Juvenile dermatomyositis is the leading cause of chronic idiopathic inflammatory myopathy of auto-immune origin in children. Lung involvement in inflammatory myopathies is well described in adults, involving mostly interstitial lung disease, aspiration pneumonia and alveolar hypoventilation. We propose to describe its specificities in children. Pulmonary involvement may be asymptomatic and therefore must be systematically screened for. In case of clinical or functional respiratory abnormality, a chest computed tomographic (CT) scan is necessary. In children, a decrease of respiratory muscle strength seems common and should be systematically and specifically searched for by non-invasive and reproducible tests (sniff test). Interstitial lung disease usually associates restrictive functional defect, impairment of carbon monoxide diffusion and interstitial lung disease on CT scan. As in adults, the first-line treatment of juvenile dermatomyositis is based on corticosteroids. Corticosteroid resistant forms require corticosteroid bolus or adjuvant immunosuppressive drugs (methotrexate or cyclosporine). There is no consensus in pediatrics for the treatment of diffuse interstitial lung disease. Complications of treatment, including prolonged steroid therapy, are frequent and therefore a careful assessment of the treatments risk-benefit ratio is necessary, especially in growing children.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1016/j.revmed.2014.04.005 | DOI Listing |
Rev Mal Respir
March 2025
Service de pneumologie, CHU de la Guadeloupe, 97110 Pointe-à-Pitre, France.
Granulomatosis with polyangiitis (GPA) was diagnosed in a 65-year-old Afro-Caribbean patient presenting initially with hearing loss and a pseudo-tumoral 6cm lung mass. Lung biopsy findings favored the diagnosis of vasculitis. Rapid disease progression was noted with near-complete deafness and lack of speech, severe renal failure necessitating dialysis, and persisting disturbance of consciousness following tonic-clonic seizures due to posterior reversible encephalopathy syndrome (PRES).
View Article and Find Full Text PDFClin Lung Cancer
February 2025
Department of Radiation Oncology, Shanghai Pulmonary Hospital, Tongji University Medical School Cancer Institute, Tongji University School of Medicine, Shanghai, China. Electronic address:
Purpose: This study aimed to investigate the risk factors for severe radiation pneumonitis (RP) after thoracic radiotherapy (RT) in patients with locally advanced non-small cell lung cancer (NSCLC), develop a prediction model to identify high-risk groups, and investigate the impact of severe RP on overall survival (OS).
Methods: We retrospectively collected clinical, dosimetric, and hematological factors of patients with stage III NSCLC receiving thoracic RT without immunotherapy. The primary and secondary end points were severe RP and OS, respectively.
Respir Med
March 2025
Respiratory Research and Rehabilitation Laboratory (Lab3R), School of Health Sciences (ESSUA), University of Aveiro, Aveiro, Portugal; Institute of Biomedicine (iBiMED), University of Aveiro, Aveiro, Portugal. Electronic address:
Aim: To explore the feasibility of integrating palliative care education in pulmonary rehabilitation (PR).
Methods: A mixed-methods feasibility study was conducted in people with chronic respiratory diseases enrolled in 12-weeks outpatient hospital-based PR. A session about palliative care was integrated in the education content.
Respir Investig
March 2025
Department of Biotechnology, Brainware University, Barasat, Kolkata, West Bengal, 700125, India.
Hypersensitivity pneumonitis (HP) and sarcoidosis are granulomatous interstitial lung diseases with overlapping clinical and immunological features, posing diagnostic and therapeutic challenges. This review offers a comprehensive assessment of their immune mechanisms, etiology, and pathogenesis. HP is predominantly triggered by exposure to environmental antigens, while sarcoidosis involves an exaggerated immune response to elusive antigens.
View Article and Find Full Text PDFRespir Investig
March 2025
Department of Health Science, Kio University Graduate School, Umamichu 4-2-2, Kitakatsuragigun-Koryocho, Nara, 635-0832, Japan.
Background: Few studies have measured the resting energy expenditure (REE) in patients with interstitial lung disease (ILD). Whether REE characteristics in patients with chronic obstructive pulmonary disease (COPD) and ILD are similar remains unknown. We aimed to investigate whether REE and its associated factors were similar in patients with COPD and ILD.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!