Idiopathic chronic eosinophilic pneumonia (CEP) is a rare disorder of unknown cause with nonspecific respiratory and systemic symptoms but rather characteristic peripheral alveolar infiltrates on imaging, developing mainly in women and in atopic subjects. The disorder is highly responsive to oral corticosteroid therapy, but relapses are frequent on reducing or stopping treatment. The long-term course of the disease and data regarding outcome, particularly the need for prolonged oral corticosteroid therapy and the development of severe asthma, are somewhat contradictory. A multicentric retrospective study was conducted in an attempt to describe better the initial features and, above all, the later course of CEP in a large homogeneous series of 62 stringently selected patients of whom 46 were followed for more than 1 year. The prevalence of smokers was low (6.5%) and about half of our patients (51.6%) had a previous, and often prolonged, history of asthma. The clinical and roentgenographic features were in keeping with previous studies, but we found that computed tomography could disclose ground glass opacities not detected by X-ray, and that migratory infiltrates before treatment were more frequent (25.5%) than reported previously. The bronchoalveolar lavage cellular count always showed a striking eosinophilic pattern, thus allowing distinction between CEP and cryptogenic organizing pneumonia, both syndromes sharing many common clinical and imaging features. About two-thirds of the patients (68%) showed a ventilatory defect in pulmonary function tests, with about one-half of these presenting with an obstructive pattern, sometimes without previous asthma. Along with the submucosal eosinophilic infiltration noted in 2 patients without ventilatory defect, this is strong evidence to confirm that CEP is not only an alveolointerstitial but also an airway disease. The dramatic response to oral corticosteroid therapy was observed in all treated patients. Although only 1 patient initially treated for less than 6 months did not relapse, longer oral corticosteroid therapy in no way provided protection from further relapses. We thus propose to try to wean oral corticosteroid therapy after 6 months in patients without severe asthma, because recurrences remain responsive to oral steroids. However, prolonged oral corticosteroid therapy was necessary in the majority of patients, with 68.9% of those followed for more than 1 year still on oral corticosteroid therapy at the last follow-up, either because of relapse or because of severe asthma.

Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder characterized by epistaxis, telangiectasia, and visceral vascular manifestations. Infectious and ischemic central nervous system (CNS) manifestations due to embolism through pulmonary arteriovenous malformations (PAVMs) represent the main causes of morbidity. To improve the phenotypic characterization of HHT with PAVM, we conducted a retrospective multicenter study of patients with HHT and at least 1 PAVM detected by chest computed tomography (CT) and/or pulmonary angiography, with particular attention to CNS and infectious manifestations. The study included 126 patients (47 men, 79 women), with a mean age of 43.1 ± 17.4 years; 45 patients had a mutation of the ENG gene and 16 had a mutation of ACVRL1. PAVMs were diagnosed as a result of systematic screening procedures (29%), incidental imaging findings (15%), dyspnea (22%), or CNS symptoms (13%). The PAVMs were diagnosed at a mean age of 43 ± 17 years, with a linear distribution of diagnosis between 20 and 75 years. Dyspnea on exertion was present in 56% of patients. Four patients had a hemothorax, including 1 during pregnancy. Fifty-three CNS events directly related to HHT (excluding migraine) were observed in 35% of patients: cerebral abscess (19.0%), ischemic cerebral stroke (9.5%), transient cerebral ischemic attack (6.3%), and cerebral hemorrhage (2.4%). The median age of onset was 33 years for cerebral abscesses (range, 11-66 yr), and 53.5 years for ischemic cerebral events (range, 2-72 yr). Migraine was reported in 16% of patients. The diagnoses of PAVM and HHT were made at the time of the cerebral abscess in 13 cases (54%). Forty-three percent of patients were hypoxemic at rest. Contrast echocardiography showed intrapulmonary right-to-left shunting in 87% of tested patients. PAVMs were seen on chest radiograph in 54% of patients, and on the CT scan in all patients. One hundred five patients (83%) underwent treatment of the PAVM, by percutaneous embolization (71%) and/or by surgical resection (23%). A high frequency of CNS and infectious complications was observed in this large series of patients with HHT-related PAVM. Physicians may not be sufficiently aware of the clinical manifestations of this orphan disorder. Patients diagnosed with HHT should be informed by physicians and patient associations of the risk of PAVM-related complications, and systematic screening for PAVM should be proposed, regardless of a patient's symptoms, familial history, or genetic considerations.

The characteristics of patients with rheumatoid arthritis (RA) who develop obliterative bronchiolitis characterised by severe airflow obstruction have been hitherto poorly investigated.A retrospective study of 25 patients with RA and functional evidence of obliterative bronchiolitis (forced expiratory volume in one second (FEV₁)/forced vital capacity (FVC) 140% predicted) was conducted.Patients (mean±sdage 64±11 yrs) included 17 never-smokers and eight ex-smokers (10.5±5.4 pack-yrs). The diagnosis of RA preceded respiratory symptoms in 88% of cases. Dyspnoea on exertion was present in all patients and bronchorrhea in 44%. High-resolution computed tomography findings included: bronchial wall thickening (96%), bronchiectasis (40%), mosaic pattern (40%), centrilobular emphysema (56%), and reticular and/or ground-glass opacities (32%). Pulmonary function tests showed: FEV₁41±12% pred, FEV₁/FVC 49±14%, FVC 70±20% pred, RV 148±68% pred and RV/TLC 142±34% pred. Lung biopsy, available in nine patients, demonstrated constrictive, follicular and mixed bronchiolitis. Patients were followed for 48.2±49 months. Treatment was poorly effective. Chronic respiratory failure occurred in 40% of patients, and four patients died.Obliterative bronchiolitis associated with rheumatoid arthritis is a severe and under-recognised condition leading to respiratory failure and death in a high proportion of patients.

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 (56versus23%) and had a lower number of relapses per year of follow-up (median 0versus0.24). Moreover, they were treated more frequently with long-term inhaled corticosteroids (88versus31%) 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.