Medical Management
At our institution, we have found standardization of diagnostic testing and medical management provided more consistent care for patients with SJIA-ILD. These complex patients should be routinely evaluated by a multidisciplinary group including rheumatology, pulmonology, nutrition, social work, respiratory therapy, and physical therapy for diagnosis, surveillance, and management of disease and comorbidities. Our recommended diagnostic testing and medical management are summarized in Table 5.
In conclusion, sJIA-LD is a rare presentation of an uncommon disease process. Our cohort is similar to those previously described. These children have high morbidity from both disease and iatrogenic side-effects requiring multi-disciplinary care. It is important to note though, at the time of this article, in our cohort none are deceased and with aggressive interventions, can have improvement in their clinical symptoms. Further research is necessary to describe the pathophysiology of sJIA-LD, appropriate treatment, and effective management of this complex disease process.