SJÖGREN’S SYNDROME

DEFINITION

An immunologic disorder characterized by progressive lymphocytic destruction of exocrine glands most frequently resulting in symptomatic eye and mouth dryness; can be associated with extraglandular manifestations; predominantly affects middle-age females; may be primary or secondary when it occurs in association with other autoimmune diseases. Lymphoma can occur in 6% with glandular MALT being most common.

CLINICAL MANIFESTATIONS

  • Constitutional: fatigue
  • Sicca symptoms: KCS and xerostomia
  • Dryness of other surfaces: nose, vagina, trachea, skin
  • Extraglandular features: arthralgia/arthritis, Raynaud’s, lymphadenopathy, interstitial pneumonitis, vasculitis (usually cutaneous), nephritis, lymphoma

EVALUATION

  • History and physical examination: with special attention to oral, ocular, lymphatic examination and presence of other autoimmune disorders.
  • Presence of autoantibodies is a hallmark of disease (ANA, RF, anti-Ro/SS-A, anti-La/SS-B).
  • Other laboratory tests: ESR; CBC; renal, liver, and thyroid function tests; serum protein electrophoresis (SPEP) (hypergammaglobulinemia or monoclonal gammopathy common); UA.
  • Ocular studies: to diagnose and quantitate KCS; Schirmer’s test, Rose bengal staining.
  • Oral examination: unstimulated salivary flow, dental examination.
  • Labial salivary gland biopsy: demonstrates lymphocytic infiltration and destruction of glandular tissue.

DIAGNOSIS

International classification criteria based on clinical and laboratory features have been established (Table 383-5, HPIM-19, p. 2168).

Treatment

  • Regular follow-up with dentist and ophthalmologist.
  • Dry eyes: artificial tears, ophthalmic lubricating ointments, local stimulation with cyclic adenosine monophosphate or cyclosporine drops.
  • Xerostomia: frequent sips of water, sugarless candy.
  • Pilocarpine or cevimeline: may help sicca manifestations.
  • Hydroxychloroquine: may help arthralgias.
  • Glucocorticoids: not effective for sicca symptoms but may have role in treatment of extraglandular manifestations.

Outline

SJÖGREN’S SYNDROME is the Word of the Day!

SJÖGREN’S SYNDROME is a topic covered in the Harrison's Manual of Medicine.

To view the entire topic, please or purchase a subscription.

Harrison’s Manual of Medicine 19th edition provides 600+ internal medicine topics in a rapid-access format. Download Harrison’s App to iPhone, iPad, and Android smartphone and tablet. Explore these free sample topics:

Harrison’s Manual of Medicine - App + Web