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Interstitial pulmonary diseases. Pathologe.
2006 Mar;27(2):116-32.
Interstitial
pneumonia is a rare disease, posing a diagnostic challenge to
pneumologists, pediatricians, radiologists and pathologists. Only by
the combined efforts of the European Respiratory Society (ERS) and the
American Thoracic Society (ATS) has has been possible to standardize
the formerly different European and Northern American nomenclature of
interstitial lung diseases (alveolitis versus interstitial pneumonia)
in adults and to clearly and unambiguously define the diagnostic
criteria. The ATS/ERS classification of 2002 comprises seven entities:
usual interstitial pneumonia (UIP), non-specific interstitial
pneumonia (NSIP), desquamative interstitial pneumonia (DIP),
respiratory bronchiolitis-associated interstitial lung disease (RB-ILD),
cryptogenic organizing pneumonia (COP), lymphocyte interstitial
pneumonia (LIP), and acute interstitial pneumonia (AIP). Using the ATS/ERS
classification of interstitial pulmonary diseases in premature
infants, infants and children is problematic, since UIP, RB-ILD and
AIP do not occur at this age. Although infants with severe respiratory
insufficiency may sometimes show morphological features similar to DIP
or NSIP, this entity should rather be classified as chronic
pneumonitis of infancy (CPI) because of differences in etiology,
pathogenesis and prognostic outcome.
New
classification of interstitial lung disease.Rev
Pneumol Clin. 2005 Jun;61(3):133-40.
The
pathological classification of interstitial lung disease (ILD)
includes two general groups, diffuse infiltrative pneumonia with a
specific histological presentation due to primary disease of unknown
or unrecognized cause and idiopathic ILD. Diagnosis is established on
the basis of clinical, radiological and pathological findings. In the
first group of diffuse infiltrative pneumonia, the diagnosis is
usually straightforward, established on endoscopic biopsy, alveolar
lavage or surgical material depending on the case. The pathological
classification of idiopathic ILD requires a surgical specimen. The
entities redefined by the American Thoracic Society and the European
Respiratory Society (ATS/ERS) are: usual interstitial pneumonia, non
specific interstitial pneumonia, chronic organized pneumonia, diffuse
alveolar damage, desquamative interstitial pneumonia, desquamative
interstitial pneumonia with respiratory bronchiolitis and lymphocytic
interstitial pneumonia. The diagnosis of idiopathic pulmonary fibrosis
is established in a precise clinical and radiological context with an
aspect of common interstitial pneumonia of the biopsy material. It is
important to recognized common interstitial pneumonia because of the
severe prognosis and to distinguish it from non-specific ILD.
Idiopathic
interstitial pneumonia: a clinicopathological perspective.Semin
Respir Crit Care Med. 2006
Dec;27(6):569-73.
Idiopathic
interstitial pneumonia (IIP) represents a diverse group of lung
disorders with variable prognoses and responses to therapy. As the
name implies, the etiology is unknown. In the more severe forms of IIP,
such as idiopathic pulmonary fibrosis and acute interstitial
pneumonia, no effective therapies have been identified. In this
perspective, the spatiotemporal variability in the histopathology of
these disorders is discussed. It is proposed that common etiologies or
injurious agents may produce variable histopathological "reactions" in
the lung due to complex interactions between the host
(genetic/epigenetic factors, age) and environmental factors. Accurate
clinical and biological phenotyping may be necessary to stratify or
group patients who are most likely to respond to specific modes of
therapy.
Relationship
between histopathological features and the course of idiopathic
pulmonary fibrosis/usual interstitial pneumonia.Thorax.
2006 Dec;61(12):1091-5. Epub 2006 Jun 12.
BACKGROUND:
Fibroblastic focus (FF) is the typical histopathological feature of
idiopathic pulmonary fibrosis (IPF)/usual interstitial pneumonia (UIP).
A study was undertaken to analyse FF at diagnosis, to analyse the
histopathological findings at necropsy, and to examine their
association with the course of the disease. METHODS: A retrospective
study was made of 76 UIP cases collected over a period of 30 years
from one university hospital; 64 had idiopathic IPF. The surface area
of one slide of each lung biopsy specimen was defined by image
analysis and the total number of FF was quantified. The histological
features of necroscopic lung samples were re-analysed in 11 cases.
Clinical follow up information was obtained from the registers.
RESULTS: Patients with < or =50 FF/cm(2) (n = 34) in the lung biopsy
specimen had a median survival of 89 months (95% CI 38 to 140)
compared with 49 months (95% CI 36 to 62) in those with >50 FF/cm(2)
(n = 42, p = 0.0358). Diffuse alveolar damage (DAD) was detected in 10
necropsy samples and almost prevented the histopathological
confirmation of UIP in six cases. Accumulation of neutrophils occurred
in nine cases. There was no association between FF at diagnosis and
DAD at necropsy, or between FF and exacerbation of the disease before
death. CONCLUSIONS: The number of FF in lung samples before death is
associated with poor survival but not with DAD, which is a common
feature in necropsy specimens of patients with UIP. FF cannot predict
an acute exacerbation of IPF.
Clinicopathologic study of 50 autopsy cases of idiopathic pulmonary
fibrosis and non-diffuse usual interstitial pneumonia.Nihon
Kokyuki Gakkai Zasshi. 2005
Oct;43(10):569-77.
We
investigated 50 autopsy cases of idiopathic pulmonary fibrosis (IPF)
and non-diffuse usual interstitial pneumonia (UIP), and subgrouped
them into three subtypes based on morphologic differences in the
fibrosis (honeycomb): typical thick-walled honeycomb type (16 cases),
atypical thin-walled honeycomb type (27 cases) and atelectatic
indurated type (6 cases), with one undetermined case. In the
thin-walled type, the percentage of males (93%), the percentage of
smokers (89%), and the percentage of lung cancer cases (52%) were
significantly higher than in the other two subtypes (p < 0.02, p <
0.001 and p < 0.05, respectively). However, in the thick-walled and
indurated types there were significantly higher percentages of DAD
(38% and 67%, respectively) than in the thin-walled type (15%) (P <
0.05). Accordingly, each subtype was thought to be closely related to
its IPF clinical features, and showed differences in the development
of acute exacerbation and lung cancer. This study proposes the
existence of a UIP subset and suggests that this subgrouping can help
in the management of the disease.
Inflammatory
cell phenotyping of the pulmonary interstitium in idiopathic
interstitial pneumonia. Respiration.
2007;74(2):159-69. Epub 2006 Nov 14.
BACKGROUND:
Several studies have implicated the role of inflammation in the
pathogenesis of lung damage in idiopathic interstitial pneumonias (IIPs).
Investigations of inflammatory cells in IIP have show that eosinophils,
neutrophils and T cells may be associated with a poorer prognosis.
OBJECTIVES: The aim of our study was to map, by quantitative analysis,
the number of inflammatory cells in the lung tissue of patients with
non-specific interstitial pneumonia/non-specific interstitial
pneumonia (NSIP/NSIP), acute interstitial pneumonia/diffuse alveolar
damage (AIP/DAD) and idiopathic pulmonary fibrosis/usual interstitial
pneumonia (IPF/UIP) and to correlate them with lung function tests and
survival. METHODS: After immunohistochemical staining, we quantified
the content of inflammatory cells [macrophages, neutrophils (elastase+),
plasma cells, and CD3, CD4 and CD8 T lymphocytes (TLs)] in 20 NSIP, 20
DAD and 20 UIP surgical lung biopsies. RESULTS: The total density of
inflammatory cells was significantly increased in DAD and NSIP when
compared to UIP (p = 0.04). TLs were increased in DAD and NSIP when
compared to UlP lungs (p < 0.05). The density of inflammatory cells in
UIP showed significant differences in normal, intervening and dense
fibrosis areas (p < 0.05). The most numerous cells infiltrating the
mural fibrosis and honeycombing areas were plasma cells, neutrophils (elastase+),
CD20+, CD3+, CD4+ and CD8+ (p < 0.05). In UIP, CD3+ TLs were directly
correlated with forced expiratory volume in 1 s/forced vital capacity
ratio x 100 (p = 0.05). CD68+ cells presented a significant positive
correlation with the forced expiratory volume in 1 s (p = 0.04);
neutrophil (elastase+) cells significantly correlated with residual
volume (p = 0.02), residual volume/total lung capacity (p = 0.04) and
carbon monoxide transfer factor (p = 0.03). The most important
predictor of survival in UIP was CD3+ TLs (p = 0.05). CONCLUSION: The
total density of inflammatory cells and lymphocytes presents a
different distribution within the pulmonary parenchyma in AIP/DAD,
NSIP/NSIP and IPF/UIP evolutionary adapted responses to injury. There
is a localized distribution of inflammation in the normal, intervening
and dense fibrosis areas of UIP for CD3+, associated with a lethal
deterioration of the pulmonary function and poor survival. Our
findings provide further evidence of the importance of inflammation in
the pathophysiology of IIPs.
Abnormal
deposition of collagen/elastic vascular fibres and prognostic
significance in idiopathic interstitial pneumonias.Thorax.
2007 May;62(5):428-37. Epub 2007 Jan 24.
BACKGROUND:
Vascular remodelling has recently been shown to be a promising
pathogenetic indicator in idiopathic interstitial pneumonias (IIPs).
AIM: To validate the importance of the collagen/elastic system in
vascular remodelling and to study the relationships between the
collagen/elastic system, survival and the major histological patterns
of IIPs. METHODS: Collagen/elastic system fibres were studied in 25
patients with acute interstitial pneumonia/diffuse alveolar damage, 22
with non-specific interstitial pneumonia/non-specific interstitial
pneumonia and 55 with idiopathic pulmonary fibrosis/usual interstitial
pneumonia. The Picrosirius polarisation method and Weigert's
resorcin-fuchsin histochemistry and morphometric analysis were used to
evaluate the amount of vascular collagen/elastic system fibres and
their association with the histological pattern of IIPs. The
association between vascular remodelling and the degree of parenchymal
fibrosis in usual interstitial pneumonia (UIP) was also considered.
RESULTS: The vascular measurement of collagen/elastic fibres was
significantly higher in UIP than in the lungs of controls, and in
those with diffuse alveolar damage and those with non-specific
interstitial pneumonia. In addition, the increment of collagen/elastic
fibres in UIP varied according to the degree and activity of the
parenchymal fibrosis. The most important predictors of survival in UIP
were vascular remodelling classification and vascular collagen
deposition. CONCLUSION: A progressive vascular fibroelastosis occurs
in IIP histological patterns, probably indicating evolutionarily
adapted responses to parenchymal injury. The vascular remodelling
classification and the increase in vascular collagen were related to
survival in IIP and possibly play a role in its pathogenesis. Further
studies are needed to determine whether this relationship is causal or
consequential.
2000
classification of idiopathic interstitial lung diseases.
Rev Med Interne. 2004
Dec;25(12):891-905.
OBJECTIVES:
Diagnosis of interstitial lung diseases was recently improved by the
use of diagnostic tools, such as high-resolution Computed Tomography,
and by new insights in their pathogenesis and histology. This led the
American Thoracic Society and the European Respiratory Society to
propose a new classification of these diseases, in the aim to
facilitate early diagnosis and specific care. CURRENT KNOWLEDGE AND
KEY POINTS: Standard radiography gives the first suspicion of chronic
diffuse infiltrative lung disease, and anamnesis and physical
examination are essential steps of etiological diagnosis.
High-Resolution computed tomography confirms the diagnosis of diffuse
infiltrative lung disease. Longitudinal lung function tests are
essential to assess the consequences of the lung disease. Lung
biopsies are often, but not systematically, a useful tool. The 2000
classification consists of seven entities of idiopathic interstitial
diseases which are defined on clinical, radiological and pathological
criteria: idiopathic pulmonary fibrosis, non-specific interstitial
pneumonia, cryptogenic organizing pneumonia, acute interstitial
pneumonia, respiratory bronchiolitis associated interstitial lung
disease, desquamative interstitial pneumonia and lymphoid interstitial
pneumonia. The most frequent is Idiopathic Pulmonary Fibrosis, which
has a poor prognosis. FUTURE PROSPECT AND PROJECTS: This new
classification results from a multidisciplinary confrontation with
chest physicians, radiologists and pathologists. A better
characterization of anatomoclinical entities should lead to a better
pronostic evaluation, more informative comparisons of published
studies, and therefore to rational therapeutic approach.
Diffuse idiopathic
interstitial pneumonias. International multidisciplinary consensus
classification by the American Thoracic Society and the European
Respiratory Society, principal clinico-pathological entities, and
diagnosis.Rev Mal
Respir. 2004 Apr;21(2 Pt 1):299-318.
INTRODUCTION:
The classification of the idiopathic interstitial pneumonias includes
seven clinico-pathologic entities. The diagnosis is based on a
multidisciplinary approach, integrating the clinical evaluation, the
high-resolution computerised tomography, and the pathological pattern.
STATE OF THE ART: A definitive diagnosis of idiopathic pulmonary
fibrosis relies on the association of a suggestive clinico-radiological
profile and a pathological pattern of usual interstitial pneumonia.
Nonspecific interstitial pneumonia is a recently described clinico-pathologic
entity, with a better prognosis than that of idiopathic pulmonary
fibrosis. Cryptogenic organising pneumonia has been included in the
group of idiopathic interstitial pneumonias because of its idiopathic
and multifocal characteristics, although it does not predominate in
the lung interstitium. Desquamative interstitial pneumonia and
respiratory bronchiolitis with interstitial lung disease are rare
entities with predominance in young smoking adults. Lymphoid
interstitial pneumonia, usually encountered in the context of
Sjögren's syndrome, is very rare in its idiopathic form. Acute
interstitial pneumonia is responsible for idiopathic acute respiratory
distress syndrome. PERSPECTIVES: The current classification of
idiopathic interstitial pneumonias better defines the diagnostic
criterias of each clinico-pathologic entity, and is expected to
facilitate clinical research. CONCLUSIONS: This classification has
clinical implications, with prognostic and therapeutic significance.
Idiopathic
interstitial pneumonias: CT features.
Radiology. 2005;236(1) :10-21.
Idiopathic
interstitial pneumonias comprise usual interstitial pneumonia (UIP),
nonspecific interstitial pneumonia (NSIP), desquamative interstitial
pneumonia (DIP), respiratory bronchiolitis-associated interstitial
lung disease (RB-ILD), cryptogenic organizing pneumonia (COP), acute
interstitial pneumonia (AIP), and lymphoid interstitial pneumonia
(LIP). Each of these entities has a typical imaging and histologic
pattern, although in practice the imaging patterns may be variable.
Each entity may be idiopathic or may be secondary to a recognizable
cause such as collagen vascular disease or inhalational exposure. The
diagnosis of idiopathic interstitial pneumonia is made by means of
correlation of clinical, imaging, and pathologic features. The
characteristic computed tomographic (CT) features of UIP are
predominantly basal and peripheral reticular pattern with honeycombing
and traction bronchiectasis. NSIP is characterized by predominantly
basal ground-glass opacity and/or reticular pattern, often with
traction bronchiectasis. DIP and RB-ILD are smoking-related lung
diseases characterized by ground-glass opacity and centrilobular
nodules. COP is characterized by patchy peripheral or
peribronchovascular consolidation. AIP manifests as diffuse lung
consolidation and ground-glass opacity. LIP is associated with a CT
pattern of ground-glass opacity sometimes associated with perivascular
cysts.
Cigarette
smoking in interstitial lung disease: concepts for the internist. Med
Clin North Am. 2004 Nov;88(6):1643-53,
xiii.
Cigarette
smoking is a common cause of lung disease. It is clearly implicated in
the development of chronic obstructive pulmonary disease and lung
cancer. Importantly, cigarette smoking has also been implicated in the
development of interstitial lung diseases such as respiratory
bronchiolitis interstitial lung disease, desquamative interstitial
pneumonia, pulmonary Langerhans' cell histiocytosis, and idiopathic
pulmonary fibrosis. The exact role of cigarette smoking in the
development of interstitial lung diseases is still being defined; the
relatively low prevalence of interstitial lung disease makes
epidemiologic studies difficult.
The
idiopathic interstitial pneumonias. Curr
Probl Diagn Radiol.2004
Sep-Oct;33(5):189-99.
This review
includes the seven idiopathic interstitial pneumonias defined by The
American Thoracic Society and The European Respiratory Society 2002
publication. Idiopathic pulmonary fibrosis is the clinical term for
usual interstitial pneumonia. The radiologic pattern includes basal
and subpleural ground glass and reticular opacities and honeycomb
lung. Nonspecific interstitial pneumonia is characterized with a
radiologic pattern of subpleural and basal ground glass and reticular
opacities. Cryptogenic organizing pneumonia is manifest radiologically
by peribronchial ground glass opacities and subpleural consolidation.
Acute interstitial pneumonia is the clinical term for idiopathic
diffuse alveolar damage and the exudative phase is characterized
radiologically with diffuse ground glass opacification and dependent
consolidation with the additional feature of lung architectural
distortion in the organizing phase. Respiratory bronchiolitis
associated interstitial lung disease manifests as centrilobular ground
glass opacities on CT. Desquamative interstitial pneumonia is
characterized by ground glass opacities with lower zone predominance
on CT. Lymphoid interstitial pneumonia manifests by ground glass
opacities and nodular interlobular septal thickening on CT. The
diagnosis of an IIP should be rendered ideally only after all
clinicoradiologic-pathologic data have been reviewed.
Demystifying
idiopathic interstitial pneumonia.Arch
Intern Med. 2003 Jan 13;163(1):17-29.
Careful
histopathological evaluation has shown the traditionally clinical
diagnosis of idiopathic interstitial pneumonia to be more
heterogeneous than once thought. Its subclassification, based on
clinicopathological criteria, has important therapeutic and prognostic
implications. The most important distinction is the presence of usual
interstitial pneumonia, the histopathological pattern seen in
idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis has a
worse response to therapy and prognosis. New insight into the
pathophysiology of idiopathic pulmonary fibrosis suggests a distinctly
fibroproliferative process, and antifibrotic therapies show promise.
Although the clinical and radiographic diagnosis of idiopathic
pulmonary fibrosis can be made confidently in some cases, many
patients require surgical lung biopsy to determine their underlying
histopathological pattern. A structured, clinicopathological approach
to the diagnosis of idiopathic interstitial pneumonia, with particular
attention to the identification of idiopathic pulmonary fibrosis,
ensures proper therapy, enhances prognosis, and allows for further
investigation of therapies aimed at the distinct pathophysiology.
The prognostic
significance of the histologic pattern of interstitial pneumonia in
patients presenting with the clinical entity of cryptogenic fibrosing
alveolitis.
Am J Respir Crit Care Med. 2000 Dec;162(6):2213-7
Lone cryptogenic
fibrosing alveolitis (CFA) is a progressive interstitial lung disease,
with a median survival of 3 to 6 yr from the onset of dyspnea. CFA can
be subdivided into prognostically significant histopathologic
patterns, including nonspecific interstitial pneumonia (NSIP). We
reviewed 78 patients with a clinicopathologic diagnosis of CFA,
biopsied between 1978 and 1989, to evaluate the prevalence and
prognostic significance of these histopathologic patterns, in
particular NSIP. Biopsy appearances were reclassified by two pulmonary
histopathologists as usual interstitial pneumonia (UIP) (47%), NSIP
(36%), or desquamative interstitial pneumonia (DIP)/respiratory
bronchiolitis-associated interstitial lung disease (RBILD) (17%). The
kappa coefficient of agreement between pathologists was 0.49. In 67
cases, follow-up was complete to death or 10 yr after biopsy, with 50
deaths during a median follow-up of 42 mo (UIP, 89%; NSIP, 61%, DIP/RBILD,
0%). Survival was highest in DIP/RBILD and higher in NSIP than UIP, p
< 0.0005. When analysis was confined to patients with UIP or NSIP, the
mortality of UIP remained higher, p < 0.01, but the 5-yr survival in
patients with fibrotic NSIP was only 45%, indicating that this
histologic appearance is often associated with a poor outcome. A
response to treatment was more frequent in DIP/RBILD than in NSIP (p <
0.01) or UIP (p < 0.0005). This study confirms the prognostic value of
subclassifying patients with CFA according to histopathologic pattern.
However, in patients with clinically typical CFA, a histologic
diagnosis of fibrotic NSIP needs to be interpreted with caution and
does not necessarily denote a good outcome.
Classification
and recent advances in idiopathic interstitial pneumonia.
Curr Opin Pulm Med. 1998
Sep;4(5):256-60.
Idiopathic
interstitial pneumonia (IIP) is a heterogeneous group of diseases
comprising acute interstitial pneumonia, bronchiolitis obliterans
organizing pneumonia (BOOP), nonspecific interstitial pneumonia,
desquamative interstitial pneumonia, and idiopathic pulmonary fibrosis
and usual interstitial pneumonia (IPF/UIP). We review the
clinicopathological spectrum of IIP and introduce recent advances in
classification, treatment, and prognosis. BOOP can be clinically
categorized as an interstitial pneumonia, though prominent granulation
tufts are seen in the airspaces. Though differences between the
nonspecific interstitial pneumonia and other lips can be
histopathologically clarified, the focus of clinical research on NSIP
is differentiation from BOOP, or from IPF and UIP. IIP can be
categorized into two groups: groups with acute or subacute lung
injuries or fibrosis, such as in acute interstitial pneumonia, BOOP
and nonspecific interstitial pneumonia, and groups with chronic
injuries or fibrosis, such as IPF/UIP. This classification accords
well with the maturity of fibrosis, CT findings, bronchoalveolar
lavage fluid cell findings, and prognosis. The most critical problem
is the treatment of IPF/UIP, because of its high mortality.
The alphabet soup revisited: the chronic interstitial pneumonias in
the 1990s.Radiographics.
1996 Sep;16(5):1009-33; discussion 1033-4.
Liebow classified the idiopathic
interstitial pneumonias as usual (UIP), desquamative (DIP),
bronchiolitis obliterans (BIP), lymphoid (LIP), and giant cell (GIP)
interstitial pneumonias. This classification was modified to exclude
LIP and GIP. UIP, the most common type, is characterized by
synchronous foci of inflammation, collagen deposition, and fibrosis
with interspersed normal lung. It usually affects men 40-60 years old
and manifests radiologically with bilateral, basilar irregular
opacities and volume loss. In most cases, a confident diagnosis can be
made at high-resolution computed tomography because of characteristic
subpleural irregular linear opacities, ground-glass opacities,
honeycombing, and traction bronchiectasis. DIP affects younger
patients and is characterized by diffuse intraalveolar macrophage
aggregation. Typical radiologic features include bilateral, basilar
ground-glass opacities and preserved lung volumes. BIP, renamed
bronchiolitis obliterans with organizing pneumonia, affects
middle-aged patients and manifests with multifocal plugs of immature
fibroblasts in the air spaces. Typical radiologic features include
bilateral consolidations and normal lung volumes. Recently described
entities include acute (AIP) and nonspecific (NIP) interstitial
pneumonias and respiratory bronchiolitis with interstitial lung
disease (RB-ILD). AIP is a rapidly progressive, often fatal, illness
characterized by diffuse alveolar damage and manifests with clinical
and radiologic features of adult respiratory distress syndrome. NIP is
a heterogeneous group of fibrosing disorders that cannot be otherwise
classified. RB-ILD is a disease of smokers with a good prognosis.
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