PeripheralPulmonary Eosinophilia with pulmonary symptoms

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AM Report

• Feb 15, 2006

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HPI

• 38 yo white male previously healthy
• 6 months prior to admission gradual onset of dry
  cough, low grade fever, DOE, wheezing.
• Multiple visits to PCP, treated as asthma with
  inhalers, abx and steroid taper with rapid
  improvement, but relapse 1-2 weeks after
  finishing treatment.

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HPI

• Exposure to a flooded rental apartment, and
  pulled up a wet, stinky carpet. Immediately
  after he experienced bout of coughing and SOB.

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PMHx

• Glaucoma
• Mild seasonal allergies

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Meds

• Xalatan 0.005 eye drops
• Tylenol prn
• Albuterol MDI prn
• Advair 1 puff bid
• Completed course of Ketek/prednisone taper 2
  weeks ago

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ROS

• fatigue, intermittent low grade fevers, night
  sweats
• SOB, DOE, wheezing, dry cough
• No rhinitis/sinusitis
• No CP, orthopnea, PND
• No rash, skin lesions
• No myalgias/arthralgias
• No LAD
• No GI/GU/neuro complaints

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FHx SHx

• Father in his 60s with DM2, recent MI
• Mother HTN
• 2 Brothers healthy

• Linconton, NC with wife and 2 children
• Mechanic
• Non-smoker
• 5 years ago Salmon Fishing in Alaska
• Pulled up a wet carpet prior to initial
  presentation

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PE

• VS 37.9, 96, 24, 130/76, 87 RA
• Mild increased work of breathing.
• HEENT PERRL, no sinus drainage or nasal polyps,
  no mucosal ulcers
• CV RRR, S1, S2, PMI nondisplaced, no m/r/g. No
  JVD. Pulses 2.
• Lungs scattered crackles and wheezing
• Abdomen unremarkable
• Skin No rash

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Labs

• WBC 22.6 21 eosinophils, AEC 4.75
• Eosinophils decreased to 0 on steroids
• Chem 10, LFTs wnl
• CXR- diffuse/patchy alveolar opacity
• Chest CT- peripheral, upper lobe predominant,
  patchy pneumonitis

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Labs

• IgE 2473
• Expectorated sputum 40 eosinophils
• Stool O/P negative x4
• Sputum O/P, AFB, fungal, Cx negative
• Aspergillus Ab lt 18
• Strongyloides Ab negative
• Toxocara Canis Ab negative
• ANCA lt 116

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Labs

• PFTs FEV1 76 pred
• TTE EF gt 55, nl valves
• CXR cleared with treatment
• Bone Marrow Bx normocellular with normal marrow
  elements, no cytogenetic abnormalities
• Flow cytometry no evidence of monoclonality

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Idiopathic Chronic Eosinophilic Pneumonia
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DDx Blood Eosinophilia
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DDx of Pulmonary Eosinophilia

• Idiopathic
• Pulmonary
• Idiopathic chronic eosinophilic pneumonia (ICEP)
• Idiopathic acute eosinophilic pneumonia (IAEP)
• Pulmonary extrapulmonary
• Churg Strauss Syndrome (CSS)
• Hypereosinophilic syndromes (HES)
• Parasitic infections
• Wuchereria bancrofti, Ascaris, Toxocara Canis,
  Strongyloides
• ABPA and other fungi/yeast

• Drugs/Toxins
• Aspirin, Captopril, Naproxen, Penicillin,
  Ethambutol, GM-CSF, Ibuprofen, Bactrim,
  L-tryptophan, crack/heroin, dust
• Radiation to the Chest
• Miscellaneous lung disease with lesser degree of
  eosinophilia lt25
• Sarcoidosis
• Asthma and eosinophilic bronchitis
• Lung Transplantation
• Langerhans Cell Granulomatosis
• Paraneoplastic Eosinophilic Pneumonia
• Acute eosinophilic leukemia, lymphoma

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Eosinophilic Pneumonia

• Pulmonary symptoms
• Infiltrate on CXR/CT
• Pulmonary eosinophilia BAL gt25

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Acute Eosinophilic Pneumonia

• Acute onset of EP
• Often quite severe meeting criteria for ALI or
  ARDS and requiring intubation.
• Rapid response to steroids
• No relapse after recovery
• Often associated with exposure 9/11 firefighter,
  tear gas, tobacco smoke

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Churg Strauss Syndrome

• Small vessel vasculitis associated with
  asthma/rhinitis and eosinophilia
• Dx asthma eosinophilia 2 or more
  extrapulmonary organs(skin,GI, cardiac, neuro),
  frequently ANCA
• Tx Corticosteroids
• Cytotoxic agents for poor prognostic factors.

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Hypereosinophilic Syndrome-HES

• Lymphocytic Variant
• Clonal TH2 lyphocytes overproducing IL-5
• Cutaneous papules or urticarial plaques
  infiltrated with eosinophils/lymphocytes
• Tx corticosteroids
• anti- IL5

• Myeloproliferative Variant
• 4q12 deletion causing Fip1L1-PDGFRa fusion
  protein
• Cardiac involvement
• endomyocardial fibrosis/mural thrombus
• HSM, anemia, thrombocytopenia, mucosal ulcers
• Acute eosinophilic Leukemia
• Tx corticosteroids
• Gleevec

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Idiopathic Chronic Eosinophilic Pneumonia

• gradual and progressive onset of EP without
  documented etiology.
• Lack of extrapulmonary disease.

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Epidemiology

• Non-smoker
• 21 female to male
• Asthma in 50 prior to Dx
• Mean age at Dx 45

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Clinical Presentation

• Pulmonary/systemic symptoms- non-productive
  cough, dyspnea, wheezing, fatigue, malaise, fever
• Gradual/progressive onset over weeks
• Lack of extrapulmonary symptoms
• Lung exam wheezes/crackles

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Diagnosis

• BAL eosinophilia gt 25 but usually gt40, mean 58
  
• Peripheral blood eosinophilia AEC gt 1000
• Typically gt 6 of WBC, mean 32
• ESR, CRP, IgE often elevated
• Infiltrate on CXR/CT
• Lung biopsy rarely indicated

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Imaging

• CXR- peripheral predominance of alveolar
  infiltrates classically described as the
  photographic negative of pulmonary edema
• CT- bilateral, diffuse, patchy, upper lobe
  predominant with coexisting ground glass and
  consolidation
• May see small pleural effusion, mediastinal LAD
• Opacities resolve rapidly with corticosteroids

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Treatment

• Uniformly responsive to Corticosteroids
• Prednisone 1 mg/kg tapered 2 week after
  symptom resolution, then 0.5 mg/kg x 8 weeks,
  then decrease 5 mg q 4 weeks.
• Symptoms improve within 48 hours to 1 week
• CXR opacities clear within 1 week
• Relapse is common 50-80
• Frequently require years of steroids (mean 1.9
  years)
• Inhaled steroids may decrease relapse rate
• 2nd Line cytotoxic agents, anti-IL5

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Natural history

• May resolve spontaneously 10
• The majority resolve after 6 months to years of
  corticosteroids.
• Relapse not indicative of worse prognosis or
  increased morbidity.
• Some require lifelong corticosteroids.
• Occasionally the intial manifestation of CSS,
  prior to the vasculitic phase.
• Rarely can result in physiologically detectable
  fibrosis.

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References

• Cottin, V. Cordier, J.-F. (2005) Eosinophilic
  pneumonias. Allergy 60 (7), 841-857.
• Jederlinic, PJ, Sicilian, L, Gaensler, EA.
  Chronic eosinophilic pneumonia. A report of 19
  cases and a review of the literature. Medicine
  1988 67154.
• Naughton, M, Fahy, J, FitzGerald, MX. Chronic
  eosinophilic pneumonia. A longterm followup of 12
  patients. Chest 1993 103162.

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