Sjogren's syndrome

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Classification according to ICD-10
M35.0 Sicca syndrome [Sjogren's syndrome]
ICD-10 online (WHO version 2019)
Parotid swelling in Sjogren's syndrome

The Sjogren's syndrome [ ɧøːɡreːn ] (also Dacryo-sialo Adenopathia atrophicans called) is a chronic autoimmune disease from the rheumatic type and the group of collagen , in certain immune cells especially the salivary and lacrimal glands attack and other inflammatory changes to internal organs and can lead to the central nervous system. Sjogren's syndrome manifests itself in morphological changes in the lacrimal and salivary glands. The main symptoms of dry eyes and dry mouth (xerostomia) are also known as sicca syndrome . A distinction is made between primary Sjogren's syndrome (pSS) and secondary Sjogren's syndrome (sSS), which can occur in conjunction with other autoimmune diseases such as lupus erythematosus , rheumatoid arthritis or systemic sclerosis . The initial appearance of sicca symptoms suggests pSS.

The disease is named after the Swedish ophthalmologist Henrik Sjögren , who first described it in his doctoral thesis in 1933 .

Epidemiology

The classification criteria are different. It should be noted that the disease affects women much more often than men (up to 20: 1) and usually occurs after the menopause . The prevalence of the more common sSS is around 0.4%.

association

Sjögren's syndrome can occur as an independent disease or more often as an accompanying symptom of other rheumatic diseases: rheumatoid arthritis , lupus erythematosus , primary sclerosing cholangitis , ankylosing spondylitis , Raynaud's syndrome .

It is not uncommon for Sjogren's syndrome to be associated with autoimmune thyroid disease , and in 6% of cases also with the development of malignant lymphoma . A so-called pseudolymphoma , which is histologically difficult to differentiate from a real lymphoma, can occur even more frequently .

Symptoms

General symptoms

Symptoms include severe tiredness, poor concentration, exhaustion, depression, joint inflammation ( arthritis ), sometimes dry skin, nose and vaginal dryness , inflamed salivary glands and Raynaud's syndrome . Other organs such as the kidneys , blood vessels , lungs , liver , pancreas or the brain can rarely be affected. By exudates , it may be about to the formation of pleural effusions come.

eyes

Mouth and throat

Dry mouth ( xerostomia ) was found in 94 to 100% of Sjogren's patients. 80% report problems in the throat area. Late manifestations are hoarseness, knot formation, and voice disorders.

The most common symptoms of the oral mucosa in primary and secondary Sjogren's syndrome are:

  • Inflammation of the corner of the mouth ( angular cheilitis )
  • Inflammation of the lips (cheilitis)
  • Decreased lip moisture
  • Unspecific ulcerations , aphthae and aphtha-like conditions

Fatigue, depression

In people with primary Sjogren's syndrome, the prevalence of abnormal fatigue (exhaustion, tiredness) is 70%. This leads to a massive deterioration in quality of life. A study of patients with primary Sjogren's syndrome in the US found that 37% of these patients had depression .

Myalgia, fibromyalgia, and joint pain

Joint pain ( arthralgia ), morning stiffness and joint inflammation ( arthritis ) are common in Sjogren's patients. 40 to 75% of Sjogren's patients suffer from joint pain with no detectable joint inflammation. Myalgia was found in 33% of patients with primary Sjogren's syndrome and fibromyalgia in 47 to 55% of patients .

Skin involvement

Drying out of skin and mucous membrane surfaces as well as brittle, brittle hair, fingernails and toenails are typical symptoms of Sjogren's syndrome. Dry skin is often accompanied by itching. Eczema can develop on the eyelids, possibly with the perception of foreign bodies. Various forms of inflammation of the subcutaneous fat ( panniculitis ) can occur, such as: B. Erythema nodosum and amyloidosis . The risk of developing lymphoma is 6.5 to 40 times higher. Sjogren's syndrome can be associated with subacute cutaneous lupus erythematosus , a rare form of lupus erythematosus . Skin changes associated with rheumatoid arthritis or systemic sclerosis (hardening of the connective tissue) are also possible in connection with Sjogren's syndrome . In addition, Sjogren's syndrome can be accompanied by vasculitis (inflammation of blood vessels caused by autoimmunological processes).

Nose, sinuses and ears

Most of the studies examining the prevalence of nose involvement in Sjogren's syndrome are purely observational studies with mixed results. The most common symptoms observed are dry nose, crust formation, nasal congestion, increased sense of smell and nosebleeds. It is known to have moderate hearing loss at high frequencies.

Involvement of the nervous system

The syndrome can cause severe polyneuropathies. Patients with dry mouth, dry eyes and circulatory disorders are usually treated by rheumatologists who are familiar with the syndrome. Patients with severe paralysis are admitted to neurological clinics, where Sjogren's syndrome is rather unknown. Since mostly older people are affected, many doctors see the disease only as a symptom of old age.

Non-Hodgkin lymphoma

One possible complication of Sjogren's syndrome is non-Hodgkin lymphoma , a malignant disease of the lymphatic system that often occurs within the salivary glands.

Diagnosis

Fine tissue section of a biopsy

Like other collagenoses , Sjögren's syndrome can lead to a decline in various cell lines in the blood, as well as high levels of antinuclear antibody (ANA) or rheumatoid factor . The inflammation increases the sedimentation rate (ESR) and C-reactive protein (CRP). However, ss-A and ss-B autoantibodies are specific in up to 70% of patients and sometimes antibodies against epithelial cells in the ducts of the salivary glands. A biopsy can be taken from the inside of the lip to confirm the diagnosis . An inflammation of the gland with lymphocyte infiltration can be seen in the biopsy .

Regular ophthalmological checks are advisable. Mention should be made of the Schirmer test , in which the production of tear fluid in the eye is checked with blotting paper. In Sjogren's syndrome, the blotting paper stays dry.

Therapy (as of 2017)

A causal treatment of Sjogren's syndrome is not known, and standard therapy has not been established. Therapeutic approaches are used that are also used for other autoimmune diseases. The symptoms can be alleviated: Careful oral hygiene, sufficient fluid intake, sun protection of the eyes, eye drops ("artificial tear fluid"), if necessary dental implants. A possible mycosis of the oral mucosa is treated with antifungal agents. The following drugs are used depending on the manifestation: corticosteroids, NSAID, hydroxychloroquine, pirfenidone, nintedanib, antidepressants, rituximab, plasmapheresis.

See also

literature

  • U. Pleyer: Inflammatory Eye Diseases . Springer-Verlag, 2014, ISBN 978-3-642-38419-6 , pp. 119 ff.
  • Steven Carsons, Elaine K. Harris (Eds.): The New Sjogren's Syndrome Handbook. Oxford University Press, New York / Oxford 1998, ISBN 0-19-511724-7 .

Web links

Commons : Sjogren's Syndrome  - collection of pictures, videos and audio files

Individual evidence

  1. ↑ Ludwig Heilmeyer , Wolfgang Müller: special forms of chronic polyarthritis. In: Ludwig Heilmeyer (ed.): Textbook of internal medicine. Springer-Verlag, Berlin / Göttingen / Heidelberg 1955; 2nd edition, ibid. 1961, pp. 331–333, here: p. 332.
  2. ↑ a b c d Clinic m. S. Rheumatology and Clinical Immunology: Sjögren's Syndrome, Berlin 2015.
  3. ↑ M. Ramos-Casals: Treatment of primary Sjogren's syndrome: a systematic review. In: JAMA. 304 (4), Jul 28, 2010, pp. 452-460. doi: 10.1001 / jama.2010.1014 .
  4. ^ G. Westhoff: Epidemiology of primary Sjogren's syndrome. In: Z Rheumatol. 69 (1), Feb 2010, pp. 41-49. doi: 10.1007 / s00393-009-0518-3 .
  5. ↑ Berthold Jany, Tobias Welte: Pleural effusion in adults - causes, diagnosis and therapy. In: Deutsches Ärzteblatt. Volume 116, No. 21, (May) 2019, pp. 377-385, here: p. 379.
  6. ↑ A. Jaksche: The dry eye. University Eye Clinic Bonn.
  7. ↑ Guideline No. 11: "Dry eye" (Sicca syndrome) of the professional association of ophthalmologists in Germany (BVA)
  8. ↑ a b c d e f g h i j k Manuel Ramos-Casals, John H. Stone, Haralampos M. Moutsopoulos (eds.): Sjögren's Syndrome. Diagnosis and Therapeutics . Springer, London 2012, ISBN 978-0-85729-946-8 , doi : 10.1007 / 978-0-85729-947-5 .
  9. ↑ a b c d K. Błochowiak, A. Olewicz-Gawlik, A. Polańska, M. Nowak-Gabryel, J. Kocięcki, H. Witmanowski, J. Sokalski: Oral mucosal manifestations in primary and secondary Sjögren syndrome and dry mouth syndrome . In: Postepy dermatologii i alergologii . tape 33 , no. 1 , February 2016, p. 23-27 , doi : 10.5114 / pdia.2016.57764 , PMID 26985175 , PMC 4793060 (free full text).
  10. ↑ Tabea Seeliger, Nils K. Prenzler, Stefan Gingele, Benjamin Seeliger, Sonja Körner, Thea Thiele, Lena Bönig, Kurt-Wolfram Sühs, Torsten Witte, Martin Stangel, Thomas Skripuletz: Neuro-Sjögren: Peripheral Neuropathy With Limb Weakness in Sjögren's Syndrome . In: Front Immunol. July 11, 2019, doi : 10.3389 / fimmu.2019.01600 , PMID 26985175 , PMC 6637792 (free full text).
  11. ^ Ana-Luisa Stefanski: Diagnosis and Therapy of Sjögren's Syndrome, The diagnosis and treatment of Sjögren's syndrome. In: Dtsch Arztebl Int. 114 (20), 2017, pp. 354-361. doi: 10.3238 / arztebl.2017.0354