What Is ANOTHER Syndrome?

Sjogren's syndrome (SS) is a chronic inflammatory autoimmune disease mainly involving exocrine glands, also known as autoimmune exocrine gland epithelial cell disease or autoimmune exocrine disease. In addition to the loss of salivary glands and lacrimal glands in clinic and dry mouth and dry eyes, there are also other exocrine glands and other organs outside the glands that are involved in multi-system damage. There are a variety of autoantibodies and hyperimmunoglobulinemia in the serum. The disease is divided into two categories, primary and secondary.

Basic Information

Also known as

siccasyndrome; Sjögren syndrome

English name

Sjogrensyndrome; SS

English alias

siccasyndrome

Visiting department

Internal medicine

Multiple groups

40 to 50 year old women

Common locations

Exocrine glands

Common symptoms

Dry mouth and eyes

Contagious

no

Clinical manifestations of Sjogren's syndrome

The onset of this disease is mostly hidden, and the clinical manifestations are diverse.

Local performance

(1) Xerostomia due to salivary gland lesions, which causes salivary mucin deficiency, causes the following common symptoms: Most patients complain of dry mouth. In severe cases, oral mucous membranes, teeth and tongue are sticky, so they need to drink water frequently during speech When entering solid food, it must be taken with water or liquid food. Critical caries is one of the characteristics of this disease. About 50 patients have multiple dental caries that are difficult to control and develop. The teeth are gradually blackened, and then small pieces fall off, leaving only residual roots. Mumps in adults, 50 patients showed intermittent alternating parotid swelling and pain, involving unilateral or bilateral. Most of them resolve on their own in about 10 days, but sometimes they are persistently swollen. A few have submandibular glands and less sublingual glands. Tongue manifests as tongue pain, dry and cracked tongue surface, tongue nipples shrink and smooth. Oral ulcers or secondary infections.

(2) Dry keratoconjunctivitis Due to the reduction of mucin secreted by the lacrimal gland, symptoms such as dry eyes, foreign body sensation, and less tears appear. In severe cases, there is no tears. Some patients have recurrent purulent infection, conjunctivitis, and keratitis.

(3) Other superficial parts such as the nose, hard palate, trachea and its branches, the exocrine glands of the mucosa of the digestive tract, and the vaginal mucosa can be affected, resulting in less secretion and corresponding symptoms.

2. System performance

In addition to dry mouth and eyes, patients may also develop systemic symptoms such as fatigue and low fever. System damage occurs in about two thirds of patients.

(1) Allergic purpura-like rash can occur on the skin, which is more common in the lower extremities. It is a red papul with a clear boundary of rice grain size. It does not fade and appears in batches. Each batch lasts about 10 days and can resolve on its own with brown pigmentation.

(2) Joints Joint pain is more common, and joint structure is not damaged.

(3) Kidney About half of the patients have renal damage, mainly involving the distal tubules, and renal tubular acidosis can occur. A small number of patients have more obvious glomerular damage, clinical manifestations are a large number of proteinuria, hypoalbuminemia, and even renal insufficiency.

(4) Lungs Most patients have no respiratory symptoms. The mildly affected person developed dry cough, and the severely affected person had shortness of breath. The main pathology of the lung is interstitial disease, and a small number of patients develop pulmonary hypertension. Patients with pulmonary fibrosis and severe pulmonary hypertension have a poor prognosis.

(5) Digestive system Non-specific symptoms such as atrophic gastritis, decreased gastric acidity, and indigestion may occur, and patients may have liver damage.

(6) Nerves A few involve the nervous system. Peripheral nerve damage is more common.

(7) Blood system This disease may appear to reduce white blood cell count or (and) thrombocytopenia, severe bleeding may occur in patients with low platelet count. The incidence of lymphoma in this disease is much higher than that in the normal population.

Sjogren's syndrome diagnosis

The currently recognized diagnostic criteria are the International Classification (Diagnosis) criteria for Sjogren's syndrome in 2002, as follows:

Table 1 Items of classification criteria for Sjogren's syndrome

. One or more of the three symptoms of oral symptoms .

1. Daily dry mouth lasts for more than 3 months;

2. Repeated or persistent swelling of the parotid gland in adulthood;

3. Use water when swallowing dry food.

. One or more of the three eye symptoms

1. Dry eyes that feel intolerable daily for more than 3 months;

2. There is repeated feeling of sand entering eyes or sanding;

3. Use artificial tears 3 or more times a day.

. Eye signs Any one or more of the following tests are positive

1. Schirmer I test (+);

2. Corneal staining (+).

. Histological examination The pathology of the lower labial gland shows lymphocytic foci

. Salivary gland damage Any one or more of the following tests are positive

1. saliva flow rate (+);

2. Parotid angiography (+);

3. Salivary gland isotope examination (+).

. Autoantibodies anti-SSA or anti-SSB (+) (double diffusion method)

Table 2 Specific classification of the above items

1. Primary Sjogren's Syndrome Without any underlying disease, the following two can be diagnosed:

a. It meets 4 or more in Table 1, but must contain item IV (histological examination) and / or item VI (autoantibodies);

b. Three of the items III, IV, V, and VI are positive.

2. Patients with secondary Sjogren's Syndrome have underlying diseases (such as any connective tissue disease), and meet any one of Tables I and II, and also meet any of Articles III, IV, and V.

3. Must be excluded . History of neck, head and face radiotherapy, hepatitis C virus infection, AIDS, lymphoma, sarcoidosis, GVH disease, application of antiacetylcholine drugs (such as atropine, scopolamine, bromoproline, belladonna, etc.)

Differential diagnosis of Sjogren's syndrome

Systemic lupus erythematosus

Sjogren's syndrome is more common in middle-aged and older women. Fever, especially high fever, is rare. There is no rash on the crotch, obvious dry mouth and eyes. Renal tubular acidosis is the most common and major kidney damage. Hyperglobulinemia is obvious. Complementary blood disease is rare.

2. Rheumatoid arthritis

Sjogren's syndrome rarely has joint bone destruction, deformity, and limited function. People with rheumatoid arthritis rarely have anti-SSA and anti-SSB antibodies.

3. Dry mouth for non-autoimmune diseases

Such as the decline of senile exocrine gland function, diabetic or drug-induced dry mouth depends on the history and the characteristics of each disease to identify.

Sjogren's syndrome treatment

There is currently no cure for this disease. Mainly to take measures to improve symptoms, control and delay the progress of tissue and organ damage caused by the immune response, and secondary infections.

Improve symptoms

(1) Reduce the symptoms of dry mouth, keep the mouth clean, rinse your mouth frequently, and reduce the possibility of dental caries and secondary oral infections.

(2) Dry keratoconjunctivitis can be given artificial tear drops to reduce dry eye symptoms and prevent corneal damage.

(3) Non-steroidal anti-inflammatory drugs and hydroxychloroquine can be used for muscle and joint pain.

2. Systemic damage should be treated with damaged organs and severity

For patients with neurological diseases, glomerulonephritis, interstitial lung disease, liver damage, low blood cells, especially low platelets, and myositis, adrenal corticosteroids should be given at the same dosage as other connective tissue disease treatments. For those with rapid disease progression, immunosuppressants such as cyclophosphamide and azathioprine can be combined. Patients with malignant lymphoma should be treated with combined chemotherapy in an active and timely manner.