Pediatric Sjogren Syndrome Clinical Presentation

  • Author: Eyal Muscal, MD; Chief Editor: Lawrence K Jung, MD   more...
 
Updated: Aug 25, 2011
 

History

Clinical manifestations of pediatric Sjögren syndrome may vary more than those seen in adult patients.[14] 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).[17]

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.[8, 9, 18]

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.[8, 9]

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[19]

Additional findings

The following symptoms may also be seen in Sjögren syndrome:

  • Fatigue
  • Depression
  • Insomnia
  • Cognitive impairment
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Physical Examination

The following may be noted on physical examination:

  • Parotid gland enlargement and recurrent parotitis (most prominent feature in pediatric populations)[8, 9, 18]
  • 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 is observed in adults), and myalgias

Oral cavity manifestations of Sjögren syndrome may include the following:

  • 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
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Contributor Information and Disclosures
Author

Eyal Muscal, MD  Assistant Professor, Section of Pediatric Rheumatology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital

Eyal Muscal, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Rheumatology, and Clinical Immunology Society

Disclosure: Nothing to disclose.

Coauthor(s)

Marietta Morales DeGuzman, MD  Assistant Professor, Department of Pediatrics, Baylor College of Medicine; Consulting Staff, Section of Pediatric Rheumatology, Department of Pediatrics, Texas Children's Hospital, Ben Taub General Hospital

Marietta Morales DeGuzman, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Rheumatology, and Texas Pediatric Society

Disclosure: Nothing to disclose.

Lawrence K Jung, MD  Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Catherine M Flaitz, DDS, MS  Professor of Oral and Maxillofacial Pathology and Pediatric Dentistry, Department of Diagnostic Sciences, University of Texas Health Sciences Center at Houston, Dental Branch

Catherine M Flaitz, DDS, MS is a member of the following medical societies: American Academy of Oral and Maxillofacial Pathology, American Academy of Oral Medicine, American Academy of Pediatric Dentistry, American Dental Association, International Association for Dental Research, and International Association of Oral Pathologists

Disclosure: Trimira, LLC Clinical contract for study Co-investigator on clinical grant; Trimira, LLC Honoraria Speaking and teaching; GC America Clinical contract for study Co-investigator on clinical grant

Specialty Editor Board

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David D Sherry, MD  Director, Clinical Rheumatology, Attending Physician, Pain Management, The Children's Hospital of Philadelphia; Professor of Pediatrics, University of Pennsylvania School of Medicine

David D Sherry, MD is a member of the following medical societies: American College of Rheumatology and American Pain Society

Disclosure: Nothing to disclose.

Chief Editor

Lawrence K Jung, MD  Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, and New York Academy of Sciences

Disclosure: Nothing to disclose.

References

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Lower facial appearance of a 14-year-old adolescent girl with Sjogren syndrome. She exhibits both parotid and submandibular gland enlargement and chapped lips.
Intraoral view of a 14-year-old adolescent girl with Sjogren syndrome. Hyposalivation results in erythema of the mucosa, gingivitis, decalcification or white spot lesions of the teeth at the cervical margin, and dental caries with extensive restorations of the posterior teeth.
Erythema of the labial mucosa with enlargement of the minor salivary glands and superficial mucoceles.
The dorsal surface of the tongue demonstrates generalized atrophy of the filiform papillae, mild fissuring, and median rhomboid glossitis.
A 14-year-old adolescent girl with Sjogren syndrome with painful unilateral swelling of the knee and hyperpigmentation of the overlying skin.
The dorsal tongue demonstrates hyperplastic candidiasis with focal erosions and a brown hairy tongue. Ulcerated fissures are observed on the corners of the mouth that represent angular cheilitis.
Biopsy of the minor salivary glands of the lower lip may be useful in the diagnosis of Sjögren syndrome. A 1.5- to 2-cm incision of normal-appearing mucosa allows for the harvesting of 5 or more salivary gland lobules.
Low-power photomicrograph of a minor salivary gland lobule showing multiple lymphocytic foci that are replacing the acinar structures (hematoxylin-eosin, 40 X).
Intermediate-power photomicrograph demonstrating a chronic inflammatory aggregate of more than 50 lymphocytes and plasma cells with a periductal pattern. The inflammatory focus is adjacent to normal appearing acini (hematoxylin-eosin, 200 X).
High-power photomicrograph of the chronic inflammatory aggregate consists of lymphocytes and plasma cells around a ductal structure (hematoxylin-eosin, 400 X).
 
 
 
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