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Sjögren syndrome in Child
See also in: External and Internal Eye,Oral Mucosal Lesion
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Sjögren syndrome in Child

See also in: External and Internal Eye,Oral Mucosal Lesion
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Contributors: Sophia Delano MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Sjögren syndrome (SS) is an autoimmune disease of the exocrine glands resulting in the inflammatory infiltrate of the lacrimal and salivary glands. This glandular dysfunction leads to xerophthalmia (dry eyes) and keratoconjunctivitis sicca in the eyes and xerostomia (dry mouth). SS may also present with extra-glandular involvement including pulmonary (bronchiolitis, bronchiectasis, pneumonitis), renal (renal tubular acidosis), neurologic (neuropathy, cerebellar ataxia, encephalopathy), and cutaneous (vasculitis, purpura, xerosis) manifestations. SS may be primary or secondary to an autoimmune condition, most commonly systemic lupus erythematosus but also scleroderma and rheumatoid arthritis.

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.

Codes

ICD10CM:
M35.00 – Sicca syndrome, unspecified

SNOMEDCT:
83901003 – Sjögren syndrome

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • 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.

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 05/18/2017
Last Updated: 12/14/2018
Copyright © 2019 VisualDx®. All rights reserved.
Sjögren syndrome in Child
See also in: External and Internal Eye,Oral Mucosal Lesion
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Sjögren syndrome : Dry skin, Parotid enlargement, Arthralgia, Dry eyes, Dry mouth
Clinical image of Sjögren syndrome
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