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.