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A Slice of PIE

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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 ... – PowerPoint PPT presentation

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Title: A Slice of PIE


1
A Slice of PIE
  • Neal Waechter, MD

2
Disclosure and Objectives
  • No financial support
  • Present case
  • Discuss approach to case
  • Discuss outcome of case

3
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

4
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

5
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

6
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

7
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

8
  • 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

9
  • 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

10
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

11
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

12
Case
  • CXR
  • (IMAGE)

13
What next?
  • What are the likely possibilities?
  • What can we not miss?

14
Initial Thoughts Cant Miss
  • Atypical infectious pneumonias
  • Fungal
  • TB/mycobacterial
  • Collagen Vascular Diseases
  • Vasculitis (esp. Churg-Strauss)
  • Cancer
  • Venous Thromboembolism and other embolic disease

15
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 gt1.030, 2-5 wbc, 0-1 rbc, neg dip
  • One sputum for AFB (difficulty producing adequate
    specimen) pending

16
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17
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

18
Differential Diagnosis
  • Airway Disorders
  • Asthma
  • CF
  • Pulmonary infections
  • TB
  • Other mycobacteria
  • Fungi
  • Parasites
  • Opportunistic organisms
  • Cancer

19
Differential Diagnosis
  • Pulmonary vascular disorders
  • Pulmonary embolism/infarction
  • Vasculitis and Pulmonary Renal Syndromes
  • Wegeners
  • Goodpastures
  • Churg-Strauss
  • Environmental/Occupational Lung disease
  • Hypersensitivity pneumonitis

20
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)

21
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, doesnt 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 yesterdays blood

22
On to something
  • Diff
  • 6950 neutrophils
  • 3210 lymphs
  • 40 basophils
  • 302 monocytes
  • 2180 eosinophils

23
PIE
  • Pulmonary Infiltrates with Eosinophilia (PIE)
  • Infections
  • Helminths
  • Lofflers 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

24
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

25
Coming to a diagnosis
  • Churg-Strauss Vasculitis
  • (Allergic granulomatosis and angiitis)
  • Eosinophilic, small arterial and venous
    vasculitis
  • Asthma in gt95 of cases, usually severe requiring
    chronic corticosteroids
  • Multiple organ involvement (mononeuritis in gt70,
    skin rash in majority, eosinophilic
    gastroenteritis in majority)
  • P-ANCA positive in gt70
  • 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

26
Coming to a diagnosis
  • Idiopathic Hypereosinophilic syndromes
  • Rare, multi-organ progressive syndromes with high
    morbidity
  • Chronic peripheral eosinophilia, gt1500 for gt6
    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

27
Coming to a diagnosis
  • Acute eosionphilic pneumonia
  • Less than 7 days of illness at presentation
  • Hypoxemic respiratory failure is common (gt50 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

28
Coming to a diagnosis
  • Idiopathic Chronic Eosinophilic Pneumonia
  • (AKA Carringtons 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

29
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

30
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 lt5 develop BOOP with pulmonary
    fibrosis, even fewer with clinically significant
    fibrosis

31
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)

32
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33
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.

34
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