Pediatric Sjogren Syndrome Clinical Presentation

Updated: Sep 09, 2021
  • Author: Marietta Morales De Guzman, MD; Chief Editor: Lawrence K Jung, MD  more...
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Clinical manifestations of pediatric Sjögren syndrome may vary more than those seen in adult patients. [17] The constellation of symptoms seen in children (eg, lower frequency of sicca syndrome, higher rates of parotid enlargement, higher prevalence of immunologic markers) may be similar to those found in young adult patients (ie, < 35 y). [20, 21]

Sicca syndrome

Symptoms of keratoconjunctivitis include dry eyes with reduced tear production, gritty or sandy sensation under the lids, red eyes, and photosensitivity. Keratoconjunctivitis is less prominent in primary juvenile Sjögren syndrome. Lacrimal gland enlargement appears to be a feature in primary and secondary pediatric Sjögren syndrome. The management of keratoconjunctivitis includes the use of artificial tears and conservation of natural tear flow. [9, 10, 22]

Symptoms of xerostomia include decreased saliva production and difficulties with chewing, swallowing, and even speech; abnormality in taste and smell; dental caries; mucosal burning sensation; sensitivity to spicy and acidic foods and beverages; increased risk for oral candidiasis; hoarseness of voice, and dysphonia (common in adults). Recurrent parotitis appears to be the most common oroglandular manifestation in pediatric populations. [9, 10]

Musculoskeletal symptoms

Symptoms include the following:

  • Arthralgia (often noninflammatory), morning stiffness, and nonerosive arthritis

  • Myalgia and muscle weakness

Cutaneous findings

Symptoms include the following:

  • Raynaud phenomenon

  • Nonthrombocytopenic purpura, especially of lower extremities

  • Nasal, vaginal, and cutaneous dryness

Gastrointestinal symptoms

Symptoms include the following:

  • Dysphagia, nausea, and epigastric and abdominal pain

  • Achalasia (in children)

  • Achlorhydria and chronic atrophic gastritis (adult patients)

  • Primary biliary cirrhosis

Pulmonary findings

Symptoms include the following:

  • Dyspnea due to mild interstitial disease

  • Dry cough

Renal symptoms

Symptoms include the following:

  • Interstitial nephritis

  • Renal tubular acidosis [23]

Additional findings

The following symptoms may also be seen in Sjögren syndrome:

  • Fatigue

  • Depression

  • Insomnia

  • Cognitive impairment


Physical Examination

The following may be noted on physical examination:

  • Parotid gland enlargement and recurrent parotitis (most prominent feature in pediatric populations) [9, 10, 22, 24]  
  • Upper lid prominence due to lacrimal gland enlargement
  • Corneal ulceration, vascularization, and uveitis
  • Vasculitic lesions - Purpura and erythema nodosum
  • Lymphadenopathy
  • Autoimmune thyroiditis
  • Nervous system manifestations - Peripheral sensorimotor neuropathy; central nervous system (CNS) disorders such as cognitive impairment, movement disorder, transverse myelopathy, encephalopathy, aseptic meningitis, dementia, optic neuropathy, and cranial neuropathies (in both adult and pediatric patients)
  • Musculoskeletal manifestations - Intermittent synovitis, chronic nonerosive oligoarticular or polyarthritis (Jaccoud arthropathy has been observed in adults), and myalgias

Oral cavity manifestations of Sjögren syndrome may include the following: 

  • Poor or absent pooling of saliva underneath the tongue.
  • Mild erythema and thinning of the mucosa

  • Dental caries

  • Traumatic erosions and ulcers, angular cheilitis, and chapped lips

  • Frothy, ropey, and thickened saliva

  • Erythema, fissuring, coating, and depapillation of the dorsal tongue

  • Halitosis

  • Gingivitis/periodontitis

  • Superficial mucoceles