Sjögren's Syndrome

 

Shilpa. S

 

ABSTRACT:

Sjögren's syndrome is an immune system disorder characterized by dry eyes and dry mouth. With this disorder, the body's immune system attacks its own healthy cells that produce saliva and tears. Sjögren's syndrome often occurs with other such disorders, such as rheumatoid arthritis and lupus. Treatments include eye drops, medication and eye surgery.

 

 

INTRODUCTION:

Immunity is a state of responsiveness to foreign substances such as microorganisms and tumor proteins. Immune responses serve three functions; defense, homeostasis and surveillance. when the immune system is incompetent or under responsive, severe infections, immunodeficiency diseases, and malignancies may occur.

 

DEFINITION:

Sjögren's syndrome is a chronic autoimmune disease in which cells of the immune system destruct exocrine glands, specifically the salivary and lacrimal glands, that produce saliva and tears, respectively. The immune-mediated attack of the salivary and lacrimal glands lead to the development of dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca), respectively, in association with lymphocytic infiltration of the glands^.

 

 

HISTORY:

In 1930, Henrik Sjögren (1899-1986), an ophthalmologist in Jonkösping, Sweden, observed a patient with low secretions from the lacrimal and salivary glands Sjögren introduced the term keratoconjunctivitis sicca for the symptom of dry eye. In 1933, he published his doctoral thesis, describing 19 females, most of whom were postmenopausal and had arthritis, showing clinical and pathological manifestations of the syndrome. Sjögren clarifies that keratoconjunctivitis sicca, resulting from water deficiency, had no relation to xerophthalmia, resulting from vitamin A deficiency. After extensive research and data collection, Sjögren published an essential paper in 1951, describing 80 patients with keratoconjunctivitis sicca, 50 of whom also had arthritis. His subsequent follow-up conference trips pertaining to his paper led to an international interest in Sjogren’s syndrome The term keratoconjunctivitis sicca coined by Sjögren himself began to be identified as Sjögren’s syndrome in literature.

 

 

TYPES:

1.     Primary Sjogren’s syndrome:

The disease seen alone

 

2.     Secondary Sjogren’s syndrome:

The disease can be seen in association with other auto immune rheumatic diseases like rheumatoid arthritis, SLE, Scleroderma.

 

INCIDENCE AND PREVALENCE:

Disease affects predominantly in middle aged women (female to male ratio – 9:1), although it occurring in all ages. Prevalence of primary sjogren’s syndrome is 0.5-1% and 30% of patients with auto immune rheumatic disease suffer from secondary sjogren’s syndrome.

 

ETIOLOGY:

1.     Genetic Factors:

Polymorphisms of Human Leukocyte Antigen (HLA)-DR and HLA-DQ gene regions in Sjögren’s syndrome patients show differential susceptibility to the syndrome due to different types of the resulting autoantibody production

 

2.     Hormonal Factors:

Since Sjögren’s syndrome is associated with a high prevalence in women, sex hormones, especially estrogen, are believed to affect humoral and cell-mediated immune responses affecting susceptibility to the syndrome

 

3.     Microchimerism Factors:

Microchimerism of fetal cells (offspring lymphoid cells in maternal circulation) may generate autoimmunity in women who have been previously pregnant

 

4.     Environmental (exogenous) Factors:

Viral proteins, engulfed molecules, or degraded self-structures may initiate autoimmunity by molecular mimicry and increase the chances of Sjögren’s syndrome development. Epstein-Barr virus (EBV), Hepatitis C (HCV), and human T-cell leukemia virus-1 (HTLV-1) are among the most studied infectious agents in Sjögren’s syndrome.

 

SIGNS AND SYMPTOMS:

1.     Oral symptoms

Xerostomia, Inability to swallow dry foods, Inability to speak continuously, Burning sensation

 

Ocular symptoms:

Dry eyes, Gritty feeling in eyes, Burning, Decreased tearing

 

2.     Pulmonary manifestations:

Dry cough, Dyspnea

 

3.     Renal symptoms:

Nephritis

4.     Musculo skeletal manifestations:

Myalgia, arthralgia

 

5.     Other symptoms:

Dry nose, throat, Gastritis, Dyspareunia, Lymphoma, Parotid gland enlargement

 

DIAGNOSIS:

1.     Blood Test:

blood tests can be done to determine if a patient has high levels of antibodies that are indicative of the condition. Typical Sjögren's syndrome ANA patterns are SSA/Ro and SSB/La, of which SSB/La is far more specific; SSA/Ro is associated with numerous other autoimmune conditions but are often present in this also.

 

2.     Rose Bengal test:

 Rose Bengal test measures state and function of the lacrimal glands. This test includes a nontoxic dye call Rose Bengal which is placed on to the surface of the eyes. The dye’s distinctive colour help in determining the state and functioning of tear film and the rate of tear evaporation. If any distinctive colour change is observed will point towards the condition of Sjögren's syndrome.

 

3.   Schirmer's test:

Schirmer's test measures the production of tears: a strip of filter paper is held inside the lower eyelid for five minutes, and its wetness is then measured with a ruler. Producing less than five millilitres of liquid is usually indicative of Sjögren's syndrome.

 

4      Biopsy:

A lip/salivary gland biopsy can reveal lymphocytes clustered around salivary glands, and damage to these glands due to inflammation.

 

TREATMENT:

Oral involvement:

Xerostomia           Saliva substitution, stimulation of saliva secretion

Caries                    Dental check up, fluride

Candidiasis           Flucanazole (50-100mg/ day)

 

Respiratory involvement:

Humidification, Prevention of infections

 

Gastro intestinal involvement:

Atrophic gastritis       Proton pump inhibitors, vit B12 suppliments

 

COMPLICATIONS:

·       Neonatal Lupus Erythematosus with congenital heart block .

·       Type I cryoglobulinemia

 

 

REFERENCE:

1.      B K Davidson, C A Kelly (2010) , Primary Sjögren's syndrome in the North East of England: a long-term follow-up study. Journal of medical Association, 141(4): 534-542

2.      Nadine Sadiq Ali (2003), Headache in primary Sjogren’s Syndrome: a prevalence study Journal of neurology, 9(2):239-246.

3.      Brito-Zerón, P; Baldini, C; Bootsma, H; Bowman, SJ; Jonsson, R; Mariette, X; Sivils, K; Theander, E; Tzioufas, A; Ramos-Casals, M (7 July 2016). "Sjögren syndrome". Nature Reviews. Disease Primers. 2: 16047. doi:10.1038/nrdp.2016.47. PMID 27383445

4.      Ferri, Fred F. (2010). Ferri's differential diagnosis: a practical guide to the differential diagnosis of symptoms, signs, and clinical disorders (2nd ed.). Philadelphia, PA: Elsevier/Mosby. p. Chapter S. ISBN 978-0323076999.

5.      Singh, AG; Singh, S; Matteson, EL (March 2016). "Rate, risk factors and causes of mortality in patients with Sjögren's syndrome: a systematic review and meta-analysis of cohort studies". Rheumatology. 55 (3): 450–60.

6.      Manthorpe, R; Svensson, A; Wirestrand, LE (November 2004). "Late neonatal lupus erythematosus onset in a child born of a mother with primary Sjögren's syndrome". Ann. Rheum. Dis. 63 (11): 1496–7.

7.      Lidén M, Kristjánsson G, Valtýsdóttir S, Hällgren R (August 2007). "Gluten sensitivity in patients with primary Sjögren's syndrome". Scand. J. Gastroenterol. 42 (8): 962–7.

 

 

 

 

Received on 21.10.2019          Modified on 28.11.2019

Accepted on 30.12.2019     © A&V Publications all right reserved