IDIOPATHIC CHRONIC

EOSINOPHILIC
PNEUMONIA
‫المركز التخصصي لألمراض التنفسية في ذي قار‬
 INTRODUCTION
 The eosinophilic lung diseases are a group of disorders characterized
by the presence and presumed pathogenetic role of eosinophils,
 the eosinophilic pneumonias, defined by a prominent infiltration of
the lung parenchyma by eosinophils, which respond dramatically to
corticosteroid treatment and generally heal without significant
sequelae.
 The other eosinophilic lung diseases mainly involve the airways, as in
the ABPA, hypereosinophilic obliterative bronchiolitis and
eosinophilic endotype of asthma
 GENERAL FEATURES OF
EOSINOPHILIC PNEUMONIAS
 In clinical practice, eosinophilic pneumonias may be
separated into two main etiologic categories: (1) those in
which a definite cause is found or (2) those of undetermined
origin (idiopathic), with the eosinophilic pneumonia being
either solitary or included in a systemic disorder such as
EGPA
 eosinophilia
 Of importance, blood eosinophilia is defined by an eosinophil
blood count greater than 0.5 × 109/L (500/μL) and
hypereosinophila by an eosinophil blood count greater than
1.5 × 109/L (1500/μL) on two examinations at least 1 month
apart, and/or tissue hypereosinophilia.
 In clinical practice, the diagnosis of eosinophilic pneumonia
is suspected in patients presenting with respiratory
symptoms (dyspnea, cough, or wheezing), pulmonary
opacities on chest imaging, and the demonstration of
eosinophilia in the peripheral blood or, preferably, in the
lung.
 BAL is recommended to confirm the diagnosis of
eosinophilic pneumonia in most cases. BAL is a widely
accepted noninvasive surrogate of lung biopsy for the
diagnosis in a patient with HRCT features of eosinophilic
pneumonia.
 In normal subjects, BAL eosinophils comprise less than 1%
of cells
 IDIOPATHIC CHRONIC EOSINOPHILIC
PNEUMONIA (ICEP)
 It is the most frequent cause of eosinophilic pneumonia is Western
countries.
 ICEP is characterized by a progressive onset of symptoms over a few
weeks to few months with cough, increasing dyspnea, chest pain,
malaise, Fever, weight loss, asthenia, fatigue, anorexia, weakness, and
night sweats are also common.
 ICEP 2:1 female/male ratio.
 incidence is highest in the fourth decade rare before the age of 20 years.
 A vast majority of patients are nonsmokers.
 A prior history of atopy is found in about half of the patients,
 allergic rhinitis in 12% to 24%,
 drug allergy in about 10%,
 nasal polyps in 5–13%,
 urticaria in 10%, and eczema in 5%.
 Prior asthma is present at diagnosis of ICEP in 2/3 of the
patients, develops concomitantly with the diagnosis of ICEP
in 15% of patients, and can develop after ICEP in 13%.
 The presentation of ICEP is similar in asthmatic and
nonasthmatic patients, with the exception of higher total IgE
in asthmatics.
 Allergic desensitization is not considered to trigger ICEP.
 Asthma often gets worse after the development of ICEP and
requires long-term oral corticosteroid treatment even in the
absence of recurrence of the eosinophilic pneumonia
 Wheezes at physical examination are found in one-third and
crackles in 38%.
 Upper respiratory tract symptoms of chronic rhinitis or
sinusitis are present in about 20% of patients.
 IMAGING
 Peripheral opacities on chest radiography are present in almost all
cases
 migratory in a ¼ of the cases.
 They usually consist of alveolar opacities with ill-defined margins,
with a density varying from ground-glass to consolidation
 The classic pattern of “photographic negative is seen in only ¼ of
patients
 The radiological features of ICEP is characteristic although they
may overlap with COP
 the opacities are bilateral in at least 50% of cases at chest
radiography.
 bilateral opacities may increase up to 97.5% at HRCT.
 Characteristic opacities predominate in the upper lobes and are
peripheral, with generally coexisting ground-glass and
consolidation opacities.
 Midzone distribution of opacities and centrilobular nodules are
present in <20%
 Septal line thickening is common
 On corticosteroid treatment, consolidation and ground-glass
opacities rapidly show a decrease in size and extent, with
possible change from consolidation to ground-glass
opacities or inhomogeneous opacities and later to streaky or
bandlike opacities parallel to the chest wall.
 Contrary to organizing pneumonia, the reverse halo or atoll
sign (i.e., central ground-glass opacity surrounded by denser
consolidation in a crescentic circle or ring) is rare.
 Mediastinal lymph nodes are enlarged in 15–20%.
 Compared with IAEP, in which pleural effusions are seen in
the majority small pleural effusions are present in only 10%
of cases of ICEP at HRCT.
 LABORATORY STUDIES
 Peripheral blood eosinophilia is almost always present, with
the exception of cases where treatment has been initiated
before the test.
 blood eosinophils is generally >20%
 CRP level is elevated
Continue……
 Total blood IgE level is increased in about 50% and > 1000
IU/mL in 15%
 Fractional exhaled nitric oxide is increased in ICEP and has
been proposed as a way to monitor eosinophilic parenchymal
inflammation.
 Measurement of urinary excretion of eosinophil proteins and
leukotrienes has been proposed for monitoring disease
activity
 BRONCHOALVEOLAR LAVAGE
(BAL)
 is generally recommended for the diagnosis of ICEP.
 Alveolar eosinophilia is always present >25%
 A value greater that 40% is considered most consistent with
a confident diagnosis of ICEP in the appropriate clinical
setting.
 BAL eosinophil cell count drops within a few days under
corticosteroid treatment.
 Pulmonary function tests (PFTs)
 50% obstructive pattern and 50% restrictive pattern.
 PaO2 75 mm Hg or less was present in 64% of patients
 DlCO may be decreased due to restrictive physiology, but the
transfer coefficient (DlCO/unit alveolar volume) is impaired in only
one-fourth of cases.
 An increased alveolar-arterial oxygen tension difference has been
reported in 90%
 With treatment, PFTs normalize rapidly in most patients. But 1/3
develop a persistent obstructive ventilatory defect.
 TREATMENT AND PROGNOSIS
 Response to oral corticosteroids is dramatic and rapid, with
symptomatic improvement within 48 hours in about 80% of cases
 Pulmonary opacities on chest radiography clear rapidly within 1
week of treatment in the majority of patients.
 The initial dose of corticosteroids usually varies between 20 and 60
mg/day (0.5 mg/kg/ day ) which was then tapered and discontinued
at either 3 or 6 months
 relapse >50% but well respond to corticosteroid
 Omalizumab and mepolizumab may used to prevent relapses
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