Idiopathic Chronic Eosinophilic Pneumonia Research Articles

The long-term outcomes of mepolizumab treatment at 100 mg dose on idiopathic chronic eosinophilic pneumonia: A real-life experience.

Background: The standard therapeutic regimen for idiopathic chronic eosinophilic pneumonia (ICEP) involves the administration of oral corticosteroids (OCS). However, a notable proportion of individuals experience recurrent episodes after the tapering or cessation of OCS during the course of ICEP. There has been a growing interest in exploring alternative treatment modalities for patients with ICEP at heightened risk of relapse. Objective: The aim of this study was to assess the efficacy of mepolizumab at a dose of 100 mg administered every 4 weeks in preventing relapses of ICEP and its impact on the clinical outcomes. Methods: This retrospective clinical observational study used real-world data to assess the impact of mepolizumab on patients diagnosed with ICEP accompanied by severe asthma. Demographic information and clinical characteristics were extracted from medical records. The study examined the effect of mepolizumab on the annual relapse rate, OCS dose, eosinophil count, and respiratory function parameters. Results: All patients included in the study, with a median (range) follow-up period of 19 months (4-40 months), the annual relapse rate decreased from 0.33 to 0 after the initiation mepolizumab. In addition, the maintenance OCS dose, expressed in methylprednisolone equivalents, declined from 4 mg/day to 0 mg/day. A reduction in the blood eosinophil count was observed, alongside a partial improvement in respiratory function test results among the patients. Conclusıon: A dose regimen of 100 mg of mepolizumab administered every 4 weeks emerges as a promising and well-tolerated therapeutic approach for averting relapses of ICEP.

Monoclonal antibodies in idiopathic chronic eosinophilic pneumonia: a scoping review

BackgroundIdiopathic chronic eosinophilic pneumonia (ICEP) is a rare disease characterized by pulmonary radiological alterations, peripheral eosinophilia, and demonstrated pulmonary eosinophilia. Oral steroids (OSs) are the standard management, but relapses occur in up to 50% of patients during the decrease or suspension of steroids, usually requiring reinitiation of treatment, exposing patients to secondary events derived from the management. Management with monoclonal antibodies has been proposed in these cases to control the disease and limit the secondary effects. The objective is to describe the extent and type of evidence regarding the use of monoclonal antibodies for ICEP.MethodsA panoramic review of the literature was performed. Observational and experimental studies of pediatric and adult populations that managed recurrent ICEP with monoclonal antibodies were included. Data search, selection, and extraction were performed by two independent reviewers.Results937 studies were found. After applying the inclusion and exclusion criteria, 37 titles remained for the final analysis: a retrospective, observational, real-life study, two case series publications, and 34 case reports published in academic poster sessions and letters to the editor. In general, the use of monoclonal antibodies approved for severe asthma could be useful for the control of ICEP, since most of the results show a good response for clinical and radiological outcomes. Biological drugs seem to be a safer option for controlling relapses in ICEP, allowing lowering/suspension of OSs, and sometimes replacing them in patients intolerant to them, patients with significant comorbidities, and patients who have already developed adverse events.ConclusionThe extent of the evidence supporting management of ICEP with monoclonal antibodies against IL-5 and IgE (omalizumab) is limited, but it could be promising in patients who present frequent relapses, in cortico-dependent individuals, or in patients in whom the use of steroids is contraindicated. The extent of the evidence for management with dupilumab is more limited. Studies with better design and structure are needed to evaluate quality of life and outcomes during a clear follow-up period. To our knowledge, this is the first scoping review of the literature showing the extent of the evidence for the management of ICEP with monoclonal antibodies.

The clinical question of mepolizumab in the long-term treatment of idiopathic chronic eosinophilic pneumonia; how long should we use mepolizumab?

The clinical question of mepolizumab in the long-term treatment of idiopathic chronic eosinophilic pneumonia; how long should we use mepolizumab?

Case Report: Mepolizumab in the treatment of idiopathic chronic eosinophilic pneumonia

Idiopathic chronic eosinophilic pneumonia (ICEP) is a rare interstitial lung disease of unknown cause. It usually responds well to systemic corticosteroid therapy, but relapses are frequent. We describe two cases of 21- and 27-year-old patients, presenting with dyspnea. The diagnosis of steroid-relapsing and steroid-dependent ICEP was made respectively. Mepolizumab was prescribed to both patients. This treatment resulted in successful long-term disease management with much fewer side effects than a traditional corticosteroid therapy.

A case of hypocomplementemic urticarial vasculitis syndrome complicated by eosinophilic pneumonia: a case report and review of the literature.

The primary symptom of urticarial vasculitis (UV), which is a histopathological leukocytoclastic vasculitis disease, is an eruption that resembles urticaria. Other organs may also experience accompanying symptoms. Lung lesions with UV are mostly obstructive pulmonary disease with smoking. However, the coexistence of eosinophilic pneumonia (EP) and complicated UV remains unclear. We report a man in his 70s with chronic obstructive pulmonary disease who attended our department with ring-shaped erythema, marginal edema, and pigmentation. Additionally, a skin histological analysis showed nuclear dust and perivascular neutrophil infiltration, while a blood sample showed a decrease in C3 and C1q concentrations. Administration of prednisone temporarily improved the eruption. However, he developed a cough and a new UV eruption 1 year later. Computed tomography revealed infiltration in the right upper lobe of the lungs, and a blood sample showed a high eosinophil count. He was finally diagnosed with hypocomplementemic urticarial vasculitis syndrome and idiopathic chronic EP. A previous study showed that serum C1q concentrations in patients with EP were lower when this disease was active. Whether a decline in C1q concentrations can cause EP is unclear. However, our case is unique owing to the co-onset of EP with low complement concentrations and recurrence of UV.

Case Report: Mepolizumab in the treatment of idiopathic chronic eosinophilic pneumonia

Idiopathic chronic eosinophilic pneumonia (ICEP) is a rare interstitial lung disease of unknown cause. It usually responds well to systemic corticosteroid therapy, but relapses are frequent. We describe two cases of 21- and 27-year-old patients, presenting with dyspnea. The diagnosis of steroid-relapsing and steroid-dependent ICEP was made respectively. Mepolizumab was prescribed to both patients. This treatment resulted in successful long-term disease management with much fewer side effects than a traditional corticosteroid therapy.

The role of flexible bronchoscopy in the diagnostic pathway of children with unexplained peripheral eosinophilia.

Many possible underlying causes can be seen in children with unexplained peripheral eosinophilia (UPE) with suspected pulmonary involvement. Here, we aimed to demonstrate the clinical characteristics and diagnoses of children evaluated for UPE who underwent diagnostic bronchoscopy. Thirty-one children who underwent diagnostic bronchoscopy for UPE between 2006-2018 were included. Demographic characteristics, bronchoscopy findings and final diagnosis, and treatments were evaluated. The median age at the time of diagnosis was 5 (0.25-17.5) years. The final diagnoses of 26 patients were as follows: immunodeficiency (n=6); hyper-IgE syndromes (n=4), DOCK8 deficiency + HES (idiopathic hypereosinophilic syndrome; n=1), and severe combined immunodeficiency (n=1), HES (n=3), idiopathic chronic eosinophilic pneumonia (ICEP; n=3), idiopathic pulmonary hemosiderosis (n=1), B cell lymphoma (n=1). In one child, an integrin α3 mutation + cytomegalovirus (CMV) pneumonia was detected. Congenital pneumonia was found in one patient, and parasitic infection in another. In two, eosinophilia was attributed to underlying asthma and atopy. In four, the underlying reasons could not be elucidated. Two children with HES and one with ICEP were lost to follow-up. There was no significant relationship between the peripheral eosinophil count at the time of diagnosis and the percentage of bronchoalveolar lavage eosinophils (BAL). Bronchoscopy contributed to the management of 14 (53.9%) patients. Bronchoscopy has potential diagnostic contribution in patients with UPE suspected of having pulmonary involvement. Numerous various underlying causes may be present in this patient group. There is no relation between peripheral eosinophil count and BAL eosinophil percentage.

Targeting IL-5/5R for the treatment of idiopathic chronic eosinophilic pneumonia

Targeting IL-5/5R for the treatment of idiopathic chronic eosinophilic pneumonia

Anti-IL-5 Agents for the Treatment of Idiopathic Chronic Eosinophilic Pneumonia: A Case Series.

PurposeIdiopathic chronic eosinophilic pneumonia (ICEP) is a rare, chronic respiratory disease. Corticosteroid therapy is effective for ICEP, but relapse is frequent after its tapering, which leads to chronic use and corticosteroid-related adverse effects. Currently, biological agents targeting interleukin 5 (IL-5) are considered alternatives for treating ICEP patients with frequent relapse, but the detailed effects are not fully understood.Patients and MethodsThe clinical characteristics of 30 patients with ICEP, especially 12 patients with ICEP who experienced relapse after corticosteroid dose tapering, were evaluated retrospectively. In addition, 4 ICEP patients with frequent relapse treated by IL-5-targeted biological agents were reviewed.ResultsOf the 30 patients diagnosed with ICEP, 12 patients (40.0%) recurred after corticosteroid dose tapering, and 9 (30.0%) were treated with maintenance doses of corticosteroid. Of ICEP patients who experienced recurrence, 6 (50.0%) had frequent relapses (2 or more times). All 4 patients treated with anti-IL-5 agents had their corticosteroid dose reduced without any relapses; in 3 patients, corticosteroids were withdrawn.ConclusionAnti-IL-5 agents might be alternatives for treating ICEP patients with frequent relapses.

Idiopathic Chronic Eosinophilic Pneumonia Evolving to Pulmonary Fibrosis: A Retrospective Analysis.

Patients with idiopathic chronic eosinophilic pneumonia (ICEP) may have pulmonary fibrosis. To investigate the predictors of pulmonary fibrosis in ICEP, to describe the timeline of pulmonary fibrosis after ICEP diagnosis, and to detail the radiologic pattern of fibrosis. A retrospective computer-assisted search was performed to identify patients with ICEP seen at Mayo Clinic in Rochester, Minnesota, from January 1, 1997, through September 1, 2019. Patients with follow-up chest computed tomography (CT) beyond 12 months after the ICEP diagnosis were included in the study. Demographic, clinical, radiologic, and histopathologic characteristics were analyzed. Proportional hazards regression was used to assess the predictors of pulmonary fibrosis. We identified 62 patients (mean [SD] age at ICEP diagnosis, 60 [13] years; female sex, 37 [60%]). Cough (87%) and shortness of breath (85%) were the most common presenting symptoms. Of patients, 27 (44%) had a history of smoking and 27 (44%) had a history of asthma. During follow-up, 23 patients (37%) had CT evidence of pulmonary fibrosis, of whom 16 patients (70%) had a CT pattern inconsistent with usual interstitial pneumonia. In 29% of the patients, the CT evidence of pulmonary fibrosis developed within 2 years after ICEP. Age and male sex were predictors of pulmonary fibrosis. Of note, a history of asthma decreased the likelihood of pulmonary fibrosis. Development of pulmonary fibro-sis is not uncommon in patients with ICEP, especially older men, and is associated with increased risk of death.

IDIOPATHIC CHRONIC EOSINOPHILIC PNEUMONIA EVOLVING TO PULMONARY FIBROSIS: A RETROSPECTIVE ANALYSIS

TOPIC: Diffuse Lung Disease TYPE: Original Investigations PURPOSE: Some patients with idiopathic chronic eosinophilic pneumonia (ICEP) may develop pulmonary fibrosis. This study was conducted to investigate the predictors of fibrosis developing in patients with ICEP. METHODS: A retrospective, computer-assisted search was performed to identify patients with ICEP seen at Mayo Clinic, Rochester from 1997-2019. Those patients who had follow-up chest CT beyond 12 months after the ICEP diagnoses were included in the study. Demographics, clinical, radiologic, and histopathologic features were analyzed. Proportional hazards regression was used to assess the predictors of pulmonary fibrosis. RESULTS: We identified 68 patients; mean±SD age at the time of ICEP diagnosis was 56.4±13.2 years, 60% female. Cough (87%) followed by shortness of breath (84%) and wheezing (35%) were the most common presenting symptoms. 41% had history of smoking and 46% had history of asthma. During follow-up 29 patients (43%) developed CT evidence of pulmonary fibrosis; 76% of these patients had a CT pattern inconsistent with usual interstitial pneumonia (UIP) pattern. The CT evidence of pulmonary fibrosis developed within 2 years after the diagnosis of ICEP in the majority of these patients. Age, body mass index (BMI) and male sex were the predictors of pulmonary fibrosis. On the other hand, history of asthma decreased the likelihood of developing pulmonary fibrosis. CONCLUSIONS: Development of pulmonary fibrosis is not uncommon in patients with ICEP, especially in older males with high BMI, and is associated with an increased risk of mortality. CLINICAL IMPLICATIONS: The clinical implication of our study is to recognize this phenomenon and to keep following up patients with ICEP especially those with risk factors for pulmonary fibrosis. Early institution of prednisone should be considered. DISCLOSURES: No relevant relationships by Misbah Baqir, source=Web Response No relevant relationships by Tucker Johnson, source=Web Response No relevant relationships by Tobias Peikert, source=Web Response No relevant relationships by Jay Ryu, source=Web Response No relevant relationships by Darrell Schroeder, source=Web Response No relevant relationships by Joanne Yi, source=Web Response

Evaluating the relationship between high-resolution computed tomography findings and their extent in eosinophilic lung diseases with peripheral blood eosinophil level.

PurposeEosinophilic lung diseases are a group of pulmonary disorders associated with peripheral or tissue eosinophilia. They can be classified into primary (idiopathic) and secondary groups based on their aetiology. On the other hand, blood eosinophilia is a common clinical problem with various causes. In many cases the lung infiltrations in patients with peripheral eosinophilia are non-eosinophilic. Herein, we aim to assess the relationship between blood eosinophilia and abnormal high-resolution computed tomography (HRCT) features and their extent in idiopathic chronic eosinophilic pneumonia (ICEP) (the most common form of idiopathic eosinophilic lung disease). This can help in differentiating eosinophilic from non-eosinophilic lung infiltrations in patients with blood eosinophilia.Material and methodsIn this descriptive-correlational study, all patients with proven ICEP, who were referred to Masih Daneshvari Hospital, Tehran, Iran from 2012 to 2019, were included. The ICEP diagnosis was based on lung infiltrations on imaging, in addition to increased numbers of eosinophils in bronchoalveolar lavage fluid, blood or lung biopsy samples, and rapid response to corticosteroids. Patients with known aetiologies for eosinophilic lung diseases were excluded. The HRCT findings and their extent in each patient were compared with the blood eosinophil level.ResultsPositive correlation was found only between blood eosinophil level and frequency and extent of consolidation, and with frequency of lymphadenopathy.ConclusionsHRCT can help to differentiate eosinophilic from non-eosinophilic lung infiltrations in patients with blood eosinophilia by comparing the extent of consolidation with the blood eosinophil level.

Real-Life Study of Mepolizumab in Idiopathic Chronic Eosinophilic Pneumonia.

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. 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). We included ten patients in the final analysis, with a median follow-up of 9months 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. 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.

Elevated Levels of Intelectin-1, a Pathogen-binding Lectin, in the BAL Fluid of Patients with Chronic Eosinophilic Pneumonia and Hypersensitivity Pneumonitis.

Objective Human intelectin-1 (hITLN-1) binds to galactofuranosyl residues, which are present in the microbial cell wall, but which are absent in mammalian tissues, and has been suggested to play an immunological role against microorganisms. However, the involvement of hITLN-1 in the pathogenesis of diffuse pulmonary diseases remains unknown. The aim of this study was to compare the hITLN-1 concentrations in the bronchoalveolar lavage (BAL) fluid of patients with diffuse pulmonary diseases. Methods The cell components and concentrations of hITLN-1 were analyzed in the BAL fluid of 8 patients with idiopathic chronic eosinophilic pneumonia (ICEP), 3 patients with drug-induced eosinophilic pneumonia, 4 patients with hypersensitivity pneumonitis (HP), 11 patients with sarcoidosis, 9 patients with cryptogenic organizing pneumonia, and 5 patients with idiopathic fibrosing interstitial pneumonia (fibrosing nonspecific interstitial pneumonia or usual interstitial pneumonia). Results The hITLN-1 concentrations in the BAL fluid of patients with ICEP and HP were higher than in those with other diseases. In the ICEP group, no significant difference was observed in the hITLN-1 concentrations of patients with or without a history of bronchial asthma. Conclusion The results of the present study suggest that hITLN-1 may be involved in the pathogenesis of ICEP and HP, and that an increase in the hITLN-1 concentration in the BAL fluid may represent a new biomarker for these diseases.

Chronic Eosinophilic Pneumonia Masquerading as Malignant Mesothelioma

SESSION TITLE: Diffuse Lung Disease SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Chronic eosinophilic pneumonia is a rare idiopathic disorder of increased eosinophils within lung interstitium and alveoli. This pleural-predominant disease can be mistaken for mesothelioma in persons with known asbestos exposure. CASE PRESENTATION: A 76 year old man was referred for worsening asthma. He served in the Navy and worked construction with suspected exposure to asbestos 50 years prior. He had dyspnea on exertion and a nonproductive cough for years which had progressed. He denied weight loss, fevers, night sweats, chest pain, or hemoptysis. Lung auscultation revealed decreased breath sounds over the right lower lobe. He demonstrated exercise induced hypoxia on a walk test and was started on 4 lpm oxygen (1,008 feet). Pulmonary function testing showed moderately-severe restriction with moderate reduction in diffusion capacity (FEV1 52%, FVC 55%, TLC 57%, DLCO 56%). A CT thorax revealed bilateral pleural effusions with increased pleural thickness on the right, pleural plaques bilaterally, and a right pleural nodular opacity. Thoracentesis (250cc) revealed 10% eosinophilia without malignant cells or infection. Bronchoscopy showed thick mucus throughout the tracheobronchial tree with 31% eosinophils on bronchoalveolar lavage fluid. Peripheral eosinophilia was present at 1.4x103/uL. IgE and ANCA were negative. He underwent PET/CT scan which showed mild uptake (SUV 3.0) at the right pleural nodule and within the right pleural effusion. CT guided needle biopsy of the pleural nodule revealed chronic inflammation with increased eosinophilia but no granulomas or malignant cells. He was treated with prednisone 1mg/kg for 4 weeks. On return visit, his cough and dyspnea had resolved and repeat walk test revealed reduced oxygen requirement of 2 lpm (1,206 feet). DISCUSSION: The differential diagnosis for pleural fluid eosinophilia includes malignancy and eosinophilic pneumonia among other etiologies. Given our patient’s possible asbestos exposure, CT findings, and pleural fluid eosinophils, mesothelioma was suspected. However, peripheral eosinophilia and bronchoalveolar lavage fluid eosinophils are uncommon in mesothelioma which prompted a tissue biopsy that revealed accumulation of eosinophils without malignant cells. CONCLUSIONS: Chronic eosinophilic pneumonia can present with nonspecific symptoms and pleural-based CT findings similar to mesothelioma. Reference #1: Marchand E, Renaud-Gaubert M, Lauque D. Idiopathic Chronic Eosinophilic Pneumonia A Clinical and Follow-Up Study of 62 Cases. Medicine. 1998;77(5):299-312. DISCLOSURE: The following authors have nothing to disclose: Joseph Carrington, Candace Huebert No Product/Research Disclosure Information

Chronic Eosinophilic Pneumonia: Adjunctive Therapy With Inhaled Steroids

Abstract Idiopathic chronic eosinophilic pneumonia (ICEP) is a rare form of diffuse parenchymal lung disease first identified by Carrington et al. in 1969. It is characterized by the presence of constitutional and respiratory symptoms with associated peripheral opacities on imaging and elevated serum and/or bronchoalveolar eosinophilia. Although data is limited regarding etiology or prevalence, it is known that ICEP has a 2:1 female: male predominance and typically affects non-smokers. Diagnosis rests on the clinical constellation of respiratory symptoms of at least 2–4 weeks duration, the presence of diffuse pulmonary alveolar consolidation, classically described as the “photographic negative of pulmonary edema”, the presence of eosinophils ≥40% on bronchoalveolar lavage or ≥1000/mm3 eosinophils on peripheral blood and the exclusion of other known causes of eosinophilic lung diseases such as drugs, toxins, fungi, parasites, and collagen-vascular disorders. A dramatic response is achieved with systemic corticosteroids, which is typically dosed over 6 months to 1 year. Despite this response, approximately 30–50% of patients will relapse upon cessation of steroids or during the taper. Although these patients respond well to another trial of steroids, the side effects of long term steroids are well known, including osteoporosis, diabetes, hypertension and cataracts. Inhaled corticosteroids as monotherapy has been trialed in the past without success. However, we report a case of a patient who underwent treatment with systemic corticosteroids followed by inhaled steroids who has remained in remission for 2 years.

Yellow Broncho-alveolar Lavage Fluid in Eosinophilic Pneumonia.

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).

Eosinophilic pneumonias in children: A review of the epidemiology, diagnosis, and treatment.

Pediatric eosinophilic pneumonias (EPs) are characterized by a significant infiltration of the alveolar spaces and lung interstitium by eosinophils, with conservation of the lung structure. In developed countries, EPs constitute exceptional entities in pediatric care. Clinical symptoms may be transient (Löffler syndrome), acute (<1 month and mostly <7 days), or chronic (>1 month). Diagnosis relies on demonstration of alveolar eosinophilia on bronchoalveolar lavage, whether or not associated with blood eosinophilia. EPs are a heterogeneous group of disorders divided into: (i) secondary forms (seen mainly in parasitic infections, allergic bronchopulmonary aspergillosis, and drug reactions); and (ii) primary forms (eosinophilic granulomatosis with polyangiitis, hypereosinophilic syndrome, idiopathic chronic eosinophilic pneumonia, and idiopathic acute eosinophilic pneumonia). Despite their rarity, the etiological approach to EP must be well-defined as some causes can be rapidly life-threatening without initiation of the proper treatment. This approach (i) eliminates secondary forms, with comprehensive history taking and minimal biological assessment, (ii) is oriented in primary forms by the acute or chronic setting, and the existence of extrapulmonary symptoms. Treatment of primary forms has traditionally relied on corticosteroids, usually with a dramatic response. Specific treatments or the adjunction of corticosteroid-sparing treatment or immunosuppressors are currently being evaluated in order to improve the prognosis and the side effects associated with corticosteroid treatment in a pediatric setting.

Platelet activation markers overexpressed specifically in patients with aspirin-exacerbated respiratory disease

Aspirin-exacerbated respiratory disease (AERD) is characterized by respiratory reactions on ingestion of COX-1 inhibitors and cysteinyl leukotriene overproduction. The hypersensitivity reaction is induced by low doses of aspirin that inhibit COX-1 in platelets. We sought to explore the role of platelets in the pathogenesis of AERD in patients under stable conditions and during an aspirin challenge test. Stable patients with AERD (n= 30), aspirin-tolerant asthma (ATA; n= 21), or idiopathic chronic eosinophilic pneumonia (n= 10) were enrolled. Platelet activation was estimated based on expression levels of P-selectin (CD62P), CD63, CD69, and GPIIb/IIIa (PAC-1) in peripheral platelets; percentages of circulating platelet-adherent leukocytes; and plasma levels of soluble P-selectin (sP-selectin) and soluble CD40 ligand (sCD40L). In the stable condition, expression of all surface markers on platelets, the percentage of platelet-adherent eosinophils, and the plasma levels of sP-selectin and sCD40L were significantly higher in patients with AERD compared with those in patients with ATA. P-selectin and CD63 expression on platelets and plasma sP-selectin and sCD40L levels were positively correlated with the percentage of platelet-adherent eosinophils. Among these markers, P-selectin expression and plasma sP-selectin levels positively correlated with urinary concentrations of leukotriene E4. Additionally, plasma sP-selectin and sCD40L levels were negatively correlated with lung function. In contrast, platelet activation markers in patients with AERD did not change during the aspirin challenge test. Peripheral platelets were activated more in patients with stable AERD compared with those in patients with stable ATA, patients with idiopathic chronic eosinophilic pneumonia, and control subjects. Platelet activation was involved in cysteinyl leukotriene overproduction and persistent airflow limitations in patients with AERD.

Idiopathic eosinophilic pneumonia in children: the French experience

BackgroundIdiopathic 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.MethodsWe retrospectively analyzed all cases of ICEP and IAEP in children that were retrieved from French Reference Centers for rare pediatric lung diseases.ResultsFive 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.ConclusionICEP 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.