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.