Table 3 aDescription of patients with childhood-onset primary Sjogren’s Syndrome associated with interstitial lung disease
Reference | Presenting Symptoms | Additional Positive Clinical features | Positive Lab Findings | Imaging | Biopsy | Treatment | Outcomes |
|---|---|---|---|---|---|---|---|
Our Patient | 14 yo White male with recurrent parotitis and wheezing, dyspnea, and cough recalcitrant to asthma therapy | IIH Exercise intolerance Joint hypermobility | ANA 1:320 (speckled pattern) SSA/SSB Low Forced Vital Capacity on PFTs | CT with significant small airway disease, bronchial wall thickening, ground glass nodules | Interstitial lymphocytic inflammation on lung wedge biopsy (NSIP) | Systemic oral steroids and MMF therapy | Significant clinical, radiographic and PFTs improvement and maintained on MMF |
Vermylen C 1985 Eur J Pediatr [18] | 5 yo Black female with recurrent respiratory infections including rhinopharyngitis and pneumonia, and failure to thrive | Parotitis, cervical lymphadenopathy, cheilitis, diffuse rhonchi and rales, mild hepatomegaly | Anemia, Hypergammaglobulinemia, elevated sedimentation rate/C-reactive protein | CXR with diffuse interstitial infiltrations, echography of parotids showing enlargement with sialectasia | Parotid biopsy with intra- and periductal lymphocytic infiltration | Systemic steroids | Clinical improvement within 2 weeks with complete resolution of parotid swelling and normalization lab findings |
Anaya JM 1995 J Rheumatol [19] | 24 yo Hispanic woman with 14 years fever, migratory arthralgia, cervical adenopathy and recurrent parotitis | Xerostomia, dental carries, left eye synechia and positive Schirmer test | Hypergammaglobulinemia, RF, ANA, SSA/SSB, and anemia DLCO showed 60% of predicted | CXR was clear | Minor salvatory biopsy with focal lymphocytic sialadenitis | Supportive and 1 year systemic steroids | Persistent parotid swelling and xerostomia without development of systemic findings |
Houghton KM 2005 J Rheumatol [7] | 14 yo dizygotic-twin Vietnamese-Canadian girl with cough, dry mouth, SOB with 4 episodes parotitis starting at age 3. Frequent dental carries and dental abscesses. | Cervical lymphadenopathy; mild clubbing, diffuse crackles and decrease aeration at base on lung exam; right keratitis; Dizygotic twin sister with primary SS | RF, ANA, SSA/SSB, anemia, leukopenia and hyperglammaglobulinemia Elevated FEV1/FVC ratio and decreased DLCO by 80% | CXR with multiple areas of consolidation; CT lungs showed multiple parenchymal densities with air bronchograms | Lung biopsy showing bronchial with predominant lymphocytic infiltrate | High-dose pulse IV steroids with course of oral systemic steroids and HCQ | Clinical and radiographic improvement in symptoms |
Tomiita M 1997 Acta Paediatr Jpn [20] | 61 cases reported with 52 girls and 6 boys with initial onset of symptoms ranging from ages 3 to 14. 70% noted to have primary SS w/ common symptoms including fever, sicca symptoms, recurrent parotitis, lymphadenopathy, and arthralgia | Interstitial pneumonia noted in 1 patient | ANA, hypergamma-globuminemia, RF, SSA/SSB were observed | None reported | All showed lymphocytic infiltration in a minor salivary gland biopsy | None reported | None reported |