Pulmonary manifestations of rheumatologic diseases.
OBJECTIVE
The present review intends to provide an overview of the diversity and complexity of pulmonary manifestations of rheumatologic diseases and gaps in knowledge to effectively manage them.


RESULTS
Diffuse lung disease in children with rheumatologic diseases represents a heterogeneous group of autoimmune disorders. Despite their significant morbidity and mortality, we have limited understanding about their pathogenesis. Here, we provide an overview of the pathophysiology and current management approach of these disorders, highlighting tools which assist with diagnosis, risk stratification and therapy. In this context, we address the need to develop a standardized approach to diagnose at-risk patients with rheumatologic disease and to predict their progression and the need to develop robust studies which evaluate the factors and interventions that influence pulmonary disease outcome.


CONCLUSIONS
Diffuse lung disease in children with rheumatologic diseases represents a heterogeneous group of severe autoimmune disorders. By adopting a collaborative research approach among multicenters to help diagnose, risk stratify, and understand disease progression, effective management decisions can be optimized to improve clinical outcome.
aDivision of Pediatric Rheumatology, Department of Pediatrics, USC Keck School of Medicine, Children's Hospital of Los Angeles, Los Angeles, California, USA bDivision of Pediatric Pulmonology, Department of Pediatrics, USC Keck School of Medicine, Children's Hospital of Los Angeles, Los Angeles, California, USA cDepartment of Pediatrics, University of Tübingen, Tübingen, Germany dRoche Pharma Research & Early Development (pRED), Immunology, Inflammation and Infectious Diseases (I3) Discovery and Translational Area, Roche Innovation Center, Basel, Switzerland.
Michal
M
Cidon
Manvi
M
Bansal
Dominik
D
Hartl
D001327:Autoimmune Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy; D002648:Child; D006801:Humans; D008171:Lung Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy; D015995:Prevalence; D012216:Rheumatic Diseases / Q000276:immunology / Q000503:physiopathology*; D018570:Risk Assessment