RT Journal Article
SR Electronic
T1 Why is a paediatric respiratory specialist integral to the paediatric rheumatology clinic?
JF Breathe
JO Breathe
FD European Respiratory Society
SP 200212
DO 10.1183/20734735.0212-2020
VO 16
IS 4
A1 Ramphul, Manisha
A1 Gallagher, Kathy
A1 Warrier, Kishore
A1 Jagani, Sumit
A1 Bhatt, Jayesh Mahendra
YR 2020
UL http://breathe.ersjournals.com/content/16/4/200212.abstract
AB Systemic connective tissue diseases (CTDs) are characterised by the presence of autoantibodies and multiorgan involvement. Although CTDs are rare in children, they are associated with pulmonary complications, which have a high morbidity and mortality rate. The exact pathophysiology remains unclear. The pleuropulmonary complications in CTD are diverse in their manifestations and are often complex to diagnose and manage.The most common CTDs are discussed. These include juvenile systemic lupus erythematosus, juvenile dermatomyositis, juvenile systemic sclerosis, Sjögren's syndrome and mixed connective tissue disease. We describe the clinical features of the pleuropulmonary complications, focusing on their screening, diagnosis and monitoring. Treatment strategies are also discussed, highlighting the factors and interventions that influence the outcome of lung disease in CTD and pulmonary complications of treatment.Early detection and prompt treatment in a multidisciplinary team setting, including respiratory and rheumatology paediatricians and radiologists, is paramount in achieving the best possible outcomes for these patients.Pleuropulmonary complications of CTD, though rare in paediatrics, can be associated with high morbidity and mortality. Joint management by respiratory and rheumatology paediatricians is recommended. Treatment includes steroids and other immunomodulators. https://bit.ly/2MCvEpp