A Challenging Clinical Case
Holger Link, MD
Liz Levine, M.D. PGY 2
Doernbecher Pediatric Pulmonary Program
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
In ED SpO2 82’%. Tx with Duoneb,
Dexamethasone, and transferred to DCH PICU.
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.
Fam Hx: No asthma, eczema, allergies. No other
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
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
Continued to improved while on tapering
course of Prednisone, just about back to
F/up in Pulm clinic 3 weeks later: doing well,
excellent PFT's (FEV1 of 124%), and his
azithromycin was discontinued
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
Cough worsens, increasing SOB, wheezing,
Re-admitted to hospital 2 months after initial
VS: Temp 36.8 °C BP 112/69, HR 98, RR 24, SpO2 94% Wt
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
Initially required 2-5 L O2 via NC to maintain sat’s.
Albuterol every 2-4 hrs was helping some.
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.
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
On discharge also started on ICS
Pulmonary Eosinophilic Syndromes
Acute eosinophilic PNA Asthma
Chronic eosinophilic PNA ABPA
Collagen vascular diseases
Eosinophilic granuloma Drug/toxin reaction
Infection: parasite (Loeffler’s
syndrome), helminthic, TB,
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.
Based on history, imaging, eosinophilia
1) Resp symptoms >2 weeks
2) Alveolar eosinophilia (>25%, esp >40%) or
blood eosinophilia (>1000 eos/mm3)
3) Pulmonary infiltrates on CXR, usually
4) Exclusion of other causes of eosinophilic
Unknown initiating stimulus/insult results in
accumulation of eosinophils in the lungs
TH2 lymphocytes activatedIL-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
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%
Gradual , subacute onset of symptoms which can
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
On exam, non specific findings
Expiratory wheezing in 50%
Occasionally inspiratory crackles
Peripheral eosinophilia usually found first
90% patients had >30% eosinophils
Elevated inflammatory markers
1/3 no abnormality, 1/3 restrictive, 1/3 obstructive
BAL fluid: >25% eosinophils
Mean close to 60%
“Photographic negative of pulmonary edema”
(though <25% with this finding)
Bilateral peripheral opacities, often in upper
Infiltrates can range from ground-glass,
migratory to dense consolidation
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
Spontaneous remission in 10%
Oral corticosteroids: prednisone 0.3-1
Usually have dramatic response with cessation of
Marked decrease in peripheral eosinophilia (and
Recommended course: gradual taper
between 6-12 months, though some suggest
trying 6-8 weeks
>50% relapse, requiring prolonged courses of CS
Aside from potential for relapse after cessation of
steroids, general prognosis is quite good with full
resolution of symptoms and no long-term
Individual reports of pulmonary fibrosis
Rare cases have been associated with cutaneous
T cell lymphoma, sarcoidosis, Churg-strauss
“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
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.
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.