Conclusions
Recognition, diagnosis, and treatment of chILD associated with connective tissue disease and immune-mediated disorders requires thoughtful care from a multi-disciplinary team of providers, with the pulmonologist playing a key role. These patients have highly complex systemic disorders, and ILD can have many faces – sometimes presenting subtly and progressing insidiously, while other times presenting with rapid and life-threatening consequences. The diagnostic odyssey and management challenges associated with immune associated chILD can be a significant medical, psychologic, and socio-economic burden to patients and their families. Important next steps in the care of these patients include improving therapy adherence, conducting randomized trials and developing pediatric consensus guidelines to generate more evidence-based and standardized treatment protocols, and developing novel therapeutics with reduced risk for toxicity and infectious risks. Despite significant challenges in the care of these complex patients, advances in molecular and genetic testing have aided in achieving earlier and less invasive diagnoses, and emerging therapies represent promising future innovations.
Acknowledgements : The authors would like to thank the Boston Children’s Hospital Children’s Interstitial Lung Disease Program and multi-disciplinary Multiple Immune Dysregulation and Autoimmune Syndrome (MIDAS) program for their commitment to caring for and advancing the care of patients with interstitial lung disease and other systemic complications of IEIs and autoimmunity. HW is funded by a NIAID grant T32-AI-007512-36 with related work supported by the Rheumatology Research Foundation Abstract Awards.