eMedicine Specialties > Pulmonology > Eosinophilic Lung Diseases

Pulmonary Eosinophilia: Follow-up

Author: Jussi J Saukkonen, MD, Associate Professor, Department of Internal Medicine, Division of Pulmonary/Critical Care Medicine, Boston University School of Medicine, Boston Medical Center
Contributor Information and Disclosures

Updated: Nov 20, 2009

Follow-up

Further Inpatient Care

• With extrinsic diseases, the priority is to establish the diagnosis, assure adequate oxygenation, and provide bronchodilator therapy, with or without steroids as indicated.
  • 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.
  • Chronic eosinophilic pneumonia (CEP): CT scan 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.
  • Churg-Strauss syndrome (CSS): If significant end organ damage results from vasculitis and if the damage is rapidly advancing, the urgency of treatment may be increased.
  • Idiopathic hypereosinophilic syndrome (IHES): Patients tend to respond slowly, and only 50% respond to steroids. Patients may develop deep venous thrombosis and pulmonary emboli.

Further Outpatient Care

• For extrinsic diseases, maintain clinical and radiologic follow-up with patients. Monitor oxygen saturation.
  • 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.
  • CEP: This tends to recur if treatment is stopped early. Initially, determining if the syndrome is CEP alone or an early manifestation of IHES is difficult.
  • CSS: Long-term treatment is necessary. If end organ damage is progressive from vasculitis, patients may require additional treatment beyond systemic corticosteroids.
  • IHES: 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.

Inpatient & Outpatient Medications

• Extrinsic diseases
  • AEP: Administer intravenous methylprednisolone in an inpatient setting, followed by taper to oral form
  • Fungal diseases
    • ABPA: Albuterol and/or other bronchodilators, inhaled corticosteroids, and systemic steroids, when needed, may be administered. If the patient is unresponsive to these medications, see Asthma for further options.
    • Coccidioidomycosis: Treatment is usually not necessary. For patients with progressive or disseminated disease, use amphotericin B intravenously, followed by fluconazole.
  • Parasitic diseases: If the patient has significant bronchospasm, provide bronchodilators and inhaled or systemic steroids judiciously.
    • Ascaris infections - Albendazole or mebendazole
    • Hookworms (Necator species, Ancylostoma species) - Albendazole
    • TPE - Ivermectin or diethylcarbamazine
    • Strongyloides infections - Ivermectin or thiabendazole
    • Toxocariasis (visceral larva migrans) - Ivermectin or albendazole
    • Trichinella infections - Albendazole or mebendazole, may need steroids
    • C sinensis infections – Praziquantel
    • Paragonimus infections - Praziquantel
    • Schistosoma infections – Praziquantel
    • Echinococcus infections - Surgery and/or albendazole
• Intrinsic diseases
  • CEP: Administer prednisone, as described above (see Medication).
  • CSS: Administer prednisone. Other agents include azathioprine and cyclophosphamide.
  • IHES: The initial treatment should be with prednisone in both inpatient and outpatient settings. Assess the response to therapy over weeks to months, unless the disease is rapidly progressive (see Medical Care and Medication). Imatinib has been used more recently with promising results in subsets of patients.

Transfer

• Rapidly progressive disease
  • Severe respiratory failure may require aggressive ventilatory management.
  • Treatment may be imperative while the diagnostic workup is in progress.
• Diagnostic expertise of specialists
  • These individuals may help with diagnostic reasoning and can provide knowledge from experience.
  • They can also provide procedural support.

Deterrence/Prevention

• Extrinsic diseases
  • 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 or fish.
  • Medications: Avoid ingestion/inhalation of same or same class of medications.
• Intrinsic diseases: Use recommended treatment courses. Closely monitor patients for relapse.

Complications

• Extrinsic diseases
  • AEP: Respiratory failure may occur.
  • Fungal diseases
    • ABPA: Complications may include respiratory failure, bronchiectasis, hemoptysis, aspergilloma, and/or complications of steroids.
    • Coccidioidomycosis: Respiratory failure may occur in patients with progressive or disseminated disease. Patients who are immunocompromised may present with disseminated disease or persistent primary coccidioidomycosis; both are associated with significant morbidity and mortality. Patients who are older may develop a chronic illness resembling reactivation tuberculosis.
  • Parasitic diseases - Respiratory failure, hemoptysis, and/or pneumonia
    • TPE: If TPE is left untreated for more than 6 months, it commonly leads to interstitial pulmonary fibrosis and restrictive defects.
    • Strongyloidiasis: Severe disseminated infection (hyperinfection) may occur in individuals who are immunocompromised because this nematode can replicate within humans.
    • Schistosomiasis: This results in eosinophilia and pulmonary nodules in early infection because the schistosomulas migrate through the lung. Later, granuloma formation and pulmonary arterial occlusion with chronic pulmonary hypertension are caused by embolization of ova.
• Intrinsic diseases
  • CEP: Fibrosis may develop if patients are left untreated or if the disease is extensive. If CEP is left unrecognized and untreated, it can progress, resulting in significant gas exchange abnormalities.
  • IHES: Patients with IHES may develop congestive heart failure, pulmonary emboli, and multiorgan system dysfunction.
  • CSS: Renal failure, pulmonary fibrosis, and neuropathy may develop. The mortality rate in cases of CSS has been decreasing, with approximately 75% of patients surviving 5 years.
  • EG: The course of EG is highly variable. Patients at age extremes, those with multiorgan or skin involvement, and those with pneumothoraces tend to have a poor prognosis. Diabetes insipidus may develop from pituitary involvement, and pneumothorax may develop from cystic lung disease.

Prognosis

• Extrinsic diseases
  • AEP: Patients often develop respiratory failure, but, with treatment and supportive measures, they generally survive.
  • Medication-induced and Loeffler syndrome: Removal of the offending agent usually results in a resolution of symptoms.
  • Parasitic diseases: These are usually successfully treated but may require a repeated course of therapy.
  • Hyperinfection syndrome in strongyloidiasis: Patients may be critically ill with sepsis and respiratory failure.
  • TPE: Patients may develop fibrosis with symptomatic pulmonary restriction.
  • ABPA: Patients usually have lifelong symptoms with intermittent exacerbations.
  • Coccidioidomycosis: This usually resolves spontaneously.
• Intrinsic diseases
  • CEP: Patients have a rapid response to therapy but may develop relapse within 6 months. Some patients who initially present with only pulmonary involvement actually have IHES.
  • IHES: Half the patients respond to steroids, while patients who do not respond go on to have significant disease requiring increasingly complex regimens. Now, 80% of patients survive 5 years, and 40% survive 10-15 years.
  • CSS: Patients generally respond well to steroids, but they require lifelong therapy.

Patient Education

• See Deterrence/Prevention.
• Patients should be educated about the symptoms of relapse for all diseases discussed.
• For excellent patient education resources, visit eMedicine's Procedures Center. Also, see eMedicine's patient education article Bronchoscopy.

Miscellaneous

Medicolegal Pitfalls

• These diseases may challenge a diagnostician. Failure to diagnose an infectious illness may lead to the death of the individual, particularly if the person is immunocompromised, leaving the clinician potentially liable.
• Failure to diagnose a vasculitis may result in significant morbidity, including renal failure.
• Many clinicians advocate discussing all the potential toxicities of medications used, and failure to do so may leave the clinician liable.

• Search for CME/CE on This Topic »

• Filariasis (Infectious Diseases)
• Eosinophilia (Hematology)
• Filariasis (Pediatrics: General Medicine)
• Bancroftian Filariasis (Pediatrics: General Medicine)

• Bronchoscopy Introduction
• Procedures Center
• Bronchoscopy Preparation