Sjögren’s Syndrome
Sjögren’s syndrome is an autoimmune disorder involving destruction of
exocrine glands which is characterized by sicca (dryness) syndrome,
recurrent parotitis, and other extra-glandular symptoms. Sjögren’s is
poorly defined in children with unknown incidence and variable
presentation, with no established diagnostic criteria [121].
Pulmonary manifestations in children and adults include restrictive lung
disease from myositis, ILD, airway disease. ILD in children appears to
be exceedingly rare, limited to four case reports in female children
[122-125] and one case report of a male child [121]. Given the
limited data on ILD in childhood onset Sjögren’s Syndrome, we urge
careful screening for pulmonary involvement through close review of
respiratory symptoms and monitoring of PFTs.