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].

• 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]

  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]

  […] 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]

  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]

• Hypoxemia

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

  Acute eosinophilic pneumonia (AEP) is an idiopathic, acute, febrile illness characterized by non-productive cough, dyspnea, diffuse pulmonary infiltrates, and hypoxemia with eosinophilic infiltration of the pulmonary parenchyma. [clinicaladvisor.com]

  Acute eosinophilic pneumonia is usually associated with: Respiratory symptoms of < 2 weeks Hypoxemia often causing acute respiratory failure and mimicking ARDS BAL eosinophilia is usually above 25% No or mild blood eosinophilia ( Lung biopsy evidence [swjpcc.com]

  […] required to establish a diagnosis but may demonstrate eosinophilic infiltration of the interstitium and alveolar lumen, organizing pneumonia, or inflammatory cells. 4, 9 Pulmonary function tests usually reveal a restrictive defect but can be normal. 4, 9 Hypoxemia [rc.rcjournal.com]

  Some manifestations to watch for: - Myocardial involvement (possible conduction disturbances) - Uveitis - Cranial nerve VII involvement - Erythema nodosa PFTs: Decreased volumes, compliance, DLCO, hypoxemia. [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]

  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]

  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]

  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]

• Cough

  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]

• Pleural Effusion

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

  effusions, cardiac enlargement common either dilatation or pericardial effusions, 70% have skin lesions, Pathological Features Alveoli flooded with eosinophils and macrophages Bronchiolitis obliterans in one-third Granulomas absent Clinical Presentation [chestx-ray.com]

• Sputum

  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]

  Result: CEP is a chronic pulmonary eosinophilic inflammation with unknown etiology, characterized by history of allergic disease, cough, sputum, but often breathlessness and wheezing; eosinophil in peripheral blood and/or sputum and/or bronchoalveolar [file.scirp.org]

  There was no foul odour or blood streaking of the sputum. The symptoms persisted despite coughmedicines and repeated courses of antibiotics. [lungindia.com]

• 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]

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 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]

• 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]

• Neutrophilia

  Patients with AEP should receive the standard work-up for any individual with acute-onset, febrile respiratory failure: Complete blood count with differential (Of note, patients with AEP typically have a peripheral blood neutrophilia – not eosinophilia [clinicaladvisor.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]

  effusions, cardiac enlargement common either dilatation or pericardial effusions, 70% have skin lesions, Pathological Features Alveoli flooded with eosinophils and macrophages Bronchiolitis obliterans in one-third Granulomas absent Clinical Presentation [chestx-ray.com]

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]

 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. [msdmanuals.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]

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]

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

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 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]

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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