Epidemiology
- Sex: 2-fold more common in females
- Age: peak incidence in 30’s (but can occur at any age)
- More chronic clinical presentation (>14 days of symptoms) than [[Acute Eosinophilic Pneumonia]]
- Associated With:
- Asthma/Atopic Diseases: pre-existing atopic disease (especially asthma) is present in 50% of cases
- IBD: ulcerative colitis
Physiology
- Unclear
Pathology
Pathologic Patterns
- Eosinophilic Pneumonia: interstitial and alveolar eosinophil infiltration
- Macrophages and multinucleated giant cells (giant cells may contain eosinophil fragments and crystals, which may be early Charcot-Leyden crystals)
- Large Charcot-Leyden crystals and features of BO and vasculitis may also be seen in some cases
- Usual Interstitial Pneumonia (UIP): may be seen in some cases
- BOOP/Organizing Pneumonia Features/Cellular Interstitial Pneumonia (CIP): seen in some cases
- Honeycombing: seen late
Diagnosis
CBC
- Eosinophilia (absent in about 15% of cases): mean peripheral eosinophil count is around 26%
- Neutrophilia and anemia (usually)
Sputum
- Eosinophilia
ABG
- Hypoxemia
PFT’s
- Obstructive Defect/Hyperinflation: may be seen
FOB
- BAL: eosinophilia >40% (normal <2%) is highly suggestive of eosinophilic pneumonia (BAL eosinophilia is nearly always seen, even when peripheral eosinophilia is absent)
- TBB: may be diagnostic
OLB
- Not needed in most cases (but may be useful to rule out BOOP)
CXR/Chest CT Patterns
- Alveolar infiltrates:
-“Reverse Pulmonary Edema” (”Photographic Negative of Pulmonary Edema”) With Upper Lobe-Predominant Peripheral Infiltrates: occurs in 66% of cases- Infiltrates maay be fleeting and may not conform to lobar or segmental boundaries
- Hilar adenopathy may be seen
- Similar pattern may be seen in BOOP
- Interstitial infiltrates: may be superimposed on alveolar infiltrates later in course
Serum IgE
- Normal-mildly elevated
Circulating Immune Complexes
- Positive
Clinical
(generally subacute illness that may persist for weeks-months in untreated cases/some cases present with acute respiratory failure)
- Wheezing: reported in some cases (generally recent onset)
- Cough: dry or mucoid sputum
- Dyspnea:
- Weight loss:
- Night sweats:
- Hemoptysis: occasional
- Fever:
- Lymphadenopathy:
- Hepatomegaly:
Treatment
- Steroids: rapid resolution of symptoms and infiltrates (although mean latency to clinical response is 18 days)
- Prednisone 30-60 mg/day will result in CXR improvement within 3 days (clearing within 3 weeks)
- Taper steroids, follow for relapse (may occur)
- Mean Duration of Therapy: 5 years
Prognosis
- Long-term prognosis is good
References
- xxx