A Challenging Clinical Case
Holger Link, MD
Liz Levine, M.D. PGY 2
Doernbecher Pediatric Pulmonary Program
Disclosures
 None
HPI
 16 yo teenage boy presented with worsening
wheezing, coughing, shortness of breath.
 1 mo PTA:
 Fever, coughing, dx with walking PNA, treated with
Azithromycin for 5 days with some improvement
 Cough gradually worsened with acute onset of
SOB, wheezing.
 In ED SpO2 82’%. Tx with Duoneb,
Dexamethasone, and transferred to DCH PICU.
Further history:
 PMHx: Term infant, no hospitalizations prior to those
mentioned. No surgeries. No atopy or hx of asthma. 2
episodes of PNA 2 years ago, both tx as an outpatient.
Fully vaccinated.
 Fam Hx: No asthma, eczema, allergies. No other
respiratory problems.
 Social: Junior in HS, quite active usually, plays football.
Smokes MJ “few times per month” over past 2 yrs, denies
all other drug use, including tobacco. Has been sexually
active in past, not in last 8-9 mo.
 Travel: none out of Oregon in last 2 years
 Animals/Exposures: Denies inhaled dust, smoke,
chemicals, no pets, birds, farm, or other animal exposure
 Meds: Albuterol MDI prn
 Allergies: NKA
PICU
 Needing oygen, continuous nebs, mag sulfate,
IV steroids, bronchodilators gradually weaned,
transferred to floor after 2 days.
 Pulm consult: recommended CT given unusual
presentation for asthma.
**Had received 2 days of IV steroids at time of CT
CXR day of admission
Inspiratory chest CT
Inspiratory chest CT
Expiratory chest CT
Expiratory chest CT
Expiratory chest CT
Clinical course + tests
 CT initial read: significant amount of air trapping, GG
opacities and concern for a potential bronchiolitis
obliterans w/cryptogenic pneumonia.
 Workup: AFB neg, Legionella neg, HIV neg, Resp Viral
panel neg, Hypersensitivity Pneumonitis panel neg,
Mycoplasma pneumoniae IgM neg, IgG positive.
 He improved from a respiratory standpoint.
 PFT’s with FEV1 102% day of discharge
 Discharged home with a tapering course of oral
prednisone, albuterol prn and azithromycin.
CBC + Diff
After discharge:
 Continued to improved while on tapering
course of Prednisone, just about back to
baseline
 F/up in Pulm clinic 3 weeks later: doing well,
excellent PFT's (FEV1 of 124%), and his
azithromycin was discontinued
However…
 After finishing course of steroids: increasing
cough, using albuterol more often, no other
cold-like sx. Also notes occasional night
sweats, weight loss of 15-20 lbs over past few
months.
 Cough worsens, increasing SOB, wheezing,
chest pain.
 Re-admitted to hospital 2 months after initial
PICU admission.
Physical exam
 VS: Temp 36.8 °C BP 112/69, HR 98, RR 24, SpO2 94% Wt
60.6 kg
 Gen: awake, alert, NAD
 HEENT, CV, Abd, Neuro: normal
 Resp: Non-labored, no retractions or nasal flaring,
currently on 0.5L. Chest shape is normal. Auscultation
of lungs: slight tachypnea, prolonged expiration with
wheezing (both insp and exp) in all lung fields.
 Ext: WWP, no clubbing.
 Skin: no rashes, several linear striae noted over lower
back.
On admission:
 Initially required 2-5 L O2 via NC to maintain sat’s.
Albuterol every 2-4 hrs was helping some.
 Labs:
 WBC: 22.8 (Poly 20, lymph 12, mono 4, Eos 64%)
 Lytes, LFT’s ,H/H, plt wnl
 ESR 25, CRP <0.5
 UDS: +Cannabinoids, all others negative
 Imaging: CXR: airway thickening, hyperinflation
 Pulmonology consult requested
CXR second admission
Bronchoscopy results + course
 Labs: IgE elevated at 867, rest of immunoglobulins wnl.
 Bronchoscopy:
 Mucosa: mild erythema
 Secretions: thick whitish-yellowish secretions in
bilateral airways. No blood.
 Eosinophilia of 91% on BAL
 Started on Solumedrol x 1 day, followed by Prednisone 60
mg daily
 On discharge also started on ICS
BAL findings
BAL histology
Pulmonary Eosinophilic Syndromes
Primary
Secondary
 Acute eosinophilic PNA
 Asthma
 Chronic eosinophilic PNA
 ABPA
 Churg-Strauss Syndrome
 Bronchocentric granulomatosis
 Hypereosinophilic
syndrome
 Collagen vascular diseases
 Eosinophilic granuloma
 Drug/toxin reaction
 Infection: parasite (Loeffler’s
syndrome), helminthic, TB,
coccidiodes, others
 BOOP
 Hypersensitivity pneumonitis
 Malignancies
Chronic Eosinophilic Pneumonia
 Rare disorder of unclear etiology resulting in
insidious onset of respiratory symptoms
accompanied by pulmonary consolidation and
accumulation of large numbers of eosinophils in
the lungs, blood and in BAL fluid.
Diagnosis
 Based on history, imaging, eosinophilia
 Proposed criteria:
1) Resp symptoms >2 weeks
2) Alveolar eosinophilia (>25%, esp >40%) or
blood eosinophilia (>1000 eos/mm3)
3) Pulmonary infiltrates on CXR, usually
peripheral
4) Exclusion of other causes of eosinophilic
lung disease
Etiology
 Unknown initiating stimulus/insult results in
accumulation of eosinophils in the lungs
 TH2 lymphocytes activatedIL-5, important role in
recruiting eosinophils, and inhibiting their apoptosis.
 Other chemokines involved: elevated TARC (thymus
and activation regulated chemokine), eotaxin and
RANTES act as other chemoattractants of eosinophils
Epidemiology:
 Female nonsmokers, mean age of 45 yrs
 Female/male ratio 2.1/1 in 1 review article
 Age range 18 to 80 yrs in same article
 Most patients do have asthma (52%),
atopy in general 63-75%
Clinical Presentation:
 Gradual , subacute onset of symptoms which can
include:
 Respiratory: cough (+/- productive), wheezing, SOB, chest pain
 Systemic: low grade fevers, weight loss, night sweats, malaise
 Acute respiratory failure, significant hypoxemia seen in
acute eosinophilic pneumonia
 Rarely, arthralgias, neuropathy, skin findings, GI sx
reported
Physical findings
 On exam, non specific findings
Expiratory wheezing in 50%
Occasionally inspiratory crackles
Labs
 Peripheral eosinophilia usually found first
 90% patients had >30% eosinophils
 Elevated inflammatory markers
 Elevated IgE
 PFT’s: inconsistent
 1/3 no abnormality, 1/3 restrictive, 1/3 obstructive
 BAL fluid: >25% eosinophils
 Mean close to 60%
 Normal <2%
Imaging: CXR
 “Photographic negative of pulmonary edema”
(though <25% with this finding)
 Bilateral peripheral opacities, often in upper
lobes
 Infiltrates can range from ground-glass,
migratory to dense consolidation
Imaging: CT
 May help detect small ground glass opacities
not seen on film
 Study of high resolution CT scan in 81 patients,
physicians unable to distinguish between CEP
and bronchiolitis obliterans
Treatment:
 Spontaneous remission in 10%
 Oral corticosteroids: prednisone 0.3-1
mg/kg/day
 Usually have dramatic response with cessation of
symptoms
 Marked decrease in peripheral eosinophilia (and
BAL fluid)
 Recommended course: gradual taper
between 6-12 months, though some suggest
trying 6-8 weeks
Prognosis:
 >50% relapse, requiring prolonged courses of CS
for months-years
 Aside from potential for relapse after cessation of
steroids, general prognosis is quite good with full
resolution of symptoms and no long-term
sequelae
 Individual reports of pulmonary fibrosis
 Rare cases have been associated with cutaneous
T cell lymphoma, sarcoidosis, Churg-strauss
Final twist…
 “Oil red O stain indicates a 95% positivity for
lipid laden macrophages. There are numerous
Charcot-Leyden eosinophilic crystals and a few
scattered benign goblet cells suggestive of an
asthma-like respiratory process.”
 No hx of reflux, choking, GERD symptoms
 After this obtained normal MBSS
 Stool O&P obtained, but unable to be processed
due to presence of barium
BAL histology
References:
 Alam, M. and N. Burki. Chronic eosinophilic pneumonia: a review.
Southern Medical Journal 2007, 100: 49-53.
 Cakir, C. et al. Chronic eosinophilic pneumonia with mucous plugs in
a child. Pediatric Pulmonology 2010, 45: 1040-1042.
 Katz, U. and Y. Shoeneld. Pulmonary eosinophilia. Clinic Rev Allergy
Immunol, 2008, 34:367-371.
 Uptodate
 Weschler, M. Pulmonary eosinophilic syndromes. Immonol Allergy
Clin N Am, 2007, 27: 477-492.
 Wubbel, C. et al. Chronic eosinophilic pneumonia: a case report and
national survey. Chest 2003, 123; 1763-1766.