Sjögren's syndrome (Henrik Samuel Conrad Sjögren)

A chronic autoimmune disease characterised by the occurrence of xerostomia, pharyngolaryngitis sicca, rhinitis sicca, enlarged salivary glands, keratoconjunctivitis sicca, a dry mouth, dry skin, decreased sweating, dryness and crusting of the nasal passages, blocking of the eustachian tubes which can cause deafness, vaginal dryness which can cause dyspareunia, a patchy alopecia, decrease of scalp and body hair, areas of hyperpigmentation and hypopigmentation of the skin, vasculitis, purpura, cataracts, and Raynaud syndrome. Prevalent in postmenopausal women (80-90 %) or younger women after artificial menopause. Connective tissue changes include a rheumatoid type of polyarthritis.

Some consider the Mikulicz syndrome to be identical with the Sjögren’s syndrome; according to others, the former does not include rheumatoid arthritis and, therefore, is a separate entity. Polymyositis may be substituted for rheumatoid arthritis as a component of this syndrome.

Gougerot in 1925 described the progressive insufficiency and atrophy of the salivating glands and the mucous glands, and the dryness of the mucosa. Mulock Houwer (1927) and K. Wisssmann (1932) attracted attention to the joint occurrence of keratoconjunctivitis sicca and arthritis. Sjögren in 1933 gathered the complete disease picture on the basis of previous knowledge.

Gougerot's disease is entered as a separate entity under Henri Gougerot, French dermatologist, 1881-1955.

• A. F. W. E. von Graefe:
  Demonstration in der Berliner Medizinischen Gesellschaft.
  Klinische Wochenschrift, Berlin, 1868, 5: 127.
• H. Gougerot:
  Insuffisance progressive et atrophie des glandes salivaires et muqueuses de la bouche, des conjonctives (et parfois de muqueuses, nasale, laryngée, vulvaire). “Serecheresse” de la bouche, des conjonctives, etc.
  Bulletin de la Société française de dermatologie et de syphiligraphie, Paris, 1925, 32: 376.
• A. W. Mulock-Houwer:
  Corneal infection and joint affections.
  Nederlandsch Tijdschrift voor Geneeskunde, Amsterdam, 1927, 1: 2299-2301.
• K. Wissmann:
  Keratitis filiformis als Teilsymptom innersekretorischer Störungen.
  Deutsche Medizinische Wochenschrift, Stuttgart, 1932, 58: 1525.
• H. S. C. Sjögren:
  Keratoconjunctivitis sicca. Hygiea, 1930, 92: 829. Zur Kenntnis der Keratoconjunctivitis sicca (Keratitis filiformis bei Hypofunktion der Tränendrüsen).
  Acta Ophthalmologica, Copenhagen, 1933; supplement II: 1-151.
  ·
  A new conception of kerato-conjunctivitis sicca.
  Translated by J. B. Hamilton, in Australasian Medical, Sidney, 1943. Some problems concerning keratoconjunctivitis sicca and the sicca syndrome.
  Acta Ophthalmologica, Copenhagen, 1951, 29: 33-47.