Sjogren Syndrome Differential Diagnoses

Updated: Nov 18, 2017
  • Author: Anne V Miller, MD; Chief Editor: Herbert S Diamond, MD  more...
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DDx

Diagnostic Considerations

Sjögren syndrome is associated with a wide variety of other disorders, which may contribute to underdiagnosis or misdiagnosis. A careful review of systems is needed to detect problems such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), scleroderma, polymyositis, chronic active hepatitis, idiopathic pulmonary fibrosis, primary biliary cirrhosis, [57] and autoimmune thyroid disease. [58]

Xerophthalmia, xerostomia, and enlargement of the parotid glands can result from adverse effects of drugs and other diseases. HIV infection can result in diffuse infiltrative lymphocytosis syndrome (DILS), which is characterized by parotid enlargement; involvement of the renal, lung, and gastrointestinal systems; and a low frequency of autoantibody presence. Chronic graft versus host disease may mimic symptoms associated with idiopathic Sjögren syndrome. [59] SLE might be considered, especially at onset of the disease.

Histologic findings of the following disorders can be consistent with Sjögren syndrome:

  • Sarcoidosis
  • Graft versus host disease
  • HIV infection
  • HTLV-1 infection
  • Hepatitis C virus (HCV) infection
  • Keratoconjunctivitis sicca

Sicca

Differential diagnoses to consider in patients with sicca include the following:

  • Medications (eg, antidepressants, anticholinergics, beta-blockers, diuretics, antihistamines, some antiarrhythmic and antiepileptic drugs)
  • Anxiety and depression
  • Viral infections (eg, mumps)
  • Complications from contact lenses
  • Dehydration
  • Hypervitaminosis A
  • Neurotropic keratitis
  • Mucous membrane pemphigoid
  • Environmental irritants
  • Mouth breathing
  • Chronic blepharitis
  • Chronic conjunctivitis
  • Rosacea
  • Therapeutic radiation or surgery to the head and neck
  • Age
  • Alzheimer disease
  • Parkinson disease
  • Bell palsy
  • Amyloidosis
  • Sarcoidosis
  • Lymphoma

Parotid enlargement

Differential diagnoses to consider in patients with parotid enlargement include the following:

  • Viral infection (eg, mumps, Epstein-Barr virus, cytomegalovirus, coxsackievirus A, influenza)
  • DILS associated with HIV disease
  • Granulomatous diseases (sarcoidosis, tuberculosis, leprosy)
  • Hyperlipoproteinemia
  • Hepatic cirrhosis
  • Hepatitis C
  • Bulimia
  • Recurrent parotiditis of childhood
  • Chronic pancreatitis
  • Acromegaly
  • Amyloidosis
  • Gonadal hypofunction
  • Diabetes mellitus
  • Salivary gland tumor (primarily unilateral)
  • Bacterial infection (primarily unilateral)
  • Chronic sialadenitis (primarily unilateral)
  • Lymphoma

Associated disorders

Importantly, evaluate the patient for disorders associated with Sjögren syndrome, including the following:

  • AIDS
  • RA
  • SLE
  • Scleroderma
  • Polymyositis
  • Primary biliary cirrhosis
  • Thyroiditis
  • Chronic active hepatitis
  • Mixed cryoglobulinemia
  • Celiac sprue

Differential Diagnoses