Sjögren's syndrome overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Farima Kahe M.D. [2] Ayesha A. Khan, MD[3]

Overview

In 1892, Johann von Mikulicz-Radecki was to first to describe a patient with enlargement of the parotid and lacrimal glands associated with a round-cell infiltrate and acinar atrophy. In 1933, Henrik Sjögren was the first to describe 19 females with clinical and pathological manifestations of the Sjögren's syndrome. American-European Consensus Group(AECG) and American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2002 and 2012 according to clinical findings. Common causes of Sjögren's syndrome include viral infection such as Epstein-Barr virus (EBV), Coxsackie virus, Hepatitis C virus, Cytomegalovirus (CMV), Human herpesvirus 6 (HHV-6), Retroviruses, and genetic factors. The incidence of Sjögren's syndrome is approximately 4 per 100,000 individuals worldwide. The prevalence of Sjögren's syndrome is approximately 43 per 100,000 individuals worldwide. Female are more commonly affected by Sjögren's syndrome than male. The majority of Sjögren's syndrome cases are reported in China, Japan, and California. Common risk factors in the development of Sjögren's syndrome include family history of autoimmune diseases, serological markers such as low complement levels and cryoglobulinemia and parotid gland enlargement. The symptoms of Sjögren's syndrome usually develop in the 4th and 5th decade of life, and start with symptoms such as ocular and oral dryness. Common complications of Sjögren's syndrome include blurred vision and corneal damage, optic neuritis, and lymphoma. Prognosis is generally good and presence of low complement level is associated with a particularly poor prognosis among patients with Sjögren's syndrome. The most common symptoms of Sjögren's syndrome include ocular and oral symptoms. Patients with Sjögren's syndrome may have a positive history of rheumatoid arthritis (RA), systemic lupus erythematous (SLE) and non-Hodgkin B-cell lymphoma. Physical examination of patients with is usually remarkable for dryness of all mucous membranes such as mouth, eyes, lips, anal and rectal. Laboratory findings consistent with the diagnosis of Sjögren's syndrome include elevated erythrocyte sedimentation rate (ESR), cytopenia, the presence of anti-SSA/Ro, anti-SSB/La. Findings on an ultrasound suggestive of Sjögren's syndrome are hypoechoic and inhomogeneous salivary glands, parenchymal inhomogeneity in the submandibular glands and focal or diffuse hypoechoic or anechoic foci in glands. Parotid gland CT scan may be helpful in the diagnosis of Sjögren's syndrome and finding include abnormal diffuse fat tissue deposition and diffuse punctate calcification. The most commonly used tests for dry eyes of Sjögren's syndrome include Schirmer's test, ocular surface staining and tear break-up time. Pharmacologic medical therapies for dry eye, dry mouth, and other sicca symptoms of Sjögren's syndrome are pilocarpine, cevimeline and artificial tears. The mainstay of treatment for Sjögren's syndrome is medical therapy. Surgery is usually reserved for patients with occlusion of the lacrimal puncta, salivary gland malignancy and recurrent parotitis refractory to medical management.

Historical Perspective

In 1892, Johann von Mikulicz-Radecki was to first to describe a patient with with enlargement of the parotid and lacrimal glands associated with a round-cell infiltrate and acinar atrophy. In 1933, Henrik Sjögren was the first to describe 19 females with clinical and pathological manifestations of the Sjögren's syndrome.

Classification

American-European Consensus Group(AECG) and American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2002 and 2012 according to clinical findings.

Pathophysiology

Sjögren's syndrome (SS) is a chronic autoimmune disorder that can affect several organ systems. Sjögren's syndrome is classified into a "primary" form that is a separate entity from other well-defined autoimmune disorders and a "secondary" form that is associated with other well-defined autoimmune conditions, such as SLE, rheumatoid arthritis, progressive systemic sclerosis, and primary biliary cirrhosis. These forms of Sjögren's syndrome are different in their serologic and histopathologic findings as well as their genetic components. Both genetic and immune factors contribute to the pathogenesis of the disease. In the most common presentation of Sjögren's syndrome, lymphocytes infiltrate the lacrimal and salivary glands and impair their function, hence causing the main characteristic symptoms such as dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca). CD4+ T-cells are predominant in mild and moderate salivary gland infiltrations, while B cells play the major role in severe lesions. Sjögren's syndrome may also manifest itself with dryness of skin and other mucosal surfaces or even cause systemic extraglandular disturbances such as arthritis, vasculitis, renal, pulmonary, hematopoietic, and neurologic involvement. In general, a combination of lymphocytic infiltration, B lymphocyte hyperreactivity, production of certain autoantibodies, genes mostly involved in the production of MHC molecules and certain viral infections which are all linked to the pathogenesis of Sjögren's syndrome.

Causes

Common causes of Sjögren's syndrome include viral infection such as Epstein-Barr virus (EBV), Coxsackie virus, Hepatitis C virus, Cytomegalovirus (CMV), Human herpesvirus 6 (HHV-6), Retroviruses and genetic factors.

Epidemiology and Demographics

The incidence of Sjögren's syndrome is approximately 4 per 100,000 individuals worldwide. The prevalence of Sjögren's syndrome is approximately 43 per 100,000 individuals worldwide. The mortality rate of Sjögren's syndrome is approximately 1.38. Female are more commonly affected by Sjögren's syndrome than male. The majority of Sjögren's syndrome cases are reported in China, Japan, and California.

Risk Factors

Common risk factors in the development of Sjögren's syndrome include family history of autoimmune diseases, serological markers such as low complement levels and cryoglobulinemia and parotid gland enlargement.

Screening

There is insufficient evidence to recommend routine screening for Sjögren's syndrome.

Natural History, Complications, and Prognosis

Natural History

The symptoms of Sjögren's syndrome usually develop in the 4th and 5th decade of life, and start with symptoms such as ocular and oral dryness.

Complications

Common complications of Sjögren's syndrome include blurred vision and corneal damage, optic neuritis and lymphoma.

Prognosis

Prognosis is generally good and presence of low complement level is associated with a particularly poor prognosis among patients with Sjögren's syndrome.

Diagnosis

History and Symptoms

The most common symptoms of Sjögren's syndrome include ocular and oral symptoms. Patients with Sjögren's syndrome may have a positive history of rheumatoid arthritis (RA), systemic lupus erythematous (SLE) and non-Hodgkin B-cell lymphoma.

Physical Examination

Physical examination of patients with is usually remarkable for dryness of all mucous membranes such as mouth, eyes, lips, anal, and rectal.

Laboratory Findings

Laboratory findings consistent with the diagnosis of Sjögren's syndrome include elevated erythrocyte sedimentation rate (ESR), cytopenia, presence of anti-SSA/Ro, and anti-SSB/La.

Electrocardiogram

There are no ECG findings associated with Sjögren's syndrome.

Echocardiography and Ultrasound

Findings on an ultrasound suggestive of Sjögren's syndrome are hypoechoic and inhomogeneous salivary glands, parenchymal inhomogeneity in the submandibular glands and focal or diffuse hypoechoic or anechoic foci in glands. There are no echocardiography findings associated with Sjögren's syndrome.

CT scan

Parotid gland CT scan may be helpful in the diagnosis of Sjögren's syndrome and finding include abnormal diffuse fat tissue deposition and diffuse punctate calcification.

MRI

Findings on MRI suggestive of Sjögren's syndrome include loss of homogeneity kin signal intensity on T1-weighted MR images and fat saturation suppressed focal high-intensity areas on T1-weighted images.

Other Imaging Findings

There are no other imaging findings associated with Sjögren's syndrome.

Other Diagnostic Studies

The most commonly used tests for dry eyes of Sjögren's syndrome include Schirmer's test, ocular surface staining and tear break-up time.

Treatment

Medical Therapy

Pharmacologic medical therapies for dry eye, dry mouth, and other sicca symptom of Sjögren's syndrome are pilocarpine, cevimeline and artificial tears.

Surgery

The mainstay of treatment for Sjögren's syndrome is medical therapy. Surgery is usually reserved for patients with occlusion of the lacrimal puncta, salivary gland malignancy, and recurrent parotitis refractory to medical management.

Primary Prevention

Sjögren's syndrome is inherited condition, there is no particular way to prevent developing the disease.

Secondary Prevention

Secondary prevention by Smoking cessation. Smoking can irritate and dry out your mouth. Increase your fluid intake. Take sips of fluids, particularly water, throughout the day. Avoid drinking coffee or alcohol since they can worsen dry mouth symptoms. Also avoid acidic beverages such as colas and some sports drinks because the acid can harm the enamel of your teeth. Stimulate saliva flow. Sugarless gum or citrus-flavored hard candies can boost saliva flow. Because Sjogren's syndrome increases your risk of dental cavities, limit sweets, especially between meals. Try artificial saliva. Saliva replacement products often work better than plain water because they contain a lubricant that helps your mouth stay moist longer. These products come as a spray or lozenge.