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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:
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
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
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
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
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:
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
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
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
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
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.