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Primary Sjögren disease

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Disease definition

A rare systemic autoimmune disease characterized by exocrine gland dysfunction, resulting predominately in keratoconjunctivitis sicca and xerostomia, but also affecting exocrine glands of the skin, as well as respiratory, urogenital, and digestive tract. Extraglandular manifestations include arthritis, interstitial lung disease, renal disease, and peripheral neuropathy. The disease is accompanied by a substantially increased risk to develop B-cell non-Hodgkin lymphoma, especially MALT (mucosa-associated lymphoid tissue) lymphoma.

ORPHA:289390

Classification level: Disorder

Synonym(s):
  • Primary Sjögren syndrome
  • Primary SjD

Source: PubMed ID 28613703 38331820 25387476

Prevalence: 1-5 / 10 000

Inheritance: Not applicable

Age of onset: Adult

ICD-10: M35.0

ICD-11: 4A43.20

OMIM: 270150

UMLS: C0151449

GARD: 10252

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands Ελληνικά
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

General public
Article for general public
Deutsch (2021.pdf) - Kindernetzwerk e.V.
Guidelines
Emergency guidelines
Français (2022.pdf) - Orphanet Urgences
Español (2024.pdf) - Orphanet Urgences
Clinical practice guidelines
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.