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Last updated: 10/24/2023
Years published: 1989, 2000, 2012, 2016, 2019, 2023
NORD gratefully acknowledges Evans Fernรกndez Pรฉrez, MD, MS, Division of Pulmonary, Critical Care and Sleep Medicine, National Jewish Health, for assistance in the preparation of this report.
Summary
Simple pulmonary eosinophilia (SPE), also known as Loeffler syndrome, is a rare, temporary (transient) respiratory disorder characterized by the accumulation of eosinophils in the lungs (pulmonary eosinophilia). Eosinophils are a type of white blood cell and are part of the immune system. They are usually produced in response to allergens, inflammation or infection and are particularly active in the respiratory tract. Most cases of SPE are thought to be due to an allergic reaction to drugs or infection (mainly parasites). SPE usually ranges in severity from no symptoms to development of mild respiratory symptoms. In rare cases, more significant complications can occur. Generally, no specific therapy is required as symptoms usually go away spontaneously without treatment.
Introduction
Simple pulmonary eosinophilia was first described in the medical literature in 1932. It is classified as a form of eosinophilic lung disease. SPE is considered a benign, self-limiting disorder.
SPE usually presents as a mild lung disorder characterized by a dry, unproductive cough, wheezing and a slight fever. Some affected individuals may cough up a mixture of saliva and mucus (sputum). Symptoms usually resolve on their own without treatment (spontaneous resolution) within two weeks to a month. In some people, symptoms can persist for months, especially if the person is exposed to the allergen again.
Additional symptoms can occur including chest pain, wheezing, shortness of breath (dyspnea) and a rapid breathing rate. Some individuals may complain of a general feeling of poor health (malaise). Inflammation of the mucous membranes of the nose (rhinitis), unintended weight loss and night sweats have also been reported.
Some individuals with SPE develop acute eosinophilic pneumonia (AEP). AEP is a rare, serious lung disorder that can quickly progress to cause acute respiratory failure. (For more information on AEP, see the related disorders section below).
Generally, SPE is caused by an allergic reaction. A variety of factors including parasitic infection, exposure to certain drugs or exposure to certain fungi have been linked to SPE. In some people with SPE, the triggering event or cause of SPE is unknown (idiopathic). The exact reason why there is an overproduction and accumulation of eosinophils in the lungs in individuals with SPE is not fully understood.
The passage of parasitic larvae through the lungs causes most cases, which results in an allergic reaction. Parasitic worms (helminths) such as nematodes are the most common parasitic cause associated with SPE. The term nematode is a classification (i.e., phylum) of worms characterized by long, round, generally smooth bodies. Nematodes that have been linked to SPE include hookworms and Ascaris lumbricoides, a type of roundworm.
Drugs that have been linked to cases of SPE include nonsteroidal anti-inflammatory drugs (NSAIDs), certain antibiotics, anti-microbialsand anti-seizure medications (anticonvulsants).
Some cases of SPE have been caused by exposure to certain fungi such as Aspergillus fumigatus.
SPE affects males and females in equal numbers. The exact incidence and prevalence of SPE in the general population is unknown. It is the most common form of eosinophilic lung disease. SPE can affect individuals of any age.
A diagnosis of SPE is based upon identification of characteristic symptoms (e.g., eosinophilic pneumonia), a detailed patient history, a thorough clinical evaluation, the absence of other known causes of eosinophilic lung disease and a variety of specialized tests. A physical examination may reveal wheezing and/or a rattling sound in the lungs (rales). Distinguishing SPE from other, more severe forms of pulmonary eosinophilia is especially important when obtaining a diagnosis.
Clinical Testing and Work-Up
Imaging techniques may be used to help confirm a diagnosis of SPE including chest x-ray or computerized tomography (CT) scanning. Chest x-rays in individuals with SPE generally show white patches or shadows (infiltrates) in the lungs. These infiltrates may disappear but can reappear in different areas of the lungs. A CT scan may reveal hazy areas (ground-glass opacities) that are not seen on traditional x-rays. During CT scanning, a computer and x-rays are used to create a film showing cross-sectional images of tissue structures such as the lungs. A CT scan can also show airspace consolidation, in which the tiny air sacs of the lungs (alveolar) become abnormally filled (as with eosinophils).
A procedure known as bronchoalveolar lavage (BAL) may also be used to help obtain a diagnosis of SPE. During BAL, a narrow tube (bronchoscope) is slid down the windpipe into the lungs and a sterile solution is passed through the tube washing out (lavaging) cells. This fluid is collected and then the tube is removed, allowing the cells to be studied. BAL in individuals with SPE reveals abnormally high levels of eosinophils.
Blood tests may reveal elevated levels of eosinophils (i.e., eosinophilia) and/or serum immunoglobulin E (IgE) and may coincide with pulmonary manifestations. A stool examination may reveal the presence of parasites. Analysis of sputum or fluid obtained from pumping the stomach (gastric lavage) may reveal parasitic larvae.
Additional tests may be performed to rule out other causes of pulmonary eosinophilia.
Treatment
In most patients, no treatment is required and SPE goes away on its own (spontaneous remission). Cases due to active parasitic infection should be treated with appropriate anti-parasitic medications. Drug-induced cases should be treated by stopping the suspected offending drug. Respiratory symptoms such as wheeze and cough may be managed with inhaled bronchodilators. In rare cases, after active infection has been ruled out, corticosteroid therapy may be required and is generally effective.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
TEXTBOOKS
Cordier JF, Cottin V. Eosinophilic Pneumonias. In: Interstitial Lung Disease, 5th ed. Schwarz MI, King Jr. TE, eds. Peopleโs Medical Publishing House, Shelton, CT. 2011:833-893
Cottin V. Idiopathic eosinophilic pneumonias. In: European Respiratory Monograph: Clinical Handbooks for the Respiratory Professional. Orphan Lung Diseases. Cordier JF, ed. European Respiratory Society, United Kingdom. 2011:118-139.
JOURNAL ARTICLES
Sergew A, Fernรกndez Pรฉrez ER. Current Approach to Diagnosis and Management of Pulmonary Eosinophilic Syndromes: Eosinophilic Pneumonias, Eosinophilic Granulomatosis with Polyangiitis, and Hypereosinophilic Syndrome. Semin Respir Crit Care Med. 2016; 37(3):441-56. https://www.ncbi.nlm.nih.gov/pubmed/27231866
Fernรกndez Pรฉrez ER, Olson AL, Frankel SK. Eosinophilic lung diseases. Med Clin North Am. 2011; 95:1163-87. https://www.ncbi.nlm.nih.gov/pubmed/22032433
Campos LE, Pereira LF. Pulmonary eosinophilia. J Bras Pneumol. 2009; 35:561-573. https://www.ncbi.nlm.nih.gov/pubmed/19618037
Jeong YJ, Kim KL, Seo IJ, et al. Eosinophilic lung diseases: a clinical, radiologic, and pathologic overview. Radiographics. 2007; 27:617-637. https://www.ncbi.nlm.nih.gov/pubmed/17495282
INTERNET
Sharma GD, Vinikoor MJ. Loffler syndrome. Medscape. Last Update Updated:April 14, 2022. Available at: https://emedicine.medscape.com/article/1002606-overview Accessed Oct 10, 2023.
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