Acute eosinophilic pneumonia following artenimol-piperaquine exposure.

Acute eosinophilic pneumonia (AEP) has been reported following chloroquine or mefloquine exposure, both structurally related to piperaquine. We report a case of AEP with typical CT scan patterns, hypereosinophilia in blood (9.8 109/l), and bronchoalveolar lavage (78% of 600 000 cells/ml), 10 days after artenimol-piperaquine exposure in a 26-year-old man.

Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss).

The respiratory manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) have not been studied in detail.In this retrospective multicentre study, EGPA was defined by asthma, eosinophilia and at least one new onset extra-bronchopulmonary organ manifestation of disease.The study population included 157 patients (mean±sd age 49.

Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss).

Objective: To guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA.

Methods: Organ manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease.

Results: The study population included 157 patients (mean age 49.

Adherence to guidelines in idiopathic pulmonary fibrosis: a follow-up national survey.

A new survey coordinated by the French expert centres for rare pulmonary diseases investigated French pulmonologists' diagnostic and therapeutic practice for idiopathic pulmonary fibrosis (IPF) and explored changes since a previous survey in 2011-2012. From May 16 to August 30, 2014, 524 pulmonologists were contacted. Those following at least one patient with IPF were invited to complete a questionnaire administered by telephone or e-mail.

Long-Term Outcomes Among Participants in the WEGENT Trial of Remission-Maintenance Therapy for Granulomatosis With Polyangiitis (Wegener's) or Microscopic Polyangiitis.

Objective: Findings from the WEGENT trial and other short-term studies have suggested that azathioprine (AZA) or methotrexate (MTX) could effectively maintain remission of granulomatosis with polyangiitis (Wegener's) (GPA) or microscopic polyangiitis (MPA). This study was undertaken to examine whether differences in rates of relapse or adverse events would appear after discontinuation of these 2 maintenance regimens, when assessed over a longer followup period.

Methods: Long-term outcomes in patients enrolled in the WEGENT trial were analyzed according to their randomized treatment group (AZA or MTX).

Diagnosis and management of idiopathic pulmonary fibrosis: French practical guidelines.

Idiopathic pulmonary fibrosis (IPF) is the most frequent chronic idiopathic interstitial pneumonia in adults. The management of rare diseases in France has been organised by a national plan for rare diseases, which endorsed a network of expert centres for rare diseases throughout France. This article is an overview of the executive summary of the French guidelines for the management of IPF, an initiative that emanated from the French National Reference Centre and the Network of Regional Competence Centres for Rare Lung Diseases.

¹³¹I-labeled lipiodol-induced interstitial pneumonia: a series of 15 cases.

Background: The drug (131)I-labeled lipiodol is used as internal radiotherapy for unresectable hepatocellular carcinoma. Although the drug was considered safe during preapproval studies, we observed several cases of interstitial pneumonia following its administration.

Methods: Cases were retrospectively identified through the drug safety unit database of Rennes University Hospital.

ANCA-associated lung fibrosis: analysis of 17 patients.

In this retrospective study, we analyzed 17 patients presenting with pulmonary fibrosis and a positive ANCA testing. This group was compared with a control group of 12 patients with IPF and negative ANCA testing. Patients were 15 males and 2 females, with a mean age of 66 years.

Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients.

Objective: To assess the efficacy of systemic corticosteroids (CS) alone as first-line treatment in patients with Churg-Strauss syndrome (CSS) without poor-prognosis factors, as defined by the Five-Factors Score (FFS), and to compare the efficacy and safety of oral azathioprine (AZA) versus intravenous pulse cyclophosphamide (CYC) as adjuvant immunosuppressive therapy for treatment failure or relapse.

Methods: This multicenter, prospective, randomized, open-label therapeutic trial included 72 patients with newly diagnosed CSS (FFS of 0) treated with CS alone. At treatment failure or relapse, patients were randomized to receive 6 months of oral AZA or 6 pulses of CYC.

Increased extracellular matrix metalloproteinase inducer (EMMPRIN) expression in pulmonary fibrosis.

Extracellular matrix metalloproteinase inducer (EMMPRIN) was examined on bronchoalveolar lavage fluids (BALFs) and lung tissue from patients with fibrosis (usual interstitial pneumonia-idiopathic pulmonary fibrosis [UIP-IPF], n = 15; diffuse parenchymal lung diseases without IPF characteristics on computerized tomography scan, n = 8) and without fibrosis (n = 6). In UIP-IPF, EMMPRIN staining was increased in areas of fibrosis, mainly in macrophages and in epithelial cells. EMMPRIN was also found in the extracellular medium with significant levels in patients with lung fibrosis compared to subjects without fibrosis.

Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome.

Background: Since testing for antineutrophil cytoplasmic antibodies (ANCA) became available for routine evaluation, no large homogeneous cohort of patients with the Churg-Strauss syndrome has been studied.

Objective: To define the clinical and biological characteristics of newly diagnosed Churg-Strauss syndrome, according to the presence or absence of ANCA.

Design: Cross-sectional analysis of manifestations of participants who were enrolled in treatment trials between December 1995 and December 2002.

[Pulmonary alveolar phospholipoproteinosis].

Pulmonary alveolar phospholipoproteinosis is a rare lung disease of unknown cause characterized by surfactant plugging of the alveoli. At the present time, surgical lung biopsy, long considered as the gold standard, is not necessary for positive diagnosis of pulmonary alveolar phospholipoproteinosis when computed tomography anomalies and analysis of bronchial lavage fluids present a typical pattern. Treatment requires abundant lavage, but the demonstration of anti-GM-CSF antibodies in primary forms opens new therapeutic perspectives.

Survival of patients with bronchiectasis after the first ICU stay for respiratory failure.

Study Objectives: Respiratory failure (RF) is a frequent cause of death among patients with bilateral bronchiectasis. An ICU admission is commonly required, and neither short-term or long-term outcomes have been studied.

Design: We performed a retrospective study over a 10-year period (January 1990 to March 2000).