Edit concept Question Editor Create issue ticket

Chronic Eosinophilic Pneumonia

Eosinophilic Lung Disease

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. The cause remains to be determined, but many patients suffer from asthma, suggesting an immune-mediated pathogenesis. Clinical criteria, laboratory workup identifying eosinophilia, and imaging studies in the form of X-rays and computed tomography, are used to make the diagnosis.


Presentation

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]
  • Computed tomography examination demonstrated mediastinal lymphadenopathy and peculiar, vertically oriented platelike infiltrates.[ncbi.nlm.nih.gov]
Fever
  • A 24-year-old Chinese woman presented with cough, chest pain, weight loss, low grade fever and bronchial breath sounds on auscultation.[ncbi.nlm.nih.gov]
  • A 44-year-old woman was hospitalized with a 2-day history of cough, sputum, and fever. There was no history of atopic dermatitis or asthma. On admission, the chest X-ray revealed scattered infiltration in the left upper lung fields.[ncbi.nlm.nih.gov]
  • 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]
  • Eosinophilic pneumonia is classified by its acute or chronic presentation, the distinguishing characteristics of which are based on the presence of cough, dyspnea, fever and pulmonary infiltrates with accumulation of inflammatory cells, predominantly[ncbi.nlm.nih.gov]
  • 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]
Weight Loss
  • A 24-year-old Chinese woman presented with cough, chest pain, weight loss, low grade fever and bronchial breath sounds on auscultation.[ncbi.nlm.nih.gov]
  • A 57-year-old woman was admitted to our hospital for lumbago, weight loss and weakness of her right lower extremity.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
  • A 11-year-old asthmatic girl was admitted to our clinic with a 3-month history of progressive cough, dyspnoea, weight loss and asthenia.[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]
  • 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]
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]
Constitutional Symptom
  • 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]
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]
  • A 75-year-old man developed dyspnea, cough, peripheral radiographic infiltrates, eosinophilia, and severe hypoxemia requiring mechanical ventilation.[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]
  • A 24-year-old Chinese woman presented with cough, chest pain, weight loss, low grade fever and bronchial breath sounds on auscultation.[ncbi.nlm.nih.gov]
  • A 44-year-old woman was hospitalized with a 2-day history of cough, sputum, and fever. There was no history of atopic dermatitis or asthma. On admission, the chest X-ray revealed scattered infiltration in the left upper lung fields.[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]
  • A 75-year-old man developed dyspnea, cough, peripheral radiographic infiltrates, eosinophilia, and severe hypoxemia requiring mechanical ventilation.[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]
  • We treated two patients (17- and 19-yr-old males) of chronic eosinophilic pneumonia whose symptoms were cough and dyspnea on exertion. The symptoms were recurrent while tapering off corticosteroid.[ncbi.nlm.nih.gov]
  • Each one of four attacks observed during a 4-year period was characterized by dyspnea, wheezing, peripheral blood eosinophilia, elevated serum IgE levels, and pulmonary infiltrates.[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]
  • This was a relatively rare case of T-cell lymphoma, in which an eosinophilic pneumonia and eosinophilic pleural effusion were observed.[ncbi.nlm.nih.gov]
  • Sammanys@ngha.med.sa Abstract We describe a rare case of a 29-year-old woman with chronic eosinophilic pneumonia (CEP) presenting with massive bilateral pleural effusion leading to respiratory failure, a complication that was not reported before with[ncbi.nlm.nih.gov]
  • The diagnosis was difficult due to the simultaneous presence of a pleural effusion and congestive heart failure. Radiological findings and treatment are discussed.[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]
  • We report a 15-year-old girl presenting with dry cough, exertional dyspnoea, weight loss, fever and night sweats for over 1 month. Blood tests revealed hypereosinophilia, high IgE and antinuclear antibodies levels.[ncbi.nlm.nih.gov]
  • 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]
  • A dry cough accompanied these symptoms in four of the six patients (67%). No patient gave a history of recent infection, although Patient 3 had a history of a pneumonic process treated with trimethoprim-sulfamethoxazole 4 months before biopsy.[nature.com]
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]
Chronic Urticaria
  • Anti-IgE therapy, using recombinant humanized anti-IgE antibodies, is clinically effective in patients with eosinophil-related disorders such as allergic asthma, allergic rhinitis, and chronic urticaria.[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]

Workup

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]
  • Pulmonary eosinophilia comprises a heterogeneous group of diseases that are defined by eosinophilia in pulmonary infiltrates or in tissue.[ncbi.nlm.nih.gov]
  • These features were not found in the four patients with eosinophilic pulmonary infiltrates from other causes. CONCLUSION: Eosinophils in chronic eosinophilic pneumonia show signs of activation with release of eosinophil proteins.[ncbi.nlm.nih.gov]
  • Abstract Chronic eosinophilic pneumonia (CEP) is a rare disease characterized by a progressive symptomatic deterioration of more than 1 month, pulmonary infiltrates with eosinophils, and a dramatic response to corticosteroid treatment.[ncbi.nlm.nih.gov]
  • Chronic eosinophilic pneumonia was diagnosed in a 26-year-old woman, based on the characteristic peripherally distributed pulmonary infiltrates, eosinophilia, and relatively severe symptomatology.[ncbi.nlm.nih.gov]
Bilateral Pulmonary Infiltrate
  • 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]
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]
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]
Pleural Effusion
  • Pleural effusions are rarely seen. We report a case of chronic eosinophilic pneumonia with transudative eosinophilic pleural effusion.[ncbi.nlm.nih.gov]
  • This was a relatively rare case of T-cell lymphoma, in which an eosinophilic pneumonia and eosinophilic pleural effusion were observed.[ncbi.nlm.nih.gov]
  • Sammanys@ngha.med.sa Abstract We describe a rare case of a 29-year-old woman with chronic eosinophilic pneumonia (CEP) presenting with massive bilateral pleural effusion leading to respiratory failure, a complication that was not reported before with[ncbi.nlm.nih.gov]
  • The diagnosis was difficult due to the simultaneous presence of a pleural effusion and congestive heart failure. Radiological findings and treatment are discussed.[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]

Treatment

  • 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]
  • Some recent reports suggest that treatment with inhaled steroids may be of some value in this condition.[ncbi.nlm.nih.gov]
  • 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]
  • All relapse cases showed improvement upon resumption of prednisolone treatment. No difference was observed in the rate of relapse between the 3- and 6-month prednisolone treatment groups for patients with CEP.[ncbi.nlm.nih.gov]
  • Nevertheless, relapses or development of severe asthma are frequent when tapering or withdrawing treatment. Long-term oral corticosteroid therapy is necessary in up to half of the patients.[ncbi.nlm.nih.gov]

Prognosis

  • 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]
  • This rare, idiopathic but benign condition responds well to corticosteroid treatment and the long term prognosis is excellent. The typical chest radiographic pattern of 'photographic negative of pulmonary oedema' in this condition is emphasised.[ncbi.nlm.nih.gov]
  • Emergency, S Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy. davide.tassinari@aosp.bo.it Abstract Chronic eosinophilic pneumonia (CEP) is a rare disorder in children, characterised by respiratory and systemic symptoms, with a generally good prognosis[ncbi.nlm.nih.gov]
  • A follow-up study of eight Mayo Clinic patients with chronic eosinophilic pneumonia (also called Carrington's eosinophilic pneumonitis) was done in order to ascertain, if possible, the long-term prognosis of this entity, since it has not been delineated[ncbi.nlm.nih.gov]
  • Abstract The prognosis of chronic eosinophilic pneumonia (CEP) is usually good under corticosteroid therapy (CST). The main complications are relapses when treatment is tapered or discontinued.[ncbi.nlm.nih.gov]

Etiology

  • 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]
  • Chronic eosinophilic pneumonia (CEP) is an inflammatory disease characterized by accumulations of eosinophils in the lung with unknown etiology.[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]
  • Acute eosinophilic pneumonia (AEP) is a rare disease with unknown etiology.[ncbi.nlm.nih.gov]
  • Chronic eosinophilic pneumonia and Churg-Strauss syndrome are two uncommon conditions of unknown etiology that share similar pulmonary manifestations.[ncbi.nlm.nih.gov]

Epidemiology

  • 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]
  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • To examine pathophysiology of AEP we measured the cell number of eosinophils and eosinophil active cytokines in the peripheral blood and bronchoalveolar lavage fluid (BALF) of AEP patients and compared the levels with those measured in chronic eosinophilic[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]
  • 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]

Prevention

  • 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]

References

Article

  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.

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!
Last updated: 2019-07-11 20:13