Download Interstitial lung Disease(ILD)

Interstitial Lung Disease
ILD
General description
 ILDs
represent a large and
heterogeneous group of lower
respiratory tract disorders.
 There are similar clinical signs
and X-ray features.
The characteristic of clinical
signs including:

Dyspnea after exercising
 chest X-ray shows diffuse abnormality of
pulmonary parenchymal,including
nodules,linear(reticular) infiltrates
 pulmonary function tests shows restrictive
hypoventilation reduced diffusing capacity
 tissue biopsy shows a variety pulmonary
fibrosis and aveolar inflammation
Clinical Classification of ILD
 known
cause
 unknown cause (IIP,ects)
Pathogenesis
 The
pathogenesis of ILDs is
unknown.
 But more and more facts have
shown that immune cells and their
cytokines play an important role in
the course of ILDs.
Nowadays the major courses
of the ILDs including:

Intra-alveolar inflammation
 immune cells and their cytokines injure
epithelial and endothelial cells
 intra-alveolar fibrosis/alveolar collapse
In the course of ILDs, many
cytokines, including TGF-, IGF, prostaglandin E2, plateletderived growth factor, ects,
involve in.
Clinical manifestations

Breathlessness, Progressive respiratory
insufficency
 cough without sputum.
 Some patients may have fatigue, weight loss,
joint pain.
Physical examinations


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Bilateral basilar, crepitant velcro-like rale are
found in most patients
wheezing, rhonchi and coarse rales are
occasionally heard
with advanced disease, patients may have
tachypnea and tachycardia
clubbing of the fingers and toes is common
At last, pulmonary hypertention and cor
pulmonale may be exist
Chest radiography
It is important method to diagnose the ILDs.
The majority of ILDs cause infiltrates in the
lower lung zones.
 A diffuse
ground glass pattern is seen early in the
disease
 when the disease progresses, a chest radiography
demonstrates nodules, linear(reticular) infiltrates,
or a combination of the two
 at last, the infiltrates become coarser and lung
volume is lost
 honeycomb pattern may appear at the end of the
disease
nodular
linear
nodular
linear
honeycomb
ground glass pattern
Pulmonary function tests
Pulmonary function tests of ILDs shows
restrictive hypoventilation.
It includes:
 Reduced
lung volumes(vital capacity, total
lung capacity)
 reduced diffusing capacity
 static lung compliance is decreased
BALF examination
 the
cell counts in BALF of ILDs is twice
than that of normal humans
 cell complements of ILDs is difference from
that of normal humans
 for example, the percents of neutriphils in
BALF of IPF is higher than that of normal
humans
Blood examination
Lung biopsy
For example, TBLB(transbronchial biopsy),
an open-lung or thoracoscopic biopsy are
used to diagnose the ILDs
Idiopathic pulmonary fibrosis
IPF
 IPF
is an unknown chronic interstitial lung
disease.Nowadays It has become a common
disease.
 the clinical manifestations, and some
experimental examination including
pulmonary function tests,chest radiography
examinations and lung biopsy are coincide
to that of ILDs introduced before.
Pathology: According to the pathologic classification,
there are seven types of Idiopathic interstitial
pneumonitis.
 IPF-UIP(usual



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

interstitial pneumonitis)
NSIP, nonspecific interstitial pneumonitis
DIP, desquamative interstitial pneumonitis
RBILD, respiratory brnchiolitis associated interstitial
lung disease
LIP, lymphocytic interstitial pneumonitis
COP, concealed organizing pneumonia
AIP, acute interstitial pneumonitis
How to diagnose IPF
 According
to the clinical signs and some
experimental examinations, we can
diagnose the IPF except some known
cause ILDs
 lung biopsy is an only way to give a last
diagnosis
Clinical Diagnostic Standard of
IPF
 Except
known cause ILDS
 Lung function
 HRCT
 TBLB and BAL
 Age
 Unexplained dyspnea after exercise
 Period
 Physical examination
Chest radiography
Chest radiography
Pathologic Diagnosis
 The
pathologic diagnosis of IPF is
coincide with UIP
Treatment of IPF
 Nowadays,
the treatment ways of IPF are lack
of effective ways
 corticosteroids are the main therapy
 the initial treatment of choice is prednisone
0.5mg/kg of ideal body weight per day. For 1
month, the dose is gradually tapered over
several months to a maintenance dose of 0.125
mg/kg per day
 Immunosuppressive
CTX, MTX
 lung transplantation
agents,including
Treatment
 Some
common therapies, including oxygen
therapy, antibiotic therapy when pulmonary
infections exist.
prognosis
Sarcoidosis
Definition
 Sarcoidosis
is a disease of unknown cause and
is characterized by the presence of noncaseating granulomas in one or more organ,
system. It is considered a systemic disease
 Usually lungs and the lymph nodes in the
mediastinum and hilar regions are the most site
of involvement
 The clinical course is quite variable
asymptomatic
The cause of sarcoidosis is unknown.
But many researchers have suggested that
immune mechanisms are important in
disease pathogenesis.Genetic factor may
also play an important role.
Other factors including infectious
and environmental or occupational may
also involve in the sarcoidosis.
Pathogenesis
 Antigen
processing by macrophages is believed
to trigger an oligoclonal expansion of
CD4(helper-inducer) lymphocytes of the Th1
phenotype with production of IL-2 and IFN-.
 IL-2 cause proliferation of more CD4 cells,
elaborate more cytokines.
 Many cytokines, mainly IL-2,adhension
molecules and growth factors are released from
lymphocyte and macrophages.
The basic pathogenesis includes
three main stages:
 Pulmonary
alveolus inflammation
 formation of non-caseating
granulomas
 the stage of interstitial fibrosis
Clinical manifestations
 The
clinical course is variable
 the respiratory system is the most
commonly affected
 approximately 90% of patients
demonstrate intrathoracic involvement
on a chest radiograph
 sometime with or without extrathoracic
disease
Clinical manifestations
 Almost
30 to 60 per cent of patients have no
symptoms at the time of presentation
 sometimes the disease is identified because of
abnormalities on a chest radiograph
 some patients present with respiratory
symptoms such as dyspnea and cough, which
may or may not be accompanied by
constitutional symptoms, such as fever and
malaise
Specific signs and symptoms depend on the
particular organ system(s) involved
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Respiratory system disease
Intrathoracic nodal involvement and parenchymal lung
disease are the most common ways in which sarcoidosis
affeccts the respiratory system
Hilar and medistinal lymph nodes may be affected
The pulmonary parenchyma demonstrates well
defined,non-caseating granulomas with the pulmonary
interstitium
Usually upper lobes of the lung tend to be more
involved
Extrapulmonary sarcoidosis
Including:
 skin disease: about 20-25% of patients
are involved lesions include papules,
plaques, nodules and lupus pernio,
erythema nodosum
 eye disease and neurologic disease, liver
and spleen, peripheral lymph nodes, bone
lesions and myopathy
Experimental examinations and some
specific examinations
 elevations in
the level of angiotensinconverting enzyme(ACE) (the normal
level is 17.6-34u/ml). The measurement of
serum ACE might be a useful diagnostic
and prognostic test in sarcoidosis
 hypercalcemia: a potentially important
complication of sarcoidosis
 PPD
test: about 2/3 patients with sarcoidosis
has no reaction
 Kveim antigen test: we can’t usually used
the test we have not standard antigen
 bronchial-alveolar lavage fluid
examination(BALF): the lymphocyte
percent of the BALF is elevated. Usually, the
percent of lymphocyte of BALF is more than
28%.It demonstrates that the disease is
active
 tissue
biopsy:
 an affected organ or tissue are generally used
to diagnostic biopsy, including skin, lymphy
node,ects.
 Flexible electric bronchoscopy with
transbronchial lung biopsy(TBLB)
 Mediastinoscopy is sometimes performed in the
presence of isolated mediastinal adenopathy
X-ray examination
 The
plain chest radiography is an
important way to diagnose sarcoidosis.
 The major abnormalities seen on the
chest radiograph include:
lymphadenopathy,usually involving both
hila and mediastinal,and involvement of
the pulmonary parenchyma
 Computed
tomography(CT) of the chest is used
to evaluate of suspected sarcoidosis, especially
there is need for better definition of mediastinal
lymph node involvement.
 High-resolution CT is used to demonstrate that
pulmonary parenchymal involvement is
localized around bronchovascular structures,
producing an appearance resembling budding
branches on a tree.
 Usually, The
features of HRCT of
patient with sarcoidosis shows
numerous small nodular in a
predominantly bronchovascular
distribution
Scanning with gallium citrate-67 is a
more sensitive method to evaluate the
activity of sarcoidosis
Diagnosis and differential diagnosis

According to the clinical signs, chest X-ray,
tissue biopsy, we can diagnose the sarcoidosis.
 Nowadays the standard to diagnose the
sarcoidosis includes:
 chest X-ray shows that bilateral hilar and
mediastinal adenopathy, accompany with(or
without) small nodules in pulmonary
parenchymal
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Tissue biopsy shows that characteristic of
affected organ and tissue is conincide to
sarcoidosis
The Kveim test is positive
The PPD test is negative or weak positive
Hypercalcemia and hypercalciuia
The lymphocytes of BALF is elevated
The level of SACE is elevated
Among them, the first and the second are more
important. If a patient has both the first one
and the second one, we can diagnose
Differential diagnosis including

The tuberculosis of hilar lymphy node.
Usually, the patient with tuberculosis may
have clinical signs of tuberculosis.PPD test
is positive. Biopsy shoud be done if we
couldn’t diagnose
Differential diagnosis including

lymphoma.Usually we must do biopsy
 The transferred tumor of hilar:the patient with
transferred hilar adenopathy usually shows the
clinical signs caused by primary tumor. For
example, the chest X-ray of patient with lung
cancer sometimes shows not only pulmonary
parenchymal involvement but also hilar and
mediastinal adenopathy
Treatment

Most of patients, especially those with no
clinical signs need no therapy.
 If patients with clinical signs, the need to
therapy. For example, parenchymal lung
disease is a potential indication, depending on
its effects on pulmonary function and
symptoms, not on the severity of radiographic
involvement.Nowadays corticosteroids are also
a major treatment method. They can alter its
long-term natural history, improve the clinical
signs promptly, prevent progressive pulmonary
fibrosis

Usually prednisone is started at
40mg/d.The dose can be tapered with the
good of using lowest dose that keeps the
disease under adequate control.
 Treatment durations usually is one year
 Another drugs, including cytotoxic
agents(for example methotrexate),
hydroxychloroquine
Prognosis
Table 1
Radiographic staging of intrathoracic sarcoidosis
stage
hilar adenopathy parenchymal
disease
0
No
No
1
Yes
No
(both hilar and mediastinal right paratrachcal)
2
yes
yes(usually
shows small
nodules
interstitial
fibrosis)
3(or 4)
No
yes
(with fibrosis)

1.间质性肺病的肺功能特点为:A
 A.限制性通气障碍为主,弥散功能下降
 B阻塞性通气障碍为主, 弥散功能下降
 C限制性通气障碍为主, 弥散功能正常
 D阻塞性通气障碍为主, 弥散功能正常
 E限制性通气障碍为主, 弥散功能正常

2.诊断间质性肺病的金标准是:E
 A肺功能
 B临床表现
 C肺HRCT
 D支气管镜检查
 E肺活检病理

3.目前IPF最主要的治疗药物是:C
 A抗菌素
 B支气管扩张剂
 C糖皮质激素
 D止咳化痰药
 E呼吸兴奋剂

4.结节病的基本病理改变为:B
 A干酪样肉芽肿性病变
 B非干酪样肉芽肿性病变
 C纤维化样改变
 D以嗜酸性粒细胞浸润为主的炎症
 E以中性粒细胞浸润为主的炎症

5.结节病患者的支气管肺泡灌洗液细胞成
分分析中以何种细胞为主C
 A中性粒细胞
 B嗜酸性粒细胞
 C淋巴细胞
 D嗜碱性粒细胞
 E肥大细胞

6.下列哪项不符合弥漫性间质性肺疾病的
表现？E
 A、干咳
 B、气急
 C、杵状指
 D、X 线胸片呈弥漫性炎变
 E、肺功能呈阻塞性通气功能障碍
7．24 岁，一周来低热，乏力，胸痛，气促，体检：右
胸叩诊实音，呼吸音消失，胸水常规草黄色，比 重
1.030，WBC：0.8＊10^9/L，淋巴占 80％，蛋白 34g/L，
LDH：300u/dl，胸水 LDH/血清 LDH 比值为：0.75，
最可能的诊断是：E
 A、右侧肺炎伴胸水
 B、肺癌伴胸膜转移
 C、肝硬化伴胸水
 D、慢性肾功能不全伴胸水
 E、结核性胸膜炎

8．24 岁，一周来低热，乏力，胸痛，气促，体检：右
胸叩诊实音，呼吸音消失，胸水常规草黄色，比 重
1.030，WBC：0.8＊10^9/L，淋巴占 80％，蛋白 34g/L，
LDH：300u/dl，胸水 LDH/血清 LDH 比值为：0.75，
最可能的诊断是：E
 A、右侧肺炎伴胸水
 B、肺癌伴胸膜转移
 C、肝硬化伴胸水
 D、慢性肾功能不全伴胸水
 E、结核性胸膜炎

9．45 岁男性，1 年前不明原因活动后气促，逐渐加重，
体检：轻度发绀，肺底部少量细湿罗音，有杵状 指，
胸片示双肺中下野肺纹理增多呈网状，并有结节状阴
影，血气分析：PaO2:60mmHg，PaCO2:35mmHg， 以
下哪项诊断可能性最大？D
 A、慢性支气管炎
 B、支气管哮喘
 C、支气管扩张
 D、弥漫性肺间质纤维化
 E、支气管肺癌
