Sjøgren's syndrome in children, juvenile Sjøgren's syndrome 4/5 (3)

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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

Sjøgrens syndrome is a rare one Rheumatic connective tissue disease among children (juvenile Sjøgrens syndrome) where the symptoms differ somewhat from similar disease among adults. The disease is characterized by a chronic rheumatic inflammation of the salivary and lacrimal glands, although other glands and organs can also be affected. Swollen salivary glands and lymph nodes combined with with SSA and / or SSB antibody in the blood are typical characteristics.

Occurrence

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

Symptoms

There is a greater variation in symptoms in children than among adults with Sjøgren's syndrome: Fewer children suffer from dry mouth and eyes. Several have swollen salivary glands (especially the parotid gland lying in front of the ears and against the cheeks and jaw).

General health status. The child often has signs of increased fatigue and need for sleep over time.

Salivary glands. As many as 65-75% get swollen parotid glands, even though very few children are bothered by dry mouth (Mizyuno Y, 1989).

Lymph nodes, enlarged especially on both sides of the neck, but also under the arms and in the abdominal area can be detected. Each lymph node varies in size so that they do not grow constantly.

Skin changes in the form of palpabel purpura / petekkier is common. These are often seen as many tender, small, red dots on both legs. If they are many, the skin can become so irritated that the legs swell up a bit.

Dryness. In older children who have had symptoms over time, dryness of the mucous membranes can also be felt, as is typical in in Sjøgren's syndrome in adulthood: Dryness, redness and irritation of the eyes (keratoconjunctivitis sicca). Dry mouth causes discomfort when chewing and swallowing. Increased dental problem (Caries). Hoarseness

Joint pain occurs, but arthritis (arthritis) and muscle inflammation are uncommon.

Examinations

Medical history includes fatigue, varying swellings in the salivary glands and lymph nodes, possibly other symptoms (see above).

Clinical salivary and lacrimal glands, eyes, mouth, lymph nodes, lungs and stomach area are considered. Skin and joints are also examined.

Blood tests with high ANA, SSA (Sjøgrens Syndrome A) and SSB (Sjøgrens Syndrome B) Antibodies, high immunoglobulin IgG and blood lowering (SR) (low CRP) are common.

Urine sticks done to rule out signs of kidney disease (which is uncommon).

Imaging. On ultrasound examination, juvenile Sjøgrens appears with multiple hypoechoic areas that correspond to salivary gland ducts (Tomar RPS, 2012). Swollen lymph nodes, spleen and liver can also be examined with ultrasound. In case of symptoms from the chest area and lungs, CT of the thorax is relevant.

Tissue sample (biopsy) is not always necessary, but tissue examination (histology) with a focus score of ≥ 1 strengthens the diagnosis. It is easiest to take a biopsy from small salivary glands in the lower lip.

Similar conditions / differential diagnoses

The treatment

Medications have not shown an effect against fatigue and dryness. Symptom treatment with artificial tear fluid is used if dry eyes are present. For dry mouth, good oral hygiene and sugar-free chewing gum or lozenges are among relevant measures, such as among adults with Sjøgren's syndrome. Paracetamol and NSAIDs against joint pain. Hydroxychloroquine (Plaquenil) is not used routinely, but can reduce joint pain and mild degree of arthritis. Effect on dryness or fatigue is not well documented. Increased pigmentation of the skin with dark spots and the development of eye complications after several years of use are among the side effects.

Special medications such as DMARDs and biological drugs are not commonly used, but are reserved for individual cases and after thorough, individual assessment.

Prognosis

Most people develop dryness problems such as among adults with Sjøgrens, but it often takes several years. However, there is an increased incidence 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). Overall, normal life expectancy is expected unless there is affection of internal organs (lung, heart, kidneys).

Literature

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

Children with rheumatic disease, BINDEVEVSSYKDOMMER.no


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