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A Slice of PIE
Neal Waechter, MD
Disclosure and Objectives
 No financial support
 Present case
 Discuss approach to case
 Discuss outcome of case
Case
30 year old woman with chronic cough
HPI:
 10 weeks ago: first “asthma exacerbation” (mild exercise-induced
asthma for years), reports to urgent care (Visit #1)
 Symptoms:
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Cough
Moderate to severe dyspnea
Fever 101
Fatigue/malaise
 Treatment:
 nebulizer
 Advair inhaler
Case
HPI
 8 weeks ago: Return to urgent care still feeling
ill (visit #2)
 Symptoms
 still febrile (101)
 still dyspneic despite using Advair as prescribed
 cough now productive of green, sometimes dark brown
sputum
 Treatment
 Amoxacillin x 10 days
Case
HPI
 5 weeks ago: Return to urgent care with
same complaints (Visit #3)
 Symptoms
 Improved very slightly after amoxacillin, but
promptly returned to previous levels
 Persistent fever, productive cough, dyspnea
 Treatment
 Azithromycin x 5 days
Case
HPI
 1.5 weeks ago: Return to urgent care with persistent
symptoms (Visit #4) and new chest pain
 Symptoms
 Unchanged fever, cough, dyspnea, no help from azithromycin
 New onset of sharp left-sided pleuritic chest pain, thought she
broke a rib
 Diagnostic tests
 CXR – “patchy airspace disease in RUL, suspicious for
pneumonia”
 Treatment
 Augmentin 875 BID x 10 days
Case
HPI
 1 week ago: Follow-up with PCP (Visit #5)
 Symptoms: unchanged
 Exam:
 Temp 100.2
 Diffuse wheezing
 Treatment
 Continue antibiotics
 Resume Advair
 Follow-up CXR in one week
HPI
 Current Visit: Follow-up abnormal CXR
 Symptoms:
 Still intermittent fever up to 101
 Chest pain has largely resolved
 Dyspnea, productive cough continue w/o
hemoptysis
ROS
 Negative leg pain, h/o DVT/PE (VQ
performed 2 years ago during pregnancy for
chest pain was negative), arthralgia, rash,
dysuria, GI symptoms
 Positive for mild myalgias, occasional
headaches
Case
PMH
 Mild intermittent/exercise-induced asthma, long history
 Allergic rhinitis
 Migraine
 Depression
SH
 Non-smoker
 One child age one, currently breastfeeding
 Work – case manager and social worker in Geriatrics, currently not working
Exposure History
 No known exposure to TB, last PPD April 2002, negative
 No birds, exotic pets
 No recent travel
FH:
 Mother had DVT when bedridden with acute viral hepatitis
 GM had DVT, unknown risk factor
Allergies: Cephalexin
Meds: Albuterol, Pirbuterol, Advair
Case
Exam
 230 pounds, BP 110/80, HR 76, T 96.7
 Appeared comfortable, no resp distress
 Decreased breath sounds upper right posterior
lung field, egophony
 Normal percussion and tactile fremitus
 No wheezes or rales
 No clubbing or cyanosis
 Normal ENT, lymph node, cardiovascular,
abdominal, musculoskeletal, skin
Case
CXR
(IMAGE)
What next?
 What are the likely possibilities?
 What can we not miss?
Initial Thoughts – “Can’t Miss”
 Atypical infectious pneumonias
 Fungal
 TB/mycobacterial
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Collagen Vascular Diseases
Vasculitis (esp. Churg-Strauss)
Cancer
Venous Thromboembolism and other
embolic disease
Initial Plan
Diagnostics
 CBC: WBC 12.7, Hgb 13.3, Plt 315
 ESR: 50
 CRP: 2
 Chem: Cr 0.7, ALT 26
 UA: Sp gr >1.030, 2-5 wbc, 0-1 rbc, neg dip
 One sputum for AFB (difficulty producing
adequate specimen) pending
Case Summary So Far
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History of mild intermittent asthma
Chronic Cough
Dyspnea
Intermittent fever
Leukocytosis
Persistent pulmonary infiltrates on CXR
Multiple areas of airspace disease on CT,
upper lobe/peripheral predominance
Differential Diagnosis
 Airway Disorders
 Asthma
 CF
 Pulmonary infections
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TB
Other mycobacteria
Fungi
Parasites
Opportunistic organisms
 Cancer
Differential Diagnosis
 Pulmonary vascular disorders
 Pulmonary embolism/infarction
 Vasculitis and Pulmonary Renal Syndromes
 Wegener’s
 Goodpasture’s
 Churg-Strauss
 Environmental/Occupational Lung
disease
 Hypersensitivity pneumonitis
Differential Diagnosis
 Interstitial Lung Diseases
 Idiopathic Fibrosing Interstitial Pneumonias
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UIP (IPF)
RB-ILD (DIP)
AIP
NSIP
BOOP
Sarcoidosis
Collagen Vascular Diseases
Amyloidosis
Pulmonary Alveolar proteinosis
Pulmonary Infiltrates with Eosinophilia (PIE)
“Light bulb”
 Recall cases of eosinophilic pulmonary syndromes
from residency with similar presentation
 No sig exposure to TB, no evidence of PE, cancer, on
CT, no occupational exposures, no sig travel, doesn’t
really fit other diagnoses on list
 Patient has a history of asthma
 Elevated WBC, but no diff – could this be eosinophilia?
Plan: Add differential to yesterday’s blood
On to something…
 Diff:
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6950 neutrophils
3210 lymphs
40 basophils
302 monocytes
 2180 eosinophils
PIE
 Pulmonary Infiltrates with Eosinophilia (PIE)
 Infections
 Helminths
 Loffler’s syndrome (Ascaris, hookworm, strongyloides)
 Non life cycle pulmonary invasion (paragonimiasis,others)
 Tropical pulmonary eosinophilia (Wucheria)
 Sometimes, Coccidiomycosis
 Rarely, TB
 Medications/crack cocaine
 NSAIDS/Salicylates
 Minocycline
 Trimethoprim/sulfamethoxazole
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ABPA
Churg-Strauss
Idiopathic Hypereosinophilic syndromes
Idiopathic eosinophilic pneumonia
 Acute eosinophilic pneumonia
 Chronic eosinophilic pneumonia
Coming to a diagnosis
ABPA
 Typically a sino-pulmonary syndrome with prominent sinus
symptoms
 Must have skin prick test or serum IGE/IGG positive for
Aspergillus
 Typical CT finding is widespread proximal bronchiectasis with
upper lobe predominance, mucus plugging, and patchy
infiltrates/atelectasis
In this case…
 Not entirely ruled out – did not do skin test or serum antibody tests
 No sinus disease symptoms/signs
 CT findings not characteristic (does not exclude diagnosis)
Possible…
Coming to a diagnosis
Churg-Strauss Vasculitis
(Allergic granulomatosis and angiitis)
 Eosinophilic, small arterial and venous vasculitis
 Asthma in >95% of cases, usually severe requiring chronic corticosteroids
 Multiple organ involvement (mononeuritis in >70%, skin rash in majority,
eosinophilic gastroenteritis in majority)
 P-ANCA positive in >70%
 CT may show enlarged peripheral pulmonary arteries, fleeting patchy infiltrates,
pulmonary nodules, pulmonary hemorrhage, pleural effusions
 Pleural effusions are eosinophilic, exudative
 Gold standard for diagnosis is open lung biopsy
In this case…
 P-ANCA and C-ANCA are negative
 CT findings are not characteristic
 Asthma is not severe enough, and there does not appear to be involvement of
other organs
REJECTED
Coming to a diagnosis
Idiopathic Hypereosinophilic syndromes
 Rare, multi-organ progressive syndromes with high
morbidity
 Chronic peripheral eosinophilia, >1500 for >6 months
 No identifiable cause (helminths, etc)
 Significant organ involvement (not benign eosinophilia)
In this case…
 Disease is limited to lungs
 Relatively benign course
 Only ~ 2 months of symptoms
REJECTED
Coming to a diagnosis
Acute eosionphilic pneumonia
 Less than 7 days of illness at presentation
 Hypoxemic respiratory failure is common (>50% of patients)
 Peripheral eosionphilia may be a late finding, but BAL fluid and
lung tissue/pleural fluid are highly eosinophilic
 Radiographic findings are diffuse, patchy infiltrates without a
pattern
In this case…
 Symptoms have been present for too long
 Respiratory symptoms are fairly mild
 CT findings are not characteristic - in this patient they are
peripheral, not random
REJECTED
Coming to a diagnosis
Idiopathic Chronic Eosinophilic Pneumonia
(AKA Carrington’s Disease)
 Twice as common in women as in men
 Pre-existent asthma in majority, not necessarily severe
 No association with cigarettes
 Syndrome Characterized by
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Respiratory and systemic symptoms including fever
Absence of extrathoracic organ involvement
Alveolar and peripheral eosionophilia in nearly all
Elevated inflammatory markers in most
Elevated serum total IGE levels in majority
Pulmonary infiltrates, usually peripheral on X-ray (“photographic negative” of pulmonary
edema). While not specific enough to be pathognomonic, this pattern is rare in other
diseases.
In this case…
 A good match
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patient demographic
symptoms
lab findings
x-ray findings
Idiopathic Chronic
Eosinophilic Pneumonia
 Treatment
 Little research evidence
 Could not find randomized controlled trials
 Few prospective case series
 Several review articles offering expert opinion
 Expert consensus: uniformly responsive to
corticosteroids
 Prednisone, 40-60mg/day standard initial therapy
 Gradual taper over 6-12 months
 Unknown role for inhaled corticosteroids
Idiopathic Chronic
Eosinophilic Pneumonia
 Outcome
 Nearly complete remission of symptoms
expected within a few days of treatment
 Relapses are the rule as steroids are
tapered
 Perhaps half will require long-term
corticosteroids for symptoms
 Benign course: <5% develop BOOP with
pulmonary fibrosis, even fewer with clinically
significant fibrosis
Back to the case
 Patient referred to Pulmonary Clinic once diagnosis
became clear
 Prednisone was initiated at 60mg/day, tapered to
15mg/day over 3 weeks
 The Advair was continued
 She felt much better within a few days. Fever
completely resolved.
 CXR improved by 7 days
 CXR cleared at two months
 As of 11/4, she has some residual cough and chest
tightness, with albuterol rescue 2 - 4 times per week
(vast improvement but worse than before the onset of
this illness)
Bibliography
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Up to Date
Current Medical Diagnosis and Treatment, 2004
Robbins Pathologic Basis of Disease
Marchand, E et al. “Idiopathic Eosinophilic Pneumonia.
A Clinical and Follow-up Study of 62 cases.” Medicine.
1998; 77: 299-312
 Marchand, E et al. “ICEP and Asthma. How Do They
Influence Each Other?” Eur Respir J. 2003; 22: 8-13
 Marchand, E et al. “Idiopathic Chronic Eosinophilic
Pneumonia.” Orphanet Encyclopedia, updated June
2004.