Pulmonary Eosinophilia Treatment & Management

Updated: Dec 31, 2015
  • Author: Jussi J Saukkonen, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Treatment

Medical Care

Give general supportive care by treating hypoxemia with supplemental oxygen. Treat bronchospasm with inhaled or nebulized bronchodilators. Inhaled corticosteroids may also be used when appropriate for persistent wheezing. Administer systemic steroids judiciously because they may worsen some infections.

  • Extrinsic syndromes

    • Medication-induced syndromes: These respond to the withdrawal of the offending agents, with few, if any, residual effects.

    • Loeffler syndrome: Remove any potentially offending medications or ingested substances. Loeffler syndrome is mild and self-limited. Patients rarely require systemic corticosteroids.

    • AEP: Patients with AEP respond rapidly to high doses of systemic corticosteroids and do not tend to relapse.

    • Parasitic infections: Once a diagnosis of parasitic infection is established, initiate therapy with appropriate antibiotics. Patients who are immunocompromised or patients taking systemic steroids with strongyloidiasis may develop hyperinfection syndrome, [21] often associated with gram-negative septicemia and adult respiratory distress syndrome. They may require empiric antibiotic coverage and respiratory and hemodynamic support.

    • Fungal causes: For allergic bronchopulmonary aspergillosis (ABPA), administer systemic steroids and inhaled bronchodilators. Short-term itraconazole may be used in the treatment of ABPA, as data suggest that it causes reductions in inflammatory markers and may have steroid-sparing effects. For coccidioidomycosis, the use of steroids early in infection may result in dissemination and death.

  • Intrinsic syndromes

    • Chronic eosinophilic pneumonia (CEP): Patients with CEP respond rapidly to prednisone at a dose of 30-40 mg/d, with significant symptom improvement occurring within 48 hours and radiographic clearing occurring within 10 days. Relapse is common if steroids are discontinued in the first 6 months of therapy. Continue therapy with lower doses of prednisone for several additional months. Patients rarely require permanent steroid therapy.

    • Idiopathic hypereosinophilic syndrome (IHES): Half the patients respond to corticosteroids, but others require more aggressive therapy with busulfan, cyclophosphamide, cyclosporin-A, etoposide, azathioprine, hydroxyurea, vincristine, or interferon alfa. Imatinib is used in myeloproliferative IHES.

    • Churg-Strauss syndrome (CSS): Prednisone administered at a dose of 40-60 mg/d for several weeks, followed by lower-dose therapy for a total of 1 year, generally provides efficacious therapy. High doses of intravenous methylprednisolone, cyclophosphamide, and azathioprine have been used to treat patients whose conditions are refractory. In refractory cases, high-dose intravenous immunoglobulin has been reported to be helpful. [22] Interferon-alpha and tumor necrosis factor inhibitors, such as infliximab and etanercept, have also been used. [23]

    • Eosinophilic granuloma (EG): Smoking cessation is essential. Corticosteroids are generally not beneficial.

Also see the following clinical guideline summaries:

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Surgical Care

Surgical intervention is rarely necessary for patients with these syndromes. The need for open lung biopsy is rare (see Procedures).

  • Parasitic diseases

    • Echinococcal cystectomy or lung resection is the preferred treatment for this disease. Right hepatic echinococcal cysts may be removed during the echinococcal cystectomy. Often, adjuvant medical therapy is administered.

    • Dirofilariasis may be difficult to distinguish from malignancy and may require transthoracic needle aspiration or resection.

  • Malignancy

    • Lung resection may be necessary if a solitary pulmonary nodule is present or if malignancy is in the forefront diagnostically.

    • Mediastinoscopy may be necessary if lymphadenopathy, possibly representing lymphoma, is significant.

  • Pneumothorax with obstructive lung disease

    • This may require tube thoracostomy.

    • If recurrent, such as with EG, pleurodesis may be required.

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Consultations

Often, the complexity of these patients' presentations and treatment warrants subspecialty consultation. This may facilitate diagnosis and treatment.

  • Pulmonologists provide expertise in the diagnostic approach, optimize the management of respiratory symptoms, may perform flexible bronchoscopy when indicated, and assist in the long-term management of the disease.

  • Infectious disease specialists provide diagnostic and therapeutic expertise and assistance, especially with unusual pathogens.

  • Radiologists who have good knowledge of chest radiography and tomography findings can be invaluable. A radiologist may be needed in the rare instance that a radiologically guided biopsy is warranted.

  • Consult a thoracic surgeon when an open lung biopsy is indicated.

  • Consult a hematologist for management of IHES.

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Diet

Patients who have ingested food, supplements, or medication to which the syndrome is attributed should subsequently avoid reexposure.

  • Chemically related medications should be avoided.

  • Measures should be taken to avoid ingestion of contaminated water or food.

  • Patients should avoid incompletely cooked seafood and crustaceans.

  • Patients should avoid incompletely cooked pork.

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Activity

Discourage the patient from engaging in activities linked to the syndrome or encourage the patient to take measures to avoid recurrence of the syndrome.

  • Patients should avoid contaminated water or soil known to harbor parasites or fungi.

  • Patients should avoid close contact with animals known to harbor parasites until these animals have been decontaminated.

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