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

^aAbbreviations: yo year old, ANA antinuclear antibody, SS Sjogren’s Syndrome, CT high-resolution computed tomography, PFTs pulmonary function tests, NSIP nonspecific interstitial pneumonia, MMF mycophenolate mofetil, CXR chest radiograph, RF rheumatoid factor, DLCO diffusing capacity of carbon monoxide, FEV1/FVC forced expiratory volume in 1 second/forced vital capacity, HCQ hydroxychloroquine