[Eosinophilic diseases of the lungs].

Pulmonary eosinophilias belong to a heterogenous group of the diseases characterized by pulmonary shadows related to pulmonary tissue and/or peripheral blood eosinophilia. Although the inflammatory infiltrate consists of macrophages, lymphocytes, neutrophils and eosinophils, a significant marker for the diagnosis and treatment is eosinophilia. By etiology eosinophilic diseases of the lungs fall into primary or idiopathic (common pulmonary eosinophilia, chronic eosinophilic pneumonia, hypereosinophilic syndrome), secondary or of known origin (allergic bronchopulmonary aspergillesis, bronchocentric granulematosis, parasitic invasions, drug-induced reactions, fungal and mycobacterial infection, pulmonary diseases caused by radiation or toxins). Pulmonary eosinophilia can be also associated with systemic diseases (Churg-Strauss syndrome) and tumors. Clinicoroentgenological picture of different eosinophilic diseases of the lungs is almost the same. Verification of the diagnosis is based on the presence of bronchial asthma and extrapulmonary manifestations, the level of eosinophilia in the blood, bronchoalveolar lavage and total IgE, histological and chest CT findings. This article presents modern classification, clinicoroentgenological and histological characteristics of different, primarily idiopathic, eosinophilic diseases of the lungs.

Eosinophilic lung diseases: a clinical, radiologic, and pathologic overview.

Eosinophilic lung diseases are a diverse group of pulmonary disorders associated with peripheral or tissue eosinophilia. They are classified as eosinophilic lung diseases of unknown cause (simple pulmonary eosinophilia [SPE], acute eosinophilic pneumonia [AEP], chronic eosinophilic pneumonia [CEP], idiopathic hypereosinophilic syndrome [IHS]), eosinophilic lung diseases of known cause (allergic bronchopulmonary aspergillosis [ABPA], bronchocentric granulomatosis [BG], parasitic infections, drug reactions), and eosinophilic vasculitis (allergic angiitis, granulomatosis [Churg-Strauss syndrome]). The percentages of eosinophils in peripheral blood and bronchoalveolar lavage fluid are essential parts of the evaluation. Chest computed tomography (CT) demonstrates a more characteristic pattern and distribution of parenchymal opacities than does conventional chest radiography. At CT, SPE and IHS are characterized by single or multiple nodules with a surrounding ground-glass-opacity halo, AEP mimics radiologically hydrostatic pulmonary edema, and CEP is characterized by nonsegmental airspace consolidations with peripheral predominance. ABPA manifests with bilateral central bronchiectasis with or without mucoid impaction. The CT manifestations of BG are nonspecific and consist of a focal mass or lobar consolidation with atelectasis. The most common CT findings in Churg-Strauss syndrome include sub-pleural consolidation with lobular distribution, centrilobular nodules, bronchial wall thickening, and interlobular septal thickening. The integration of clinical, radiologic, and pathologic findings facilitates the initial and differential diagnoses of various eosinophilic lung diseases.

[Clinicopathological differences between acute and chronic eosinophilic pneumonia].

Considerable confusion exists regarding the proper classification of idiopathic eosinophilic pneumonia (IEP). In addition, there are no reports that reveal clinicopathological differences between the various eosinophilic pneumonias. A problem persists in describing what the essential histological differences are between the different types of IEP. In this context, we examined the histological findings of acute eosinophilic pneumonia (AEP) and chronic eosinophilic pneumonia (CEP) and contrasted them with the clinical features and radiological findings. Radiologically, ground glass opacity and interlobular septal thickening were characteristic of the AEP cases studied, while air space consolidation was seen in all CEP cases. Histologically, interstitial edema and fibrin deposition were prominent in the AEP cases. Type II cells were detached from the alveolar walls, though the basal lamina was predominantly intact. In CEP, in addition to cellular infiltration, there was prominent intraluminal fibrosis. Disruption of the basal lamina was observed and nests of intraluminal fibrosis were directly adjacent and connected to the alveolar walls. From these findings, we conclude that the histological differences between AEP and CEP are the severity of basal lamina damage, the amount of subsequent intraluminal fibrosis, and the severity of interstitial edema. Especially in AEP, interstitial edema is an essential histological finding and this finding explains the acute onset, and the radiographic findings, as well as the rapid and complete improvement noted in such cases.

The eosinophil and the lung.

Current concepts regarding the morphology, constituents, distribution, and kinetics of the eosinophil allow an expanded understanding of the eosinophil's function in health and disease. In particular, certain eosinophil constituents may have beneficial effects (modulation of mast cell-dependent reactions and helminthotoxic properties), while others may produce detrimental effects (tissue destruction). Eosinophils may be clinically important in obstructive and infiltrative pulmonary diseases. In obstructive disease, a peripheral eosinophilia indicates reversibility, and the magnitude of the peripheral eosinophil count correlates with the severity of the reversible obstruction. Concerning infiltrative pulmonary disease, an updated classification of pulmonary infiltrates with eosinophilia, which is based on recognizable causes and syndromes, is presented, and allergic bronchopulmonary aspergillosis, chronic eosinophilic pneumonia, drug reactions, the hypereosinophilic syndrome, parasitic infestations, and the Churg-Strauss syndrome are specifically considered.

Reevaluation of eosinophilic pneumonia and its diagnostic criteria.

Eosinophilic pneumonia has been defined as pulmonary infiltration of the lung by eosinophils that may or may not be accompanied by an excess of these cells in the peripheral blood. However, the concept of this disease and its nomenclature have not yet been established. In the present study, the clinical course of 11 cases of eosinophilic pneumonia, which were clinico-pathohistologically diagnosed and found not to be associated with organic disorders producing peripheral blood eosinophilia, were investigated extensively and compared with various types of eosinophilic pneumonia, as previously reported. Of five cases of acute eosinophilic pneumonia with a history (less than 1 month) of symptoms before diagnosis, a short clinical course, and no recurrence, four cases showed peripheral blood eosinophilia, and four cases did not require treatment with steroids. Of six cases of chronic eosinophilic pneumonia with a history (greater than 2 months) of symptoms before diagnosis, a prolonged clinical course, and recurrence, all showed peripheral blood eosinophilia, four cases required treatment with steroids, and four cases were recurrent. In one case with simultaneous occurrence of asthma and another case with asthma that occurred 4 years after the appearance of eosinophilic pneumonia, abnormal shadows on chest roentgenograms had continued for several years. These results suggested that various types of previously reported eosinophilic pneumonia classified by sex, the presence or absence of peripheral blood eosinophilia, the degree of clinical symptoms or peripheral blood eosinophilia, and the degree of abnormalities on the chest roentgenograms should be extensively reevaluated.

Acute and chronic eosinophilic pneumonia: clinical evaluation and the criteria.

The clinical courses of 11 cases of eosinophilic pneumonia which were clinico-pathohistologically diagnosed and found to be unassociated with organic disorders producing peripheral blood eosinophilia were extensively investigated and compared with various types of eosinophilic pneumonia previously reported. Five cases of acute eosinophilic pneumonia fulfilled the following criteria: 1) less than a one-month history of symptoms prior to diagnosis, 2) a short clinical course and 3) no recurrence. Six cases of chronic eosinophilic pneumonia fulfilled the following criteria: 1) more than a two-month history of symptoms prior to diagnosis, 2) a prolonged clinical course and 3) recurrence. The results suggested that various types of previously reported eosinophilic pneumonia classified by sex, the presence or absence of peripheral blood eosinophilia, the degree of clinical symptoms or peripheral blood eosinophilia, and the degree of abnormalities on chest X-ray films should be extensively reevaluated.

[Malignant hypereosinophilic syndrome. Report of an exceptional case (author's transl)]. / Síndrome hipereosinofílico maligno: presentación de un caso excepcional.

The case of a 43-year-old man with a highly malignant hypereosinophilic syndrome is reported. The condition is classified as such according to Hardy and Anderson's criteria, accepted by many other authors. Other diseases of known etiology which may present high levels of eosinophils in the peripheral blood, such as parasitosis, allergies, neoplasias, collagenosis, etc., were discounted beforehand. The difficulties in distinguishing between these diseases are discussed; they are often accompanied by clinical manifestations which also arise in very different conditions including eosinophilic leukemia, Engfeldt and Zetterström's eosinophilic collagenosis, Löffler's fibroplastic endocarditis, etc. A particularly striking feature of this condition is the formation of large tumor masses of mature eosinophils. They begin in various bones, which they destroy almost completely, and invade the surrounding tissues, destroying them as well. These tumors act similarly to malignant eosinophilic myelocytomas, a fact which has not been reported previously in the literature as far as we know. Although the eosinophils act as though they were neoplastic, they maintain the characteristics of mature cells, both cytomorphologically and ultrastructurally as well as cytochemically (consistently chloroacetate esterase negative). The tendency to diagnose eosinophilic leukemia solely on the basis of the malignancy of the condition and a tissue infiltration of eosinophils without determining the existence of cytologic and/or cytochemical anomalies of the cells showing them to be leukemic is discussed. The authors were unable to find any reports in the literature in which the eosinophils were presented with unmistakeably blastic cellular characteristics. Various nosologic considerations are offered.

[The eosinophilic lung]. / Le poumon eosinophile.

Eosinophilic lung disease comprises a diverse group of disorders characterized by eosinophilic pulmonary infiltration in association with other inflammatory cells. In patients with respiratory symptoms, usually associated with radiographically documented infiltrates, blood eosinophilia is a helpful but inconsistent diagnostic finding. Currently diagnosis is confirmed more often by bronchoalveolar lavage than by lung biopsy. Possible etiologies include parasites, mycotic agents, drugs, and angeitis. Remaining cases are classified as idiopathic eosinophilic lung disease including Carrington's disease, idiopathic hypereosinophilic syndrome, acute eosinophilic pneumonia, and Loeffler's syndrome. Mild eosinophilia is also a possible finding of bronchoalveolar lavage in several other disorders but the role of eosinophils is less important. The prognosis and treatment of eosinophilic lung disease varies depending on etiology. Corticosteroids are frequently used but treatment modalities also depend on etiology.

[Chronic eosinophilic pneumonia]. / Neumonia eosinofilica crónica: informe de un caso.

Chronic eosinophilic pneumonia. The chronic eosinophilic pneumonia is part of Pulmonary Eosinophilic Syndroms. It is presented a 33-years old man, Asmathic, with dry cough, fever, night sweats and fatigue of several weeks. The chest X-ray showed opacity in the right hemithorax. He was treated with antibiotics without response. A chest TC showed multifocal involvement. The patient refused bronchoalveolar lavage (BAL) so treatment antituberculostatic was started. Despite treatment the symptoms worsened. The Chest X-ray showed migration of the infiltrates and the blood smear marked eosinophilia. Finally, bronchoalveolar lavage was carried out and it showed a high percentage of eosinophils (over 50%). The patient was treated with inmmunosuppresive doses of corticosteroids with excellent response. The blood smear in Nonresolving pneumonia is key to consider eosinophilic pneumonia, an uncommon pathology but amenable to treatment.

Eosinophilic lung diseases.

Accurate diagnosis of eosinophilic lung diseases is essential to optimizing patient outcomes, but remains challenging. Signs and symptoms frequently overlap among the disorders, and because these disorders are infrequent, expertise is difficult to acquire. Still, these disorders are not rare, and most clinicians periodically encounter patients with one or more of the eosinophilic lung diseases and need to understand how to recognize, diagnose, and manage these diseases. This review focuses on the clinical features, general diagnostic workup, and management of the eosinophilic lung diseases.

Pulmonary eosinophilia.

Pulmonary eosinophilia comprises a heterogeneous group of diseases defined by eosinophilia in pulmonary infiltrates (bronchoalveolar lavage fluid) or in tissue (lung biopsy specimens). Although the inflammatory infiltrate is composed of macrophages, lymphocytes, neutrophils and eosinophils, eosinophilia is an important marker for the diagnosis and treatment. Clinical and radiological presentations can include simple pulmonary eosinophilia, chronic eosinophilic pneumonia, acute eosinophilic pneumonia, allergic bronchopulmonary aspergillosis and pulmonary eosinophilia associated with a systemic disease, such as in Churg-Strauss syndrome and hypereosinophilic syndrome. Asthma is frequently concomitant and can be a prerequisite, as in allergic bronchopulmonary aspergillosis and Churg-Strauss syndrome. In diseases with systemic involvement, the skin, the heart and the nervous system are the most affected organs. The radiological presentation can be typical, or at least suggestive, of one of three types of pulmonary eosinophilia: chronic eosinophilic pneumonia, acute eosinophilic pneumonia and allergic bronchopulmonary aspergillosis. The etiology of pulmonary eosinophilia can be either primary (idiopathic) or secondary, due to known causes, such as drugs, parasites, fungal infection, mycobacterial infection, irradiation and toxins. Pulmonary eosinophilia can be also associated with diffuse lung diseases, connective tissue diseases and neoplasia.

Síndromes de infiltrados pulmonares con eosinofilia / Pulmonary infiltrates with eosinophilia syndrome

Los síndromes de infiltrados pulmonares con eosinofilia (síndromes PIE) constituyen un grupo heterogéneo de desórdenes poco frecuentes, de diferente etiopatogenia y presentación clínica variable. Estos cuadros tienen en común la presencia de infiltrados pulmonares y eosinofilia periférica, en el lavado broncoalveolar y en el intersticio pulmonar; aunque además pueden existir síntomas sistémicos. Actualmente se recomienda clasificar a los síndromes PIE según la etiología en idiopáticos y secundarios. Dentro de los primeros se encuentran la eosinofilia pulmonar simple (síndrome de Loeffler), neumonía eosinofílica aguda, neumonía eosinofílica crónica, síndrome hipereosinofílico idiopático, granulomatosis alérgica o síndrome de Churg- Strauss y granulomatosis broncocéntrica. Los secundarios incluyen la aspergilosis broncopulmonar alérgica, eosinofilia inducida por parásitos (forma más frecuente en pediatría) y por drogas. Los corticoides constituyen el tratamiento de elección, en cambio cuando la etiología son parásitos la terapia debe ser hecha con fármacos antiparasitarios. Se debe tener un alto índice de sospecha en el diagnóstico, debido a la escasa frecuencia que presentan estos síndromes y a la significativa morbilidad y en ocasiones mortalidad, especialmente la neumonía eosinofílica aguda, que se manifiesta frecuentemente con insuficiencia respiratoria severa de etiología desconocida.

Clinicopathological differences between acute and chronic eosinophilic pneumonia.

OBJECTIVE: Considerable confusion exists regarding the proper classification of idiopathic eosinophilic pneumonia (IEP). Furthermore, there are no reports describing the clinicopathological differences between the various forms of eosinophilic pneumonias. METHODOLOGY: The histological findings in acute eosinophilic pneumonia (AEP) and chronic eosinophilic pneumonia (CEP) were examined and the clinical and radiological features were contrasted with them. RESULTS: Radiologically, ground glass opacity and interlobular septal thickening were characteristic of the AEP cases, while air space consolidation was seen in all CEP cases. Histologically, interstitial oedema and fibrin deposition were prominent in the AEP cases. Type II cells were detached from the alveolar walls, although the basal lamina was predominantly intact. In CEP, in addition to cellular infiltration, there was prominent intraluminal fibrosis. Disruption of the basal lamina was observed and nests of intraluminal fibrosis were directly adjacent and connected to the alveolar walls. CONCLUSIONS: An essential histological difference between AEP and CEP is the severity of basal lamina damage and the amount of subsequent intraluminal fibrosis. In AEP particularly, these findings explain the radiographical findings, as well as the rapid and complete improvement noted in such cases.

[Eosinophilic lung. A case report: diagnostic approach]. / Poumon éosinophile. A propos d'un cas: approche diagnostique.

From a particular case of eosinophilic lung, we try to define a practical way to an easier diagnosis. We also review all the concerned pathologic entities and propose an aetiologic classification.

Studies on circadian periodicity of plasma 17-hydroxycorticosteroids (17-OHCS) in tropical pulmonary eosinophilia.

Circadian periodicity of plasma 17-hydroxycorticosteroids (17-OHCS) was studied in ten healthy controls and 25 patients with tropical pulmonary eosinophilia (TPE). Subjects were synchronized for 1 week with diurnal activity from 0600 to 2200 hr and nocturnal rest; meals were taken at 0830, 1300, and 2030 hr. No medications were administered. TPE patients were divided into three subgroups of fourteen, seven, and four each according to the hour of the worsening of their symptoms. Blood samples were collected at 8-hr intervals at fixed clock hours for 24 hr, starting at 0800 in all four groups. We noted a marked rhythm in plasma 17-OHCS in controls, with significant amplitude and an acrophase at 1033 hr. Similarly, all TPE patients exhibited a definite rhythm in plasma 17-OHCS with significant amplitude irrespective of the time of worsening of symptoms, suggesting thereby no relationship between worsening of symptoms and the rhythm. Thus the circadian nature and normal concentration of plasma 17-OHCS were observed to be similar in healthy controls and TPE patients.