4A43.22/M35.0 Paediatric onset Sjögren syndrome



Paediatric onset Sjögren Syndrome (PSS) is an autoimmune disorder that affects the salivary and lacrimal glands. It is caused by an abnormal immune response of the body attacking its own tissues, leading to inflammation of the glands that produce saliva and tears.


Diagnosis of PSS can be difficult due to its overlap with other conditions. However, diagnosis is generally based on a combination of medical history, physical examination, and laboratory tests. Laboratory testing may include blood tests to look for antibodies associated with PSS, as well as tests to measure the level of saliva and tears.

Differential diagnosis

Differential diagnosis of PSS includes other autoimmune diseases such as systemic lupus erythematosus, Sjögren’s syndrome, and rheumatoid arthritis.


Treatment of PSS is aimed at managing and reducing the symptoms of the condition. This may include medications such as non-steroidal anti-inflammatory drugs, immunosuppressants, and topical medications to reduce inflammation. Additionally, lifestyle modifications such as avoiding certain foods and beverages that may worsen symptoms, and increasing fluid intake, may be beneficial.


The prognosis for PSS is generally good, and with proper treatment, most children with the condition have a normal lifespan. However, long-term complications such as dryness of the eyes and mouth can occur, as well as increased risk for infections, oral cavities, and other autoimmune diseases.

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DISCLAIMER: Please note that all explAInations are generated by AI and are not fact checked by a medical professional. ICD ExplAIned do not assume liability for any injuries or harm based on the use of this medical information.