No significant diff btwn. concordant and discordant UIP grps. (p = 0.48)
75% of concordant NSIP group alive 5 years after biopsy, 17% of concordant and 37% of in the discordant UIP group alive
Monaghan H et al. Chest 2004; 125: 522 – 6
NSIP vs. UIPUIP pattern most important predictor of prognosis
Risk ratio of mortality in UIP (106 pts) >28.5, after controlling for age, symptom duration, radiology, physiology & sex
NSIP pts. (28 fibrotic & 5 cellular) more likely to respond or remain stable Honeycombing on HRCT indicated UIP – sens, 90%, spec of 86%
Dense interstitial inflammation involving 60% or of bx
Intrapleural fat
< 50% fibroblastic foci/cm2 in biopsy - median survival of 89m. cf. 49m. in those with > 50 FF/cm.2
No assocn. between FF at ∆ and DAD at autopsy
Travis WD et al. Am J Surg Pathol 2000; 24: 19-33
Titto, L et al. Thorax 2006; 61: 1091 – 5
Acute exacerbations of UIP
Defined as acute, clinically significant deteriorations of unidentifiable cause in pts. with underlying UIP
Proposed diagnostic criteria include; subjective worsening over 30 days or less, new bilateral radiographic opacities & nono infection or Identifiable aetiology. Histology – DAD (75%), OP or ↑ fib. foci
function, TGFβ), abnormal coagulation (procoagulant environ. as in ARDS), and genetic factors (polymorphism in erythrocyte complement receptor 1 and mutations in surfactant protein genes)
Churg A Am J Surg Pathol 2007; 31: 277 Kim DS et al ERJ 2006; 27: 143Collard HR et al. AJRCCM 2007; 176: 636
CFA - EBVCFA - EBV
IHC EBV DNA
CFA 12/27 14/27
control 3/28 4/28
p=0.005 p=0.007
81% of PCRassays confirmed
IHC results
Stewart, Egan, Hasleton et al. Am J Respir Crit Care Med 1999; 159: 1336
Idiopathicpleuroparenchymal fibroelastosis
Clinical presentation suggestive of chronic idiopathic interstitial pneumonia Marked pleural & parenchymal radiographic involvement with UL predominance
Pathology includes; Marked visceral pleural fibrosis ; Prominent, homogenous, subpleural fibroelastosis; Sparing of parenchyma distant from the pleura; Mild, patchy, lymphoplasmacytic infiltrates; and Small numbers of fibroblastic foci present at the leading edge of the fibrosis.
Frankel SK CHEST 2004; 126:2007–2013Becker CD Mod Pathol 2008;21: 784 – 7
BrIP - marked predilection for women (80%) in middle age (40–50 years)C/O SOB, cough, wheeze, chest pain and recurrent Pneumonia. 8/12 Churg’s pts had history of inhalational exposures (wood smoke, birds, cocaine etc) CT and PFT show restrictive diseaseMean FU of approx. 4 years in nine patients, 33% were DOD and 56% had persistent or progressive disease.? BrIP a unique entityYousem S Mod Pathol 2002; 15: 1148-53. Churg A Am J Surg Pathol 2004; 28: 62-8 Fukuaka J Am J Surg Pathol 2005; 29: 948-954