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]

  […] 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]

  Symptoms of fever, weight loss, malaise: sinusitis/allergic rhinitis (70%); other sites of involvement include skin (65%), nervous system (40-63%), heart (30-50%), abdominal viscera (20-40%). CXR: focal alveolar infiltrates. [lumen.luc.edu]

• Chills

  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]

  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]

  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

  Chronic eosinophilic pneumonia is a rare disorder characterized by the appearance of dyspnea, cough, and constitutional symptoms as a response to an abundance of eosinophils in the lungs. [symptoma.com]

  A 75-year-old man developed dyspnea, cough, peripheral radiographic infiltrates, eosinophilia, and severe hypoxemia requiring mechanical ventilation. [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]

  Chronic eosinophilic pneumonia is a rare disorder characterized by the appearance of dyspnea, cough, and constitutional symptoms as a response to an abundance of eosinophils in the lungs. [symptoma.com]

• 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]

  Löffler syndrome, a form of eosinophilic pneumonia, may cause no symptoms or mild respiratory symptoms (most often dry cough). Chest x-rays and blood tests to find elevated levels of eosinophils in the blood are needed for diagnosis. [msdmanuals.com]

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

• Pulmonary Disorder

  Chronic eosinophilic pneumonia is a subacute-to-chronic pulmonary disorder occurring most commonly in nonatopic women with cough, fever, dyspnea, weight loss, and night sweats. [ncbi.nlm.nih.gov]

  Eosinophilia in Pulmonary Disorders. Immunol Allergy Clin North Am. 2015 Aug. 35 (3):477-92. [Medline]. [emedicine.medscape.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]

  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]

• 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]

  Chest radiography and spiral chest computed tomography demonstrated bilateral patchy pulmonary infiltrates without evidence of pulmonary embolism. [cid.oxfordjournals.org]

  bilateral 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]

  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]

  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]

• Right Pleural Effusion

  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]

  Pneumonic consolidation was observed in right lower lobe (RLL) and ill-defined ground glass opacity (GGO) in left lower lobe (LLL) with small right pleural effusion ( Figure 1 ). [jtd.amegroups.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]

• Eosinophils Increased

  Eosinophilic pneumonia comprises a group of lung diseases in which eosinophils (a type of white blood cell) appear in increased numbers in the lungs and usually in the bloodstream. [msdmanuals.com]

• 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]

Our patient responded promptly to oral corticosteroid treatment in a few days. The pulmonary infiltrates and pleural effusion subsided on a 1-month follow-up chest radiograph after starting corticosteroid treatment. [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]

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]

Abstract Chronic eosinophilic pneumonia (CEP) is a disease with unknown etiology, characterized by peripheral blood eosinophilia and abnormal eosinophil accumulation in the lungs. [ncbi.nlm.nih.gov]

 Associated with Connective Tissue Disease or Vasculitis Eosinophilic Lung Disease Classification of PIE by Etiology Eosinophilic Lung Disease Specific Etiology  Drug-induced  Nitrofurantoin  Penicillin  Sulfonamides  Parasite-induced  Ascariasis [learningradiology.com]

Etiology is suspected to be an allergic diathesis. Most patients are nonsmokers. [merckmanuals.com]

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 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]

Appropriate Prophylaxis and Other Measures to Prevent Readmission. None Cottin, V, Cordier, JF. “Eosinophilic Pneumonias”.. vol. 60. 2005. pp. 841-857. [clinicaladvisor.com]

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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.