Juvenile Sjøgren's syndrome in 10 year old child with swollen salivary glands (ultrasound images) and typical tissue sample. Iro H. GMS Curr Top Otorhinolaryngol Head Neck Surg (2014). CC BY NC ND 3.0

Definition

Primarily Juvenile Sjøgrens Syndrome

Sjøgren's syndrome is rare among children (Juvenile Sjøgren's syndrome). The disease is characterized by a rheumatic inflammation of saliva and lacrimal glands. Other glands and organs can also be affected. Primarily Sjøgren's syndrome is a separate one Rheumatic connective tissue diseasewhile secondary Sjøgren's syndrome occurs as a result of others Autoimmune diseases like systemic lupus (SLE), Juvenile arthritis, rheumatoid arthritis (RA), Systemic sclerosis or Primary biliary cirrhosis (Liver disease). The further information applies in particular primarily Sjögren's syndrome.

Occurrence

Most girls (girl: boy 7: 1) get Sjøgren's childhood syndrome. The average age at start in children is 10 years, but the disease can start at any age. In adults, it is most common among women from the 40 years.

Symptoms

Some symptoms are as in disease start in adulthood:

• Dryness, redness and irritation of the eyes (keratoconjunctivitis sicca)
• Uncomfortable to look at light (photosensitivity)
• Enlarged salivary glands (applies to parotid and submandibularis glands)
• Dry mouth causes discomfort during chewing and swallowing
• Increased dental problems (carious)
• Hoarseness
• Arthralgia and / or muscle pain
• Raynaud's phenomenon
• Petechial rash (Red dots, mostly on calves)
• Fatigue and increased need for sleep

Blood tests with high ANA, SSA (Sjøgrens Syndrome A) and SSB (Sjøgrens Syndrome B) Antibodies, High immunoglobulin IgG and sedimentation rate (ESR) (low CRP).

Difference from debut in adulthood

One sees greater variation in symptoms in children than among adults with Sjøgren's syndrome

• Fewer symptoms of dryness in the mouth and eyes
• Several have swollen salivary glands (especially parotid gland)

Differential diagnosis

• Drugs that causes dryness
• Relapsing juvenile parotitis
• Other reasons for salivary gland swelling
• Candida infection in the mouth
• Gingivitis or periodontitis (gingivitis)
• Bacterial (retrograde) sialoadenitis (salivary gland inflammation)
• anorexia, bulimia (causing swollen glands)
• Mumps
• Sarcoidosis
• SLE
• Hepatitis C
• Mononucleosis (Ebstein-Barr virus infection)
• HIV
• Lymphoma
• Fabry's disease

Medical examinations

General practitioner, rheumatologist and / or pediatrician, ophthalmologist, dentist

The treatment

• As with the onset of disease in adulthood
• Eye drops with "artificial tear fluid" (for example, Viscotears eye gel)
• Good mouth hygiene
• Special medications in some cases (after thorough individual assessment)

Prognosis

Survival is hardly affected if there is no manifestations of internal organs (lung, heart, kidneys). However, there is an increased occurrence of Non-Hodgkin's lymphoma (risk factors are persistently low lymphocytes (one type of leukocyte) in blood and low complement factors C3 and C4, (reference: Jonsson MW, 2012).

Literature: The Souza, TR 2012

More generally about Sjögren's syndrome (among adults) here

Children with rheumatic disease, BINDEVEVSSYKDOMMER.no