Pulmonary Eosinophilia Treatment & Management

Updated: Dec 10, 2020
  • Author: Jussi J Saukkonen, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Treatment

Approach Considerations

With extrinsic diseases, the priority is to establish the diagnosis, assure adequate oxygenation, and provide bronchodilator therapy, with or without steroids as indicated. Note the following:

  • Acute eosinophilic pneumonia (AEP): Patients may deteriorate rapidly to respiratory failure and require mechanical ventilation, but they generally respond well to high-dose steroids.

  • Fungal infections: For allergic bronchopulmonary aspergillosis (ABPA), assure clinical improvement. For coccidioidomycosis, patients who are immunocompromised may develop disseminated infection if steroids are administered.

  • Parasitic diseases: Patients may develop a Mazzotti reaction, in which the death of parasites releases new antigens, with resultant fever, urticaria, pruritus, bronchospasm, and associated gastrointestinal symptoms. Patients who are immunocompromised and have strongyloidiasis may develop hyperinfection syndrome with use of steroids (see Medication).

With intrinsic syndromes, make aggressive attempts to exclude infections. Note the following:

  • Chronic eosinophilic pneumonia (CEP): CT scanning and bronchoscopy are generally performed. If no infection is present, the patient is treated with prednisone. Rapid clinical and radiologic improvement occurs within 24-72 hours.

  • Eosinophilic granulomatosis with polyangiitis (EGPA): If significant end organ damage results from vasculitis and if the damage is rapidly advancing, the urgency of treatment may be increased.

  • Hypereosinophilic syndrome (HES): Patients tend to respond slowly, and only 50% respond to steroids. Patients may develop deep venous thrombosis and pulmonary emboli.

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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.

Acute eosinophilic pneumonia (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, [29] 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.

Hypereosinophilic syndrome (HES): Half the patients respond to corticosteroids. Other agents for treatment of HES include hydroxyurea, interferon α, and imatinib. [24] Numerous additional agents, including chlorambucil, vincristine, etoposide, cladribine, cytarabine, methotrexate, cyclosporine, alemtuzumab, and cyclophosphamide, have been used to treat steroid-unresponsive patients with varied success and may be appropriate in some situations. [30]

Eosinophilic granulomatosis with polyangiitis (EGPA): 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. [31] Interferon-alpha and tumor necrosis factor inhibitors, such as infliximab and etanercept, have also been used. [32]

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

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Surgical Care

Surgical intervention is rarely indicated in the treatment of pulmonary eosinophilia. Note the following:

  • 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 eosinophilic granuloma (EG), pleurodesis may be required.

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Consultations

Often, the complexity of these patients' presentations and treatment warrants consultation. For example, severe respiratory failure may require aggressive ventilatory management and treatment may be imperative while the diagnostic workup is in progress. The following specialists can provide assistance with diagnostic reasoning as well as procedural support:

  • 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 hypereosinophilic syndrome (HES).

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Prevention

Encourage the patient to take measures to minimize risk of exposure linked to the syndrome. Patients who have ingested food, supplements, or medication to which the syndrome is attributed should subsequently avoid reexposure. Note the following:

  • Fungal diseases: Avoid contact/inhalation of contaminated soil, dust, or water.

  • Parasitic diseases: Avoid contact with contaminated soil, sand, water, and animals. Avoid ingestion of undercooked meat, pork, and fish.

  • Medications: Avoid ingestion/inhalation of same or same class of medications.

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Long-Term Monitoring

For extrinsic diseases, maintain clinical and radiologic follow-up with patients. Monitor oxygen saturation. Note the following:

  • Allergic bronchopulmonary aspergillosis (ABPA): Patients tend to relapse after treatment. Monitor symptoms, peak flow, spirometry, IgE levels, and oxygen saturation. Patients may develop complications of bronchiectasis (ie, infections, hemoptysis, aspergilloma).

  • Parasitic diseases: Occasionally, repeated treatment courses may be needed. For tropical pulmonary eosinophilia (TPE), provide symptomatic relief with bronchodilators and systemic and/or inhaled steroids. Patients often develop fibrosis. Monitor spirometry and lung volumes. For schistosomiasis, patients may develop pulmonary hypertension and cor pulmonale, requiring supplemental oxygen, diuretics, and supportive measures.

Intrinsic diseases are marked by a tendency to recur. Monitor oxygenation at rest and with exertion, perform pulmonary function tests as necessary, and perform radiologic evaluations. Note the following:

  • Chronic eosinophilic pneumonia (CEP): This tends to recur if treatment is stopped early. Initially, determining if the syndrome is CEP alone or an early manifestation of hypereosinophilic syndrome (HES) is difficult.

  • Eosinophilic granulomatosis with polyangiitis (EGPA): Long-term treatment is necessary. If end organ damage is progressive from vasculitis, patients may require additional treatment beyond systemic corticosteroids.

  • HES: Various agents have been used to treat those with disease that is unresponsive to steroids (see Medication). Allogeneic bone marrow transplant has been used.

  • Eosinophilic granuloma (EG): These patients may develop symptoms of diabetes insipidus from infiltration of the pituitary gland. Smoking cessation is essential.

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