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

See also in: External and Internal Eye,Oral Mucosal Lesion
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Contributors: Philip I. Song MD, Susan Burgin MD
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Synopsis

Sjögren syndrome (SS) is a systemic autoimmune disorder that affects secretory glands, in particular the salivary and lacrimal glands, as well as small blood vessels, nerves, and the musculoskeletal system. The most common features are dry mouth and dry eyes (sicca syndrome). Middle-aged women account for 80%-90% of cases. There is some familial clustering.

Dry mouth (xerostomia) causes difficulty in chewing and swallowing and increases dental caries. Dryness also predisposes to oral candidiasis, which may cause a burning sensation and altered taste. One-third to one-half of patients have slightly tender enlargement of the bilateral salivary glands.

Dry eyes (xerophthalmia) presents as a gritty or foreign body sensation in the eyes and a predisposition to corneal ulceration.

Skin manifestations, which occur in about half of patients with SS, include xerosis (most common), Raynaud phenomenon, petechiae, palpable and nonpalpable purpura, urticarial vasculitis, annular erythema (lesions on the head and neck that can resemble subacute cutaneous lupus or Sweet syndrome), pernio-like lesions, and livedo reticularis. The presence of cutaneous vasculitis is associated with an increased risk of systemic involvement.

SS may present alone (primary SS), or it may be associated with other autoimmune diseases (secondary SS), such as lupus erythematosus or rheumatoid arthritis. Systemic manifestations of SS can arise in almost every organ system, such as muscle weakness, arthralgias, interstitial lung disease, autoimmune thyroiditis, and cytopenias.

There are American-European Consensus Group criteria (AECG) and the American College of Rheumatology criteria for SS, which are mostly used for research purposes rather than clinical diagnosis. For reference, the AECG criteria include:
  • Ocular symptoms – Daily, persistent, troublesome dry eyes for more than 3 months, recurrent sensation of sand or gravel in the eyes, and/or use of tear substitutes more than 3 times a day.
  • Oral symptoms – Daily feeling of dry mouth for more than 3 months, recurrently or persistently swollen salivary glands (as an adult), and/or frequent intake of liquids to aid in swallowing of dry foods.
  • Ocular signs – Positive result for Schirmer's test or Rose Bengal score (or other ocular dye score).
  • Histopathology – A positive salivary gland biopsy confirms the diagnosis of SS; see Best Tests section for details.
  • Salivary gland involvement by measurement of decreased salivary flow.
  • Autoimmune antibodies – Presence in the serum of antibodies to Ro/SSA, La/SSB, or both.

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

  • Three common causes of xerostomia are use of anticholinergic medications, insufficient oral hydration, and psychogenic factors such as chronic anxiety and/or depression. Head and neck radiation may also cause xerostomia.
  • Dry eyes and dry mouth may be a normal finding in the elderly due to age-related atrophy of secretory glands. These symptoms may also be seen in those with chronic graft-versus-host disease.
  • Dry eyes may be seen in ocular rosacea, sarcoidosis, amyloidosis (AA, AL), cicatricial pemphigoid, and hypovitaminosis A.
  • Mumps – There is acute swelling, often associated with systemic signs of illness. This is usually seen in young patients and is self-remitting.
  • Lymphoma – Patients with SS have an elevated risk of suffering from lymphomas arising within the salivary glands or lymph nodes. Tests to detect B-cell monoclonality should be used to detect lymphomas arising in a lymphocytic proliferation.
  • Sialadenosis – Non-neoplastic, noninflammatory enlargement of salivary glands, particularly the parotid glands, may occur secondary to diabetes mellitus, general malnutrition, alcohol use disorder, and bulimia.
  • Salivary gland tumors (malignant, benign) – Sicca is not associated with these.

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: 02/15/2017
Last Updated: 05/04/2017
Copyright © 2019 VisualDx®. All rights reserved.
Sjögren syndrome in Adult
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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