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International audience; Introduction Chronic pulmonary eosinophilia is a rare condition, usually highly responsive to systemic glucocorticoids, yet relapses are frequent and require long-term treatment associated with significant morbidity. Areas covered We review the main conditions causing chronic pulmonary eosinophilia and glucocorticoid-sparing agents in this setting. Expert opinion An individually tailored etiologic assessment is mandatory in all patients presenting with chronic pulmonary eosinophilia. Reducing the cumulative exposure to glucocorticoids by treating flares with short courses of systemic glucocorticoids is both safe and feasible in idiopathic chronic eosinophilic pneumonia. In frequently relapsing patients and in those requiring high doses of glucocorticoids, alternatives to glucocorticoids should be considered. Mepolizumab has been approved in several countries for the treatment of both hypereosinophilic syndrome and eosinophilic granulomatosis with polyangiitis. Given their ability to induce rapid and sustained depletion in blood and tissue eosinophils (e.g. benralizumab and lirentelimab) and/or to curb Type 2-mediated inflammation (e.g. dupilumab), other biologics hold promise in these settings. Likewise, anti-IgE targeted therapies (i.e omalizumab) should be considered in the glucocorticoid-sparing therapeutic armamentarium of allergic or hypersensitivity-related diseases, including allergic bronchopulmonary aspergillosis. Other drugs (fevipiprant, tezepelumab) are at an early phase of development. Cost-effectiveness studies are lacking. Whether glucocorticoid-free treatment regimens are achievable in these conditions is unknown.

Although ICEP is an exceptionally rare disease, it is potentially overlooked and misdiagnosed. This case highlights the importance of peripheral eosinophilia in raising suspicion for ICEP. Without treatment, majority of patients fail to improve.

Idiopathic chronic eosinophilic pneumonia (CEP) is a rare disease of unknown cause characterized by eosinophilic alveolar and interstitial infiltration. The authors describe the case of a 46-year-old black man, presenting with insidious onset and progressive course of dyspnea on minimum exertion, cough, fever, night sweats, and weight loss for one year and worsening in the last three months. The main findings were serum eosinophilia. Chest radiographs showed multifocal infiltrations of irregular distribution in both lungs and a restrictive functional impairment. The patient underwent open lung biopsy, and the anatomopathological examination revealed consolidation by exudate constituted predominantly by macrophages (25%) and eosinophils (51%), which filled small air spaces, including respiratory and membranous bronchioles. The anatomopathological diagnosis was eosinophilic pneumonia (eosinophils > 25% is widely accepted for diagnosing eosinophilic pneumonia). The patient had a good clinical response after starting corticosteroid therapy.

Introduction: Idiopathic chronic eosinophilic pneumonia (ICEP) is an orphan lung disease characterized by concomitant systemic and local eosinophilia, along with bilateral lung infiltrates. Symptoms include dyspnea of subacute/chronic onset, cough, and general systemic signs. Although all patients do respond to oral corticosteroids, relapse rate is very high, which highlights the need for alternative therapies in case of relapsing ICEP. Mepolizumab is a fully humanized antibody directed against interleukin 5, a key growth factor of eosinophils. In the present study, we retrospectively studied the effect of off-label use of mepolizumab for relapsing ICEP. Materials and Methods: All data from patients treated with mepolizumab for relapsing ICEP were included in our database and diagnoses were reviewed. We analyzed the effect of treatment on relapse rate, oral corticosteroids (OCS) use, and lung lesions on high-resolution computed tomography (HRCT). Results: We included ten patients in the final analysis, with a median follow-up of 9 months after initiation of mepolizumab. Beside its expected effect on circulating eosinophils, treatment with mepolizumab was associated with a significant reduction of annual rate of exacerbations and a reduced consumption of corticosteroids. We also observed a remission of lung lesions on follow-up HRCT. Conclusions: In this open-label retrospective study, treatment of ICEP with mepolizumab was associated with a reduction of relapses, OCS use, and the disappearance of lung infiltrates., SCOPUS: ar.j, info:eu-repo/semantics/published

Objective: Idiopathic chronic eosinophilic pneumonia (ICEP) is a rare eosinophilic lung disorder with an unknown etiology and is characterized by subacute or chronic respiratory and general symptoms, alveolar and/or peripheral eosinophilia, and the accumulation of eosinophils in the lungs. We aimed to present diagnostic test results and follow-up outcomes of 17 patients who were diagnosed with ICEP in our hospital in light of literature. Methods: Between 2008 and 2013, we examined 17 cases of ICEP. We evaluated clinical and laboratory findings together with the long-term follow-up data. Results: The patients had a mean age of 40.8 years at presentation, and the female/male ratio was 0.8. The most common symptoms were cough (94%), shortness of breath (76%), and high fever (35%). Bronchoalveolar lavage eosinophil percentages of the patients ranged from 3% to 80%. Nine (53%) patients experienced recurrence. Six patients were maintained on low dose steroid due to repeating relapses. Among these patients, 7 (77.7%) had a total IgE level of above 500/IU/mL. Conclusion: Relapses are common in ICEP after the withdrawal of corticosteroid treatment or during dose reduction. We point out the importance of the close monitoring of patients for identifying relapse. A higher total IgE level during diagnosis may serve as a predictor of recurrence.

Eine 43-jahrige Patientin mit den klinischen Zeichen eines fieberhaften respiratorischen Infekts wurde trotz mehrfacher Antibiotikatherapie mit persistierenden beidseitigen pulmonalen Infiltraten stationar eingewiesen. In der weiteren Diagnostik fiel im Diffentialblutbild eine Eosinophilie von 31% auf. In der Bronchoskopie mit bronchoalveolarer Lavage wurde ebenfalls eine Eosinophilie gesehen. Nach Ausschluss bekannter Ursachen einer pulmonalen Eosinophilie wurde die Diagnose einer chronischen idiopathischen eosinophilen Pneumonie gestellt. Unter alleiniger Therapie mit hochdosierten Kortikosteroiden normalisierten sich die pathologischen Laborwerte, die klinischen und die radiologischen Befunde innerhalb 1 Woche annahernd vollstandig. Bei therapierefraktaren pulmonalen Infiltraten sollten die Diagnose einer akuten oder chronischen eosinophilen Pneumonie in Betracht gezogen und eine Bronchoskopie mit bronchoalveolarer Lavage veranlasst werden. Differentialdiagnostisch muss aus prognostischen und therapeutischen Grunden zwischen einer akuten und chronischen idiopathischen eosinophilen Pneumonie unterschieden werden. Ein rasches Ansprechen auf Kortikosteroide unterstutzt die Diagnose. Bei einer chronischen idiopathischen eosinophilen Pneumonie ist auf eine Therapiedauer von etwa 6 Monaten zur Vermeidung von Rezidiven zu achten.

la pneumonie chronique idiopathique a eosinophiles ou maladie de Carrington est une maladie rare de cause inconnue, caracterisee par l'association de symptomes respiratoires et generaux subaigus ou chroniques, une eosinophilie alveolaire et/ou sanguine et des infiltrats pulmonaires de topographie peripherique en imagerie. Il s'agit d'une pathologie benigne d’evolution favorable. Nous rapportons un cas de cette pathologie rare dans sa forme pseudotumorale de diagnostic difficile caracterise par l'absence d’eosinophilie sanguine et alveolaire et d’evolution fatale. Nous rappellerons aussi les caracteres cliniques, radiologiques, therapeutiques et evolutifs de la maladie de Carrington a travers une revue de litterature.

Idiopathic chronic eosinophilic pneumonia (ICEP) is characterized by subacute or chronic respiratory and general symptoms, alveolar and/or blood eosinophilia, and peripheral pulmonary infiltrates on chest imaging. Eosinophilia is present in most cases, usually in excess of 1000/mm3. In absence of significant blood eosinophilia, a diagnosis of ICEP is supported by the demonstration of bronchoalveolar lavage eosinophilia. ICEP is typically associated with eosinophil counts higher than lymphocyte counts in the bronchoalveolar lavage. ICEP is a rare disorder of unknown cause. Its exact prevalence remains unknown. ICEP may affect every age group but is rare in childhood. It is twice as frequent in women as in men. One third to one half of the ICEP patients have a history of asthma. The mainstay of treatment of ICEP is systemic corticosteroids. Response to oral corticosteroid therapy is dramatic and has led to the consideration of corticosteroid challenge as a diagnostic test for ICEP. 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.

Hypereosinophilic asthma (defined by blood eosinophils >1 G/l, especially >1.5 G/l) may occur as a primary pathology (and often difficult to treat) or as the manifestation of a hypereosinophilic disorder of determined cause (iatrogenic, parasitic, allergic bronchopulmonary aspergillosis) or idiopathic (idiopathic chronic eosinophilic pneumonia, Churg–Strauss syndrome, idiopathic hypereosinophilic syndrome).

A 34-year-old man with bronchial asthma was introduced to our clinic because his blood tests showed a white blood cell count of 25,200/μL with a differential of 70% eosinophils, and chest computed tomography revealed non-segmental multi-lobar consolidation. Bronchoscopy was performed and yellow broncho-alveolar lavage fluid (BALF) was collected (Picture 1). The supernatant of BALF was still yellow (Picture 2). The total cell count (TCC) was 110×105/mL and the cell differentiation of BALF showed a significant elevation of eosinophils (98%). As a result, a diagnosis of idiopathic chronic eosinophilic pneumonia (EP) was made. Eosinophilic cationic protein (ECP), a representative eosinophil-derived protein, in BALF was also significantly elevated (105 μg/L).

International audience; BACKGROUND: Idiopathic eosinophilic pneumonia is extremely rare in children and adults. We present herein the first series describing the specificities of idiopathic chronic (ICEP) and acute (IAEP) eosinophilic pneumonia in children. METHODS: We retrospectively analyzed all cases of ICEP and IAEP in children that were retrieved from French Reference Centers for rare pediatric lung diseases. RESULTS: Five cases of pediatric ICEP were identified. Corticosteroid or immunosuppressive therapy dramatically improved the outcome in three cases. The remaining two cases had a persistent interstitial pattern with progressive development of cystic airspace lesions. Three cases of IAEP in adolescents were reported, with one requiring four days of extracorporeal membrane oxygenation. CONCLUSION: ICEP is a rare disease with a polymorphic clinical presentation in children. We identified patients with persistent interstitial patterns progressing to cystic airspace regions, for which the boundaries with idiopathic interstitial pneumonias are difficult to establish. We therefore propose a specific pediatric definition and classification algorithm. IAEP in children remains an inflammatory reaction of the lung to an acute toxic exposure, mainly tobacco, as in adults. International studies are required to comprehensively assess the various clinical forms of the disease as well as the appropriate therapeutic regimens.

As pneumonias eosinofílicas constituem um grupo heterogéneo de quadros clínicos que podem comprometer unicamente as vias aéreas, o parênquima pulmonar, ou ambos, caracterizado pela presença de eosinofilia alveolar e infiltrados pulmonares, com ou sem evidência de eosinofilia no sangue periférico¹. A pneumonia eosinofílica crónica idiopática (PECI), descrita por Carrington5 em 1969, é uma doença pulmonar eosinofílica rara, de etiologia desconhecida, caracterizada por eosinofilia periférica, infiltrados pulmonares na telerradiografia torácica e excelente resposta à corticoterapia. A PECI afecta geralmente mulheres de meia idade, sendo os sintomas mais comuns a tosse, a dispneia, a febre e a perda de peso ². Os autores apresentam um caso de PECI numa mulher jovem, de 21 anos, raça caucasiana, não fumadora e sem antecedentes patológicos relevantes. The eosinophilic pneumonias are a heterogeneous group of pulmonary disorders, which may compromise only the air ways, the pulmonary parenchyma, or both, characterised by alveolar eosinophils and infiltration of pulmonary tissue, with or without peripheral blood eosinophilia¹. Idiopathic Chronic Eosinophilic Pneumonia (ICEP), detailed description was by Carrington5 in 1969, is a rare eosinophilic lung disease, of unknown aetiology, characterised by peripheral blood eosinophilia, chest radiograph infiltrates and prompt response to corticosteroid therapy. ICEP most commonly affects women of middle age and usual symptoms are cough, dyspnea, fever and weight loss². The authors present a case of ICEP in a young woman, 21 years old, non -smoker and previously healthy.

International audience; Since idiopathic chronic eosinophilic pneumonia (ICEP) and asthma are frequently associated, their possible reciprocal influence on clinical presentation and evolution were investigated. The clinical and follow-up features of 53 cases of ICEP, of which 41 (77%) had asthma, were reviewed retrospectively. Asthma preceded the diagnosis of ICEP in 26 patients, was contemporaneous in eight patients, and developed 17+/-12 months after ICEP in seven patients. Presentation of ICEP was similar in asthmatics and nonasthmatics with the exception of a higher level of total immunoglobulin E in the former group. Patients with asthma at the time of diagnosis of ICEP were more likely to remain free of relapse of ICEP (56 versus 23%) and had a lower number of relapses per year of follow-up (median 0 versus 0.24). Moreover, they were treated more frequently with long-term inhaled corticosteroids (88 versus 31%) at last follow-up. Asthma got worse after the diagnosis of ICEP and frequently required long-term oral corticosteroids. To conclude, among patients with idiopathic chronic eosinophilic pneumonia asthmatics have a lower frequency of relapse than nonasthmatics, possibly because of a higher use of inhaled corticosteroids. The occurrence of idiopathic chronic eosinophilic pneumonia in asthmatics is often associated with the development of severe asthma.

Background Idiopathic chronic eosinophilic pneumonia (ICEP) is a rare disorder of unknown cause characterised by subacute or chronic respiratory and general symptoms, alveolar and /or blood eosinophilia, and peripheral pulmonary infiltrates on chest imaging. Interestingly, some but not all patients diagnosed with ICEP have a history of asthma, whilst others may develop asthma after a dignosis of ICEP has been made. Methods We present this rare and interesting case, because ICEP is a rare complication of asthma, although it is seldom mentioned in reviews ant textbooks on asthma. Asthma in patients with ICEP is relatively severe and get worse after diagnosis of ICEP. Results A 70-year-old woman with a history of asthma and chronic rhinitis with polyps (diagnosed in 2003), nonsmoker, history of allergies negative. She suffered from frequent exacerbations of asthma (7 times a year with repeated courses of oral corticosteroids). In 2006 she had sudden fever, weight loss, malaise and impaired dyspnea with productive cough, mild chest pain on sternum and respiratory failure. A chest radiograph demonstrated bibasilar infiltrates. Peripheral blood smear showed a newly developed, marked eosinophilia, and a chest X-rays and HRCT scan revealed a diffuse patchy nodular infiltrate in all lung fields. Serum-precipitating antibodies against Aspergillus antigens negative, no cutaneous reactivity to Aspergillus antigen, negative findings for parasitic infections, no central bronchiectasis on previous HRCT, ANCA, ANA, ENA negative. She received an intensive course of corticosteroids with complete resolution of symptoms and the eosinophilia, as well as decreased infiltrates on chest radiograph. Doses of corticosteroids slowly reduced a maintained until June 2009. Her asthma often exacerbates so far and needs intermittent courses of corticosteroids, is difficult- to-treat, but without any relapses of ICEP. Conclusions Clinicians should consider pulmonary eosinophilia in the differential diagnosis of patients treated for asthma who develop pulmonary infiltrates with dyspnea.

Two cases of pulmonary eosinophilia associated with coccidioidal infections are reported. Pulmonary eosinophilia in these cases represents a hypersensitivity reaction to the fungus. Histologically, the pulmonary eosinophilia in these cases closely mimicked or appeared identical to idiopathic chronic eosinophilic pneumonia. Coccidioides immitis organisms were rare or absent in the areas of pulmonary eosinophilia. Recognition of this phenomenon is important for proper care of the patient.