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.

[Opportunistic infections and sarcoidosis].

Introduction: In spite of CD4+ T-lymphocytopenia and corticosteroids-induced immune suppression, the risk of opportunistic infection is not usually considered to be increased in sarcoidosis.

Methods: We describe 5 cases of opportunistic infection in patients with sarcoidosis and CD4+ T- lymphocytopenia. A systematic review of the literature was done.

Improvement of refractory rheumatoid arthritis-associated constrictive bronchiolitis with etanercept.

Rheumatoid arthritis (RA)-associated constrictive bronchiolitis is a severe condition with no established efficient treatment. A 55-year-old woman with seropositive RA developed rapidly progressive constrictive bronchiolitis confirmed by lung biopsy. Her clinical condition worsened despite steroids and azathioprine.

[Orphan lung diseases: from curiosity to concern].

As their name implies, orphan diseases are rare, often poorly studied diseases. Patients with orphan diseases are often deprived of adequate diagnosis and treatment. To promote a comprehensive study of orphan pulmonary diseases, we established a network of pulmonologists, allowing us to collect and study several series of patients.