Combined pulmonary fibrosis and emphysema in systemic sclerosis: A syndrome associated with heavy morbidity and mortality.

Background: The syndrome of combined pulmonary fibrosis and emphysema (CPFE) primarily due to tobacco smoking has been reported in connective tissue disease, but little is known about its characteristics in systemic sclerosis (SSc).

Methods: In this retrospective multi-center case-control study, we identified 36 SSc patients with CPFE, and compared them with 72 SSc controls with interstitial lung disease (ILD) without emphysema.

Results: Rate of CPFE in SSc patients with CT scan was 3.

Pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia: Correlations between computed tomography findings and cerebral complications.

Objectives: Computed tomography (CT) is the modality of choice to characterise pulmonary arteriovenous malformations (PAVMs) in patients with hereditary haemorrhagic telangiectasia (HHT). Our objective was to determine if CT findings were associated with frequency of brain abscess and ischaemic stroke.

Methods: This retrospective study included patients with HHT-related PAVMs.

Pulmonary Arterial Hypertension Associated With Systemic Lupus Erythematosus: Results From the French Pulmonary Hypertension Registry.

Background: Pulmonary arterial hypertension (PAH) is a rare complication of systemic lupus erythematosus (SLE).

Methods: We identified all patients with SLE and PAH (SLE-PAH) who were enrolled in the French Pulmonary Hypertension Registry with a diagnosis confirmed by right heart catheterization (RHC). A control group of 101 patients with SLE without known PAH was selected from SLE expert centers participating in the Pulmonary Hypertension Registry.

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.

Inflammatory bowel diseases in anti-neutrophil cytoplasmic antibody-associated vasculitides: 11 retrospective cases from the French Vasculitis Study Group.

Objective: Coexistence of ANCA-associated vasculitis (AAV) and IBD is a rare condition that is rarely described in the literature. The aim of the study was to describe the main characteristics of patients presenting with both IBD and AAV.

Methods: A retrospective study of AAV patients in the French Vasculitis Study Group cohort who also had a diagnosis of IBD was conducted.

Home-based pulmonary rehabilitation in idiopathic pulmonary fibrosis.

Introduction: Idiopathic pulmonary fibrosis (IPF) is a severe chronic lung disease. Pulmonary rehabilitation could improve the quality of life of patients with this condition.

Methods: We prospectively evaluated the impact of an 8-week home-based pulmonary rehabilitation programme over 10 months in stable patients suffering from IPF.

Effects of oxygen on exercise-induced increase of pulmonary arterial pressure in idiopathic pulmonary fibrosis.

Introduction: Idiopathic pulmonary fibrosis (IPF) is a severe disease with no known effective therapy. Patients with IPF may develop severe increase of pulmonary arterial pressure (PAP) on exercise, the mechanisms of which is not clearly identified.

Objectives: To determine whether oxygen may correct the increase of PAP developed during exercise in patients with IPF.

[Home-based pulmonary rehabilitation in idiopathic pulmonary fibrosis].

Introduction: Idiopathic pulmonary fibrosis (IPF) is a severe chronic lung disease. Pulmonary rehabilitation could improve the quality of life of patients with this condition.

Methods: We prospectively evaluated the impact of an 8 week home-based pulmonary rehabilitation program over 10 months in stable patients suffering from IPF.

Right-to-left shunt with hypoxemia in pulmonary hypertension.

Background: Hypoxemia is common in pulmonary hypertension (PH) and may be partly related to ventilation/perfusion mismatch, low diffusion capacity, low cardiac output, and/or right-to-left (RL) shunting.

Methods: To determine whether true RL shunting causing hypoxemia is caused by intracardiac shunting, as classically considered, a retrospective single center study was conducted in consecutive patients with precapillary PH, with hypoxemia at rest (PaO2 < 10 kPa), shunt fraction (Qs/Qt) greater than 5%, elevated alveolar-arterial difference of PO2 (AaPO2), and with transthoracic contrast echocardiography performed within 3 months.

Results: Among 263 patients with precapillary PH, 34 patients were included: pulmonary arterial hypertension, 21%; PH associated with lung disease, 47% (chronic obstructive pulmonary disease, 23%; interstitial lung disease, 9%; other, 15%); chronic thromboembolic PH, 26%; miscellaneous causes, 6%.

Persistent airflow obstruction in asthma of patients with Churg-Strauss syndrome and long-term follow-up.

Background: Little is known about the long-term outcome of airflow obstruction in asthma of patients with Churg-Strauss syndrome (CSS).

Methods: We conducted a retrospective study of 24 consecutive patients (aged 41.1 +/- 13.

[Pulmonary hypertension in chronic respiratory and cardiac diseases].

Pulmonary hypertension (PH) is frequent in the course of chronic respiratory diseases with hypoxia which leads to vascular remodeling, especially chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis. PH is usually moderate but may be disproportionate in some patients. In the chronic diseases of the left heart, increased atrial pressure results in pulmonary venous distension and passive PH (post-capillary) which may further lead to precapillary hypertension.

[Pulmonary amyloidosis and non-amyloid immunoglobulin deposits].

The amyloidoses are characterised histopathologically by the tissue deposition of fibrillar amyloid, specifically stained by Congo red and birefringent under polarised light. This characteristic is linked to a beta-folded structural configuration that is the common denominator of the amyloidoses which may have more than twenty distinct protein precursors. The most common is AL amyloidosis which is of immunoglobulin origin.

[Severe phenotypes in sarcoidosis pulmonary involvement].

Severe forms of pulmonary sarcoidosis are not frequent and include stage IV pulmonary involvement and specific complications of intrathoracic sarcoidosis. Pulmonary hypertension may be due to granulomatous involvement of vessels and/or extrinsic compression of pulmonary arteries by enlarged lymph nodes. Bronchial stenosis is rare and delay in therapy usually result in corticoresistance.

[Pulmonary manifestations of the vasculitides].

The vasculitides involving the lower respiratory tract are mainly the small vessels vasculitides associated with antineutrophil cytoplasmic autoantibodies including Wegener granulomatosis (WG), microscopic polyangiitis (MPA), and Churg-Strauss syndrome (SCS). WG pulmonary manifestations mainly consist of nodules and pneumonias often cavitary, and tracheobronchial stenoses. MPA often consists of pulmonary-renal syndrome with alveolar hemorrhage possibly severe.

[Idiopathic pulmonary fibrosis].

Idiopathic pulmonary fibrosis is a chronic disorder characterized histopathologically by a pattern of usual interstitial pneumonia, with heterogeneous and mutilating interstitial fibrosis with foci of proliferating fibroblasts, honeycomb lung, and little if any inflammation. The diagnosis is based on a pluridisciplinary analysis of the clinical symptoms, the chest high-resolution computerized tomography features, and pathology on video-thoracoscopic lung biopsy when indicated. In half of the cases, the typical tomodensitometric pattern allows to make a confident diagnosis without a lung biopsy.

[Interstitial lung disease in connective tissue diseases].

Interstitial lung disease (ILD) may occur in the connective tissue diseases, with a negative impact on survival. The diagnosis of ILD is established by crackles of the lung bases at auscultation, and high resolution computed tomography of the chest demonstrating diffuse opacities predominating in the bases (ground glass opacities, reticular opacities, honeycombing, and traction bronchiectases). Bronchoalveolar lavage mostly contributes to the differential diagnosis.

[Idiopathic pulmonary fibrosis].

Idiopathic pulmonary fibrosis (IPF) is currently defined by a set of histopathological-radiological-clinical criteria including an histopathological pattern of usual interstitial pneumonia, reticular opacities with honeycombing at tomodensitometry, progressive exercise dyspnea in a patient older than 60 years with fine crackles at lung bases at auscultation. The incidence of IPF is increasing. Risk factors for the disease have been identified (smoking, exposure to various dusts, genetic polymorphism) but the true cause of FPI remains unknown.

[Unusual pulmonary presentation of systemic Langerhans cell histiocytosis].

An 80-year-old nonsmoking man was referred to our hospital with bilateral perihilar pulmonary opacities. He had a history of epilepsy, sclerosing cholangitis, cutaneous lesions previously diagnosed as localised Langerhans cell histiocytosis. Symptoms included dry cough and dyspnea.

[Hypereosinophilic asthma].

Although moderate airway infiltration by eosinophils is present in the eosinophilic inflammatory phenotype of asthma, hypereosinophilic asthma differs in its frankly elevated levels of eosinophils in the blood, induced sputum, and bronchoalveolar lavage. Some etiologic pitfalls may be tricky especially wheezing in eosinophilic pneumonia induced by drugs or parasitic infections. Idiopathic chronic eosinophilic pneumonia is associated with asthma in most cases.