Sjögren syndrome in Child
SS most commonly occurs in middle-aged adult women, and there is a similar predominance in pediatric SS patients, who typically present in late childhood / early preteen years with recurrent parotitis. Compared to adults, children with SS are less likely to experience keratoconjunctivitis sicca and xerostomia.
The American-European Consensus Group's (AECG) criteria for primary SS in adults have not been validated in children. AECG criteria include eye symptoms, oral symptoms, findings of keratoconjunctivitis sicca on Schirmer or Rose-Bengal testing, lymphocytic infiltrate of minor salivary glands on biopsy, positive antibodies to SSA or SSB, and salivary gland dysfunction. The addition of recurrent parotitis increases the criteria's sensitivity in children with SS. Given the decreased incidence of xerostomia in children with SS, minor salivary gland biopsy may not have a high yield.
M35.00 – Sicca syndrome, unspecified
83901003 – Sjögren syndrome
- Parotitis – Viral (mumps, Epstein-Barr virus, adenovirus, etc), bacterial (Staphylococcus aureus, Streptococcus, and Haemophilus influenzae), physical (sialolithiasis, pneumoparotid), malnutrition-related (anorexia, diabetes, nutritional deficiencies, human immunodeficiency virus), lymphoma, autoimmune-related (sarcoidosis), and juvenile recurrent parotitis, which more often appears in boys and at a younger age compared to pediatric SS.
- Xerophthalmia / sicca – Vitamin A deficiency, allergies, medications.
- Xerostomia – Medication, radiation / chemotherapy.
- Xerosis / pruritus – Atopic dermatitis, contact dermatitis, irritant dermatitis, ichthyosis. These conditions will usually be of longer duration, have inciting triggers, and have characteristic morphologies.
- Purpura – Small-vessel vasculitides such as leukocytoclastic vasculitis induced by medication or viral exposure or immunoglobulin A vasculitis (formerly Henoch-Schönlein purpura; purpura on buttocks / legs, abdominal pain, and arthralgias), thrombocytopenia, trauma.
- Systemic lupus erythematosus (SLE) – Positive ANA. Positive anti-dsDNA and low complement levels favor SS secondary to SLE.
- Rheumatoid arthritis – Arthritis, positive rheumatoid factor.
- Scleroderma – Morphea, pulmonary involvement, CREST variant (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia).
- Cryoglobulinemia – Idiopathic cryoglobulinemia, hepatitis C.
Last Updated: 12/14/2018