Chronic eosinophilic pneumonia (CEP) is an interstitial lung disorder of unknown etiology (often termed idiopathic CEP in the literature) that is characterized by a profound deposition of eosinophils in the lungs and the subsequent development of symptoms [1] [2] [3] [4] [5]. CEP is most frequently diagnosed in adults around 50 years of age with a significant predilection toward female gender [1]. Although a clear association has not been described, a large number of individuals suffer from asthma or some other form of atopy (such as allergic rhinitis) [1] [2] [3] [4]. Additionally, isolated studies have identified radiation therapy for breast cancer as a possible risk factor [6]. The clinical presentation is distinguished by a cough, wheezing, and progressive dyspnea as main respiratory complaints that may be accompanied by constitutional symptoms - weight loss, fever, and night sweats [1] [3] [6] [7]. In rare cases, massive eosinophilic infiltration can lead to respiratory insufficiency, but most patients suffer from a milder form of the disease [1] [3]. CEP usually has a slow course, and several weeks might pass before the diagnosis is made [1] [2].

• Mediastinal Lymphadenopathy

  A 38-year-old man was hospitalized in our university hospital because of pulmonary opacities with bilateral hilar and mediastinal lymphadenopathy seen on chest radiograph. [ncbi.nlm.nih.gov]

• Fever

  An 81-year-old Japanese male with primary Sjögren syndrome (pSS) developed a low-grade fever and productive cough which were refractory to antibiotic therapy. [ncbi.nlm.nih.gov]

• Weight Loss

  A 43-year-old male with 30 years history of exposure to isocyanates was admitted with the complaint of sputum, cough, progressive dyspnoea, and weight loss. Physical examination revealed bilaterally decreased breath sounds and extensive rales. [ncbi.nlm.nih.gov]

• Malaise

  Our present patient, a 55-year-old woman, had classic symptoms of dry cough, weight loss, malaise, dyspnea, night sweats, and fevers. Significant peripheral blood eosinophilia and a right upper lobe infiltrate were present. [ncbi.nlm.nih.gov]

  The illnesses lasted between six and 20 weeks and consisted of cough, dyspnoea, malaise, and in two cases prolonged pyrexia. [thorax.bmj.com]

  He was admitted to a local hospital for 2 weeks because of shortness of breath, dry cough, malaise, anorexia, chills, fever, and leukocytosis. There was no previous history of smoking or asthma. [cid.oxfordjournals.org]

  […] identical to Loeffler’s Chronic Eosinophilic Pneumonia X-ray  Similar to Loeffler’s except infiltrates last for many days or week without steroids Chronic Eosinophilic Pneumonia Clinical  Most are asymptomatic or mild symptoms  Some have  High fever  Malaise [learningradiology.com]

  Pathological Features Alveoli flooded with eosinophils and macrophages Bronchiolitis obliterans in one-third Granulomas absent Clinical Presentation Cause unknown Typically middle aged women 50% have history asthma Cough Significant weight loss High fever Malaise [chestx-ray.com]

• Chills

  He was admitted to a local hospital for 2 weeks because of shortness of breath, dry cough, malaise, anorexia, chills, fever, and leukocytosis. There was no previous history of smoking or asthma. [cid.oxfordjournals.org]

  He denied fever, but had night sweats and chills. He did not have any significant cough or sputum production. Just prior to admission, he developed a maculopapular rash on his trunk and legs. [thoracic.org]

  One month prior to his admission he developed a low grade, intermittent fever with temperature ranging between 99 0 and 101 0 F, night sweats but no chills or rigors. [lungindia.com]

  The most common symptoms are non-productive cough, dyspnea, and fever though malaise, night sweats, chills, myalgias, and pleuritic chest pain are possible as well. [clinicaladvisor.com]

  Fever, chills, dyspnea, leukocytosis may occur 4-6 hours after exposure and eventually resolve; symptoms and signs may recur on re-exposure. CXR: Acute - normal to reticulonodular pattern; Chronic - progressive fibrosis, honeycombing. [lumen.luc.edu]

• Recurrent Urinary Tract Infection

  The authors report the case of a female patient who developed chronic eosinophilic pneumonia secondary to long-term use of nitrofurantoin for prophylaxis of recurrent urinary tract infections due to urethral stenosis. [ncbi.nlm.nih.gov]

• Cough

  Dyspnoea and cough disappeared within one week and chest CT scan showed regression of the lung infiltrates within one month. No relapse occurred during the following nine months. [ncbi.nlm.nih.gov]

• Dyspnea

  Chronic eosinophilic pneumonia (CEP) is a rare interstitial lung disease characterized by subacute dyspnea, peripheral infiltrates on imaging, and pulmonary eosinophilia. [ncbi.nlm.nih.gov]

• Pleural Effusion

  Pleural effusions are rarely seen. We report a case of chronic eosinophilic pneumonia with transudative eosinophilic pleural effusion. [ncbi.nlm.nih.gov]

  Chest X-ray showed a small right pleural effusion and chest CT showed small micronodular bilateral infiltrates with right pleural thickening. He had a leukocytosis of 12.5 cells/mm3, with 8% eosinophils. [aspergillus.org.uk]

• Dry Cough

  Our present patient, a 55-year-old woman, had classic symptoms of dry cough, weight loss, malaise, dyspnea, night sweats, and fevers. Significant peripheral blood eosinophilia and a right upper lobe infiltrate were present. [ncbi.nlm.nih.gov]

  In addition, it made diagnosis more difficult that dry cough was the only symptom without fever or asthma history. [jtd.amegroups.com]

  Her symptoms were associated with a dry cough, occasional wheezing, and heaviness in her chest. She also complained of sinus pressure and congestion. She had never smoked and was not known to have any respiratory or rheumatologic disease. [rc.rcjournal.com]

  He was admitted to a local hospital for 2 weeks because of shortness of breath, dry cough, malaise, anorexia, chills, fever, and leukocytosis. There was no previous history of smoking or asthma. [cid.oxfordjournals.org]

• Rales

  Physical examination revealed bilaterally decreased breath sounds and extensive rales. [ncbi.nlm.nih.gov]

  Physical exam findings include hypoxemia, fever, tachypnea, non-productive cough, and bilateral diffuse rales. If pleural effusions are present, there is decreased tactile fremitus and increased dullness to percussion. [clinicaladvisor.com]

• Chronic Diarrhea

  Association of chronic eosinophilic pneumonia and chronic diarrhea with arsenicosis is rare. Also pulmonary cavity formation in chronic eosinophilic pneumonia is very uncommon. [ncbi.nlm.nih.gov]

• Parotid Swelling

  Abstract A case of a woman presenting with a parotid swelling is described. She had chronic eosinophilic pneumonia (CEP) for approximately 2 years prior to presentation. [ncbi.nlm.nih.gov]

• Neglect

  The diagnosis of CEP should not be neglected in the classification of the eosinophilic pneumonias with increased serum IgE levels. [ncbi.nlm.nih.gov]

A thorough workup is necessary in the case of CEP, encompassing clinical, radiologic, and possibly histopathologic studies. They are all necessary for narrowing the broad differential diagnosis, which includes pulmonary infections (both parasitic and fungal), iatrogenic causes, neoplastic processes, autoimmune vasculitis (Churg-Strauss syndrome), and several other entities that induce pulmonary eosinophilia [1] [2] [7]. Obtaining a complete patient history that will identify basic characteristics of symptoms and their duration is the first step, followed by a detailed personal history. A meticulous physical examination should follow, although lung auscultation may not yield any pathological findings. For this reason, laboratory and imaging studies are the cornerstones in making an initial diagnosis. Detection of peripheral eosinophilia (often exceeding ≥ 1000/mm3) in blood, but also in the bronchioalveolar aspirate (obtained through a bronchioalveolar lavage, or BAL) is a valid diagnostic clue, whereas serum inflammatory markers (erythrocyte sedimentation rate and C-reactive protein), but also immunoglobulin E levels, are often elevated [1] [2] [3]. Conversely, the presence of bilateral infiltrates and ground-glass opacities at the peripheries is typical for CEP, which can be better visualized on computed tomography (CT) compared to standard X-rays of the chest [1] [2] [3] [4] [7]. Although not necessary and now rarely used for confirmation of CEP, bronchoscopy and subsequent biopsy with histopathological examination is useful for excluding other disorders, as the accumulation of eosinophils in the alveoli and the interstitium is the hallmark of CEP [1] [2]. Pulmonary function tests are not always abnormal, but several studies have identified a reduced ability of the lungs to transfer oxygen into the blood, known as the diffusing capacity of the lung for carbon monoxide (DLCO) [2] [4].

• Pulmonary Infiltrate

  A 38-year-old woman presented with worsening cough, blood eosinophilia, and pulmonary infiltrates. Bronchoalveolar lavage showed 96.4% eosinophils. [ncbi.nlm.nih.gov]

• Bilateral Pulmonary Infiltrates

  Peripheral blood eosinophilia, multiple bilateral pulmonary infiltrates to the x-ray, multiple nodules with a surrounding ground-glass halo and peripheral predominance to the chest CT suggested the diagnosis of eosinophilic lung disease (ELD). [ncbi.nlm.nih.gov]

  Chest radiograph showed bilateral pulmonary infiltrates. His physical deterioration progressed after cardiac recompensation. Pulmonary infiltrates resolved and presenting symptoms disappeared during steroid treatment. [vestnik.szd.si]

  However, soon after completion of his steroid taper, he became progressively more short of breath, and medical evaluation revealed worsened hypoxia and bilateral pulmonary infiltrates. [cid.oxfordjournals.org]

  pulmonary infiltrates. 29 The patient improved, and the eosinophilia disappeared within 18 days of stopping the rifampicin. [pharmaceutical-journal.com]

• X-Ray Abnormal

  Complete resolution of symptoms and x-ray abnormalities occurs within 14 days in most patients and by 1 mo in almost all. Symptoms and plain chest x-rays are both reliable and efficient guides to therapy. [merckmanuals.com]

  Another distinguishing feature of acute eosinophilic pneumonia is the complete recovery of both symptoms and x-ray abnormalities without recurrence or residual sequelae, within weeks of treatment. [apfed.org]

  Complete resolution of symptoms and x-ray abnormalities occurs within 14 days in most patients and by 1 month in almost all. Symptoms and plain chest x-rays are both reliable and efficient guides to therapy. [msdmanuals.com]

• Chest X-Ray Abnormal

  Table 1 Diagnostic criteria of Acute Eosinophilic Pneumonia Acute febrile illness with respiratory manifestations of Hypoxemic respiratory failure (inability to breathe, low oxygen levels) Diffuse pulmonary infiltrates on chest x-ray (abnormal x-ray) [apfed.org]

• Left Pleural Effusion

  Leukocytosis was evident with marked eosinophilia (65.5% 46,000/mm3), and the chest roentgenogram showed diffuse reticular shadows throughout both lung fields and a left pleural effusion. [ncbi.nlm.nih.gov]

• Eosinophils Increased

  The number of eosinophils increases during many inflammatory and allergic reactions, including asthma, which frequently accompanies certain types of eosinophilic pneumonia. [merckmanuals.com]

• Lymphocytic Infiltrate

  Histopathological findings revealed extreme eosinophil, plasma cell, and lymphocytic infiltration and interstitial fibrosis. These conjunctival specimen findings were similar to those seen in the alveolar lesions in CEP. [ncbi.nlm.nih.gov]

• Pleural Effusion

  Pleural effusions are rarely seen. We report a case of chronic eosinophilic pneumonia with transudative eosinophilic pleural effusion. [ncbi.nlm.nih.gov]

  Chest X-ray showed a small right pleural effusion and chest CT showed small micronodular bilateral infiltrates with right pleural thickening. He had a leukocytosis of 12.5 cells/mm3, with 8% eosinophils. [aspergillus.org.uk]

One patient had worsening of CEP after 2 months of treatment, and another had relapse of CEP at 3.5 years while receiving 1.6 mg/day of BDP. All patients thus finally had worsening or relapse of CEP during treatment with BDP. [ncbi.nlm.nih.gov]

Abstract Objective The long-term clinical course and prognosis of patients with chronic eosinophilic pneumonia (CEP) including factors predictive of the relapse of CEP have not been fully investigated. [ncbi.nlm.nih.gov]

Chronic eosinophilic pneumonia is one form of eosinophilic lung disease that includes both idiopathic and known etiological cases. [ncbi.nlm.nih.gov]

We review the epidemiology, clinical, diagnosis, and therapy of the CEP. [file.scirp.org]

Chronic eosinophilic pneumonia in Iceland: clinical features, epidemiology and review. Laeknabladid 2007;93:111–6. PubMed Google Scholar 57. Thomeer MJ, Costabel U, Rizzato G, et al. [link.springer.com]

Long-term use of prednisone has many side effects, including increased infections, osteoporosis, stomach ulcers, Cushing's syndrome, and changes in appearance. [6] Epidemiology [ edit ] Eosinophilic pneumonia is a rare disease. [en.wikipedia.org]

Epidemiology Frequency United States Intrinsic syndromes are uncommon. Regarding extrinsic syndromes, medication- or food-related syndromes are sporadic. [emedicine.medscape.com]

The pathophysiological role of eosinophils in autoimmune diseases is not well defined, however it has been shown that the production of pro-inflammatory cytokines stimulate and activates different cell groups, and can simultaneously induce autoantibodies [ncbi.nlm.nih.gov]

The pathophysiological role of eosinophils in autoimmune diseases is not well defined; however, it has been shown that the production of pro-inflammatory cytokines stimulates and activates different cell groups, and can simultaneously induce autoantibodies [reumatologiaclinica.org]

Clinical, pathophysiologic, and therapeutic considerations. Ann Intern Med. 1982 Jul. 97(1):78-92. [Medline]. Jederlinic PJ, Sicilian L, Gaensler EA. Chronic eosinophilic pneumonia. A report of 19 cases and a review of the literature. [emedicine.medscape.com]

Pathophysiology [ edit ] Eosinophilic pneumonia can develop in several different ways depending on the underlying cause of the disease. Eosinophils play a central role in defending the body against infection by parasites. [en.wikipedia.org]

These data suggest that the long-term prognosis for patients with CEP is excellent but the majority will require long-term low-dose oral corticosteroid therapy in order to prevent relapse. [ncbi.nlm.nih.gov]

Treatment and prognosis The long-term prognosis is considered excellent but the majority often will require long-term low-dose oral corticosteroid therapy in order to prevent relapse 7-8. [radiopaedia.org]

The present study provides further evidence for a possible role of inhaled corticosteroids in preventing relapses of ICEP. [erj.ersjournals.com]

The following are some preventive measures to avoid Eosinophilic Pneumonia: Decreasing exposure to risk factors that increase the chances of getting the diseases (Example: the use of certain medications) Smoking cessation There is also a pneumococcal [dovemed.com]

Treatment with intravenous (IV) steroids or other medications which suppress the immune system may help stop or decrease the inflammation and prevent respiratory failure. [nationaljewish.org]

1. Kolb AG, Ives ST, Davies SF. Diagnosis in Just Over a Minute: a Case of Chronic Eosinophilic Pneumonia. Journal of General Internal Medicine. 2013;28(7):972-975.
2. Alam M, Burki NK. Chronic eosinophilic pneumonia: a review. South Med J. 2007;100(1):49–53.
3. Marchand E, Cordier J-F. Idiopathic chronic eosinophilic pneumonia. Orphanet J Rare Dis. 2006;1:11.
4. Yalcin F, Sak ZH, Boyaci N, Gencer M. A chronic eosinophilic pneumonia case with long exposure to isocyanates. J Pak Med Assoc. 2014;64(10):1191-1194.
5. Jaimes-Hernández J, Mendoza-Fuentes A, Meléndez-Mercado CI, Aranda-Pereira P. Chronic eosinophilic pneumonia: autoimmune phenomenon or immunoallergic disease? Case report and literature review. Reumatol Clin. 2012;8(3):145-148.
6. Cottin V, Frognier R, Monnot H, Levy A, DeVuyst P, Cordier JF. Chronic eosinophilic pneumonia after radiation therapy for breast cancer. Eur Respir J. 2004;23:9–13.
7. Blanc S, Albertini M, Leroy S, Giovannini-Chami L. Chronic eosinophilic pneumonia with persistent decreased diffusing capacity for carbon monoxide. BMJ Case Rep. 2013. doi:10.1136/bcr-2012-008238.