Patient Presentation
A 14-year-old female came to clinic with fever to 101.2°F and sore throat for 36 hours. She also complained of some nausea but denied emesis, rash, cough, rhinorrhea or diarrhea. She had been around people with strep throat at school. Her past medical history was positive for Sjögren Syndrome diagnosed at age 9 after she had recurrent parotitis and multiple caries. Her disease had progressed to include some arthritis and she was treated with pulse steroids and an anti-inflammatory biological agent which did not work for her. She had been off her steroids for more than 1 year. The family history was positive for systemic lupus erythematosus. The pertinent physical exam showed a tired looking female. Her eyes were normal. Tympanic membranes were also normal. Her mouth had erythema of the posterior pharynx, and pus on her tonsils and palatal petechiae. She had some shotty anterior cervical nodes. Her lungs, abdomen and heart examinations were normal. Her skin did not show any rashes.

The laboratory evaluation of a rapid streptococcal test was positive. The diagnosis of of a patient with strep throat was made. She was prescribed penicillin. She was very knowledgeable about her disease and told the physician that “My doctor told me that as long as I am not taking the steroids then my immune system is like everyone elses.” Her mother agreed and said how they were all so glad to have her not taking the steroid medications, but they knew that her symptoms could get worse again. The girl had a followup appointment with her immunologist the following week, and so her annual influenza vaccine was deferred until that appointment.

Discussion
Sjögren Syndrome (SS) is named for Swedish ophthalmologist Henrik Sjögren who published a case series in 1933 describing patients with dry eyes and arthritis. SS is a “chronic autoimmune inflammatory exocrinopathy” that is characterized by lymphocytic infiltration of the lacrimal and salivary glands and has various degrees of systematic involvement. Keratoconjunctivitis sicca and xerostomia are the main clinical symptoms.

Sicca is a Latin word meaning dry. Dryness of the eyes and mouth without evidence of autoimmune disease is called Sicca syndrome or Sicca complex. SS can be primary or secondary. Primary includes keratoconjunctivitis sicca and xerostomia which is the same as Sicca syndrome. Secondary includes keratoconjunctivitis sicca and xerostomia and evidence of connective tissue disease, usually rheumatoid arthritis. Many people with Sicca syndrome may have SS, but not all. SS is an autoimmune disease and therefore the cause of the Sicca syndrome symptoms needs to be established that it is caused by an autoimmune problem and not because of another reason. Symptoms may take time to develop and therefore patients can be diagnosed with Sicca syndrome before SS is diagnosed.

SS in adults is a clinical diagnosis with various professional organizations having diagnostic criteria for making its diagnosis. Patients usually have dry eyes, dry mouth, and abnormal salivary gland biopsies and abnormal laboratory testing. Patients are usually female and between 40-50 years old. SS can be found with other autoimmune disorders such as rheumatoid arthritis or systemic lupus erythematosus.

SS is thought to be rare or possibly underdiagnosed in children as it’s classical manifestations seen in adults are less likely to occur in children. Although dry eyes or mouth are less common, detailed history may find additional symptoms. Abnormal laboratory testing especially Anti-SS-A or Anti-SS-B or antinuclear antibodies are often positive. Recurrent parotitis is one of the first symptoms, and is the most frequent and most consistent symptom at diagnosis and followup. Causes of parotitis can be reviewed here.

Pediatric patients with SS are usually female as well (5:1 being reported) and patients can be as young as 4 years old. Average is around 10 years in some studies.

Learning Point
Pediatric patients with SS may have the following:

• Oral symptoms and signs
  • Recurrent parotitis (64%)
  • Xerostomia – (27%) may be present with detailed history
  • Caries, multiple
  • Candidiasis, erythematous
• Ocular symptoms and signs
  • Dry eyes – (33%)
  • Negative Schirmer or Rose Bengal testing is common
• Salivary gland involvement
  • Histopathology changes of the parotid gland on biopsy are relatively common
  • Histopathology changes of the minor salivary glands on biopsy are less common
• Abnormal laboratory testing
  • Anti-SS-A (Ro) positivity (67%)
  • Anti-SS-B (La) positivity (62%)
  • Antinuclear antibodies positivity (82%)
  • Rheumatoid factor positivity (53%)

Other systemic extraglandular problems include:

• Dermatological
  • Vasculitis – can look like Henoch-Sch&oum;nlein purpura, erythema nodosum or granuloma annulare
• Gastrointestinal
  • Hepatitis, autoimmune
  • Pancreatitis
• Hematological
  • Anemia
  • Hypergammaglobulinemia
  • Lymphoma
• Nervous system
  • Central nervous system
    • Cerebellar ataxia
    • Meningoencephalitis
    • Optic neuritis
    • Transverse myelitis
  • Headache
  • Peripheral neuropathy
• Musculoskeletal
  • Arthralgia
  • Hypokalemic paralysis
  • Rheumatoid arthritis
  • Polyarthritis
  • Raynaud phenomenon
• Pulmonary
  • Alveolitis
  • Interstitial lymphocytosis
• Renal
  • Glomerulonephritis
  • Renal tubular acidosis

Note: percentages are from 2012 systematic review

Questions for Further Discussion
1. What treatment is available for Sjögren Syndrome?
2. What specialists should be involved in the care of patients with Sjögren Syndrome?
3. How do you perform a Schirmer test?

Related Cases

Disease: Streptococcal Infections | Sjogren’s Syndrome

Symptom/Presentation: Sore Throat

Specialty: Dentistry / Orthodontia | Immunology | Ophthalmology | Medical History

Age: Teenager

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Sj&ouml:gren’s Syndrome

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Singer NG, Tomanova-Soltys I, Lowe R. Sjögren’s syndrome in childhood. Curr Rheumatol Rep. 2008 Apr;10(2):147-55.

de Souza TR, Silva IH, Carvalho AT, Gomes VB, Duarte AP, Leão JC, Gueiros LA. Juvenile Sjögren syndrome: distinctive age, unique findings. Pediatr Dent. 2012 Sep-Oct;34(5):427-30.

Maciel G, Crowson CS, Matteson EL, Cornec D. Incidence and Mortality of Physician-Diagnosed Primary Sj&ouml:gren Syndrome: Time Trends Over a 40-Year Period in a Population-Based US Cohort. Mayo Clin Proc. 2017 May;92(5):734-743.

Olschowka N. The History of Henrich Sjögren. Association Du Syndrome De Sjögren. Available from the Internet at: http://sjogrens.ca/en/the-history-of-henrick-sjogren (rev. 2017, cited 10/10/17).

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa

Date
December 11, 2017