Methods At age 7, she developed autoimmune hepatitis. She was diagnosed with lupus nephritis (class II + V) from pathological finding and pulmonary hypertension (PH) at age 10, and treated with immunosuppressive therapy (methylprednisolone pulse therapy, cyclophosphamide pulse therapy and mycophenolate mofetil) and home oxygen therapy at night. Comprehensive examination about PH was carried out...

BACKGROUND Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease associated with high morbidity and mortality. Effective treatments for IPF are limited. Several recent studies have investigated novel therapeutic agents for IPF, but very few have addressed their comparative benefits and harms. METHODS We performed a Bayesian network meta-analysis (NMA) to assess the effects of di...

BACKGROUND Present guidelines for the diagnosis of idiopathic pulmonary fibrosis require histological confirmation of surgical lung biopsy samples when high-resolution CT images are not definitive for usual interstitial pneumonia. We aimed to assess the predictive value of high-resolution CT in a cohort of patients with suspected idiopathic pulmonary fibrosis from a previous randomised trial. ...

We report a case of neonatal-onset autoinflammatory syndrome that resembles Nakajo-Nishimura syndrome (NNS), a rare autoinflammatory syndrome caused by a homozygous PSMB8 mutation. The patient is a 7-yearold boy who demonstrated clinical findings that differ from those of typical NNS. Exudative erythemas on his face, trunk, and extremities developed 2 weeks after birth. At 1 month of age, the p...

TOPIC: Pulmonary Vascular Disease TYPE: Fellow Case Reports INTRODUCTION: Unilateral absence of pulmonary artery (UAPA) is a rare disorder that can present as an isolated lesion or in association with other congenital heart defects. An important complication UAPA the development hypertension (PH). Herein, we discuss patient severe PH setting and highlight hemodynamic response to combination vas...

Background Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis with high mortality. Although new drugs and treatment strategies developed in the last years, prognosis prevalent patients has not been sufficiently improved. Objectives We aimed to analyze prevalence, mortality, prognostic factors SSc-PAH from our single-center SSc cohort. Methods retrospectively ev...

Pulmonary artery (PA) aneurysm is sometimes found in patients with pulmonary arterial hypertension (PAH), and PA dissection is a rare but life-threatening complication. The pathological background of PA aneurysms and dissection remains poorly understood. A 29-year-old man was diagnosed with PAH associated with patent ductus arteriosus (PDA; Movie I in the Data Supplement) and was in World Healt...