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.