Diagnostic points]
Symptoms and signs
Oral symptoms
Dry mouth every day for more than 3 months, need to drink frequently, wake up in the middle of the night to drink water, etc;
Recurrent or persistent enlargement of the parotid gland after adulthood;
Difficulty in swallowing dry food, must use water to assist;
Rampant dental caries, dry and cracked tongue, oral cavity often secondary to mycobacterial infection;
Eye symptoms
Daily unbearable dry eyes that last for more than 3 months;
Feeling of repeated “sand” blowing into the eye or a feeling of abrasiveness;
Need to use artificial tears 3 or more times a day;
Other symptoms include vaginal dryness, dry itchy skin, clinical or subclinical renal tubular acidosis, or other systemic symptoms as described above.
Ancillary Examination
Ocular
Schirmer (filter paper) test (+), i.e. ≤5mm/5 points (>5mm/5 points in normal subjects);
Corneal staining(+), >10 staining spots in each eye;
Tear film fragmentation time(+), i.e. ≤10 seconds (>10 seconds in normal subjects);
Oral cavity
Salivary flow rate(+), i.e. only natural outflow of saliva ≤1.5ml was collected in 15 minutes (>1.5ml in normal subjects);
② Parotid gland contrast (+), i.e., spillage of contrast agent from the terminal gland is seen as a dotted or globular shadow;
Salivary gland nuclide examination (+), i.e. poor absorption, concentration and excretion of nuclide from the salivary gland;
Histological examination of the lacrimal gland biopsy (+), i.e. 50 lymphocytes aggregated in 4 mm2 tissue is called a foci, and any foci showing ≥1 lymphocytes is (+).
Urine PH multiple times >6 necessitates further examination of indicators related to renal tubular acidosis.
Peripheral blood tests may reveal low platelets or occasionally hemolytic anemia.
Serum immunological tests
Anti-SSA antibodies are the most common autoantibodies in this disease and are seen in 70% of patients;
Anti-SSB antibodies are said to be the marker antibodies of the disease and are seen in 45% of patients;
hyperimmunoglobulinemia, both polyclonal, seen in 90% of patients.
Other tests such as pulmonary imaging and liver and kidney function measurements may reveal evidence of corresponding systemic damage.
Diagnostic criteria
The international classification (diagnosis) criteria of dry syndrome in 2002 are as follows.
Table 1 Items of dry syndrome classification criteria
I. Oral symptoms: 1 or more of the following 3 items
1.Sensation of dry mouth daily for more than 3 months
2.Recurrent or persistent enlargement of parotid glands in adulthood
3.Swallowing dry food with the help of water
II. Eye symptoms: 1 or more of the following 3 items
1. Daily unbearable dry eyes for more than 3 months
2.Repeated sand in the eye or gritty feeling
3.Need to use artificial tears 3 times or more per day
III. Ocular signs: Positive for any one or more of the following tests
1, Schirmer I test (+) (£5mm/5 points)
2. Corneal staining (+) (³4 van Bijsterveld scoring method)
IV. Histological examination: pathology of the lower lacrimal gland shows foci of lymphocytes ³1 (referring to a foci of at least 50 lymphocytes aggregated in the interstitium of the lacrimal gland in 4mm2 tissue).
V. Salivary gland damage: Positive for any 1 or more of the following tests
1. Salivary flow rate (+) (£1.5 ml/15 min)
2.Parotid gland angiography(+)
3.Salivary gland isotope examination(+)
VI, autoantibodies: anti-SSA or anti-SSB (+) (double diffusion method)
Table 2 Specific classification of the above items
1.Primary dry syndrome: In the absence of any underlying disease, the diagnosis is made if 2 of the following items are present.
4 or more items in Table 1 are met, but must contain entry IV (histological examination) and/or entry VI (autoantibodies);
b. Positive for any 3 of the 4 entries III, IV, V, VI.
2.Secondary dry syndrome: The patient has an underlying disease (such as any connective tissue disease) while meeting any 1 of entries I and II of Table 1 and any 2 of entries III, IV, V.
3. Must exclude: history of cervical head and facial radiation therapy, hepatitis C virus infection, AIDS (AIDS), lymphoma, nodular disease, graft-versus-host (GVH) disease, and application of anti-acetylcholine drugs (e.g., atropine, scopolamine, bromopamine tylenol, belladonna, etc.).
This disease needs to be differentiated from the following diseases
Systemic lupus erythematosus Dry syndrome is mostly seen in middle-aged and elderly women, fever, especially high fever is uncommon, no zygomatic rash, dry mouth and eyes are obvious, renal tubular acidosis is its common and main renal damage, hyperglobulinemia is obvious, hypocomplementemia is rare, prognosis is good.
Rheumatoid arthritis The symptoms of joint inflammation in dry syndrome are much less pronounced and severe than in rheumatoid arthritis, and there is rarely joint bone destruction, deformity and functional limitation. Anti-SSA and anti-SSB antibodies are rarely present in those with rheumatoid arthritis.
Dry mouth in non-autoimmune diseases, such as those caused by age-related decline in exocrine gland function, diabetes mellitus or medications, depends on the medical history and the individual characteristics of each disease to differentiate them.
Treatment options and principles]
There is no cure for this disease. The main measures are to improve the symptoms, control and delay the progression of tissue and organ damage caused by the immune response and secondary infection.
Improvement of symptoms
It is difficult to reduce dry mouth, so you should stop smoking, drinking alcohol and avoid taking drugs that cause dry mouth, such as atropine. Keep the mouth clean and rinse the mouth regularly to reduce the possibility of dental caries and secondary infections in the mouth. Overseas, parasympathetic acetylcholine stimulants such as pilocarpine tablets and similar products can be taken to stimulate the secretion of undamaged glands in salivary glands to improve dry mouth symptoms. They have some efficacy but also more adverse effects such as sweating and frequent urination.
Dry keratoconjunctivitis can be treated with artificial tear drops to reduce dry eye symptoms and prevent corneal damage. Some eye ointments can also be used to protect the cornea. Overseas, some people use self-serum treated with eye drops.
For muscle and joint pain, NSAIDs can be used.
Hypokalemia: To correct hypokalemic episodes of paralysis, intravenous potassium supplementation (potassium chloride) can be used. After the condition stabilizes, oral potassium salt solution or tablets are used instead, and some patients need to take them for life to prevent the recurrence of hypokalemia. Most patients can still live and work normally after the correction of hypokalemia.
Systemic damage should be treated according to the severity of the damaged organ. Adrenocorticotropic hormone should be given in the same doses as in other connective tissue diseases in cases of combined neurological, glomerulonephritis, interstitial lung lesions, liver damage, low blood cells, especially low platelets, and myositis. Immunosuppressive agents such as cyclophosphamide and azathioprine may be used in combination with other drugs for rapidly progressive disease. Combination chemotherapy is recommended for those with malignant lymphoma in an aggressive and timely manner.
Prognosis
The prognosis of this disease is good. Most of the patients with visceral damage can be controlled to achieve remission after appropriate treatment, but the disease can recur after stopping treatment. Among the visceral damage, those with progressive pulmonary fibrosis, central neuropathy, glomerular damage with renal insufficiency, and malignant lymphoma have a poor prognosis, while the rest of the systemic damage are mostly in remission and even resume daily life and work after appropriate treatment.
Diagnostic points]
Symptoms and signs
Oral symptoms
1. Dry mouth for more than 3 months, need to drink water frequently, wake up in the middle of the night to drink water, etc;
2.Recurrent or persistent enlargement of parotid gland after adulthood;
3.Swallowing dry food is difficult and must be assisted by water;
4.Rampant dental caries, dry and cracked tongue, oral cavity often secondary to mycobacterial infection;
Eye symptoms
1.Insurmountable daily dryness of the eyes lasting for more than 3 months;
2.Feeling the feeling of repeated “sand” blowing into the eye or frosting sensation;
3.Need to use artificial tears 3 or more times a day;
Other symptoms include vaginal dryness, dry itchy skin, clinical or subclinical renal tubular acidosis, or other systemic symptoms as described above.
Auxiliary examination
Ophthalmology
1.Schirmer (filter paper) test (+), i.e. ≤5mm/5min (>5mm/5min in normal subjects);
2, corneal staining (+), >10 staining points in each eye;
3, tear film fragmentation time (+), i.e. ≤10 seconds (>10 seconds in normal subjects);
Oral cavity
1.Salivary flow rate(+), i.e. only natural outflow of saliva ≤1.5ml was collected in 15 minutes (normal people >1.5ml);
2, parotid gland contrast (+), i.e., spillage of contrast agent from the terminal gland is seen as a dotted or globular shadow;
3, salivary gland nuclear examination (+), i.e. poor absorption, concentration and discharge of nuclide from salivary glands;
4, lipid gland biopsy histological examination (+), that is, in 4mm2 tissue with 50 lymphocytes aggregation is called a foci, where there are lymphocytes foci ≥ 1 for (+).
5, urine PH many times > 6 then it is necessary to further check the indicators related to renal tubular acidosis.
6, Peripheral blood tests can reveal low platelets or occasionally hemolytic anemia.
Serum immunological examination
1.Anti-SSA antibody is the most common autoantibody in this disease, which is found in 70% of patients;
Anti-SSB antibodies are said to be the marker antibodies of the disease and are seen in 45% of patients;
3. hyperimmunoglobulinemia, all polyclonal, seen in 90% of patients.
Others, such as pulmonary imaging and liver and kidney function measurements, may reveal evidence of corresponding systemic damage.
Diagnostic criteria
The international classification (diagnosis) criteria of dry syndrome in 2002 are as follows.
Table 1 Items of dry syndrome classification criteria
I. Oral symptoms: 1 or more of the following 3 items
1.Sensation of dry mouth daily for more than 3 months
2.Recurrent or persistent enlargement of parotid glands in adulthood
3.Swallowing dry food with the help of water
II. Eye symptoms: 1 or more of the following 3 items
1. Daily unbearable dry eyes for more than 3 months
2.Repeated sand in the eye or gritty feeling
3.Need to use artificial tears 3 times or more per day
III. Ocular signs: Positive for any one or more of the following tests
1, Schirmer I test (+) (£5mm/5 points)
2. Corneal staining (+) (³4 van Bijsterveld scoring method)
IV. Histological examination: pathology of the lower lacrimal gland shows foci of lymphocytes ³1 (referring to a foci of at least 50 lymphocytes aggregated in the interstitium of the lacrimal gland in 4mm2 tissue).
V. Salivary gland damage: Positive for any 1 or more of the following tests
1. Salivary flow rate (+) (£1.5 ml/15 min)
2.Parotid gland angiography(+)
3.Salivary gland isotope examination(+)
VI, autoantibodies: anti-SSA or anti-SSB (+) (double diffusion method)
Table 2 Specific classification of the above items
1.Primary dry syndrome: Without any underlying disease, the diagnosis is made if 2 of the following items are present
a. Four or more of the items in Table 1 are met, but must contain entry IV (histological examination) and/or entry VI (autoantibodies);
b. Positive for any 3 of the 4 entries III, IV, V, VI.
2.Secondary dry syndrome: The patient has an underlying disease (such as any connective tissue disease) while meeting any 1 of entries I and II of Table 1 and any 2 of entries III, IV, V.
3. Must exclude: history of cervical head and facial radiation therapy, hepatitis C virus infection, AIDS (AIDS), lymphoma, nodular disease, graft-versus-host (GVH) disease, and application of anti-acetylcholine drugs (e.g., atropine, scopolamine, bromopamine tylenol, belladonna, etc.).
This disease needs to be differentiated from the following diseases
1, systemic lupus erythematosus Dry syndrome is mostly seen in middle-aged and elderly women, fever, especially high fever is uncommon, no zygomatic rash, dry mouth and eyes are obvious, renal tubular acidosis is its common and main renal damage, hyperglobulinemia is obvious, hypocomplementemia is rare, good prognosis.
2.Rheumatoid arthritis
The symptoms of joint inflammation in dry syndrome are much less obvious and serious than rheumatoid arthritis, and there are rarely joint bone destruction, deformity and functional limitation. Rheumatoid arthritis people rarely appear anti-SSA and anti-SSB antibodies.
3, non-autoimmune diseases of dry mouth, such as aging exocrine gland function decline, diabetes or drug-induced dry mouth depends on the medical history and each disease’s own characteristics to identify.
Treatment options and principles]
There is no cure for this disease. The main measures are to improve the symptoms, control and delay the progression of tissue and organ damage caused by the immune response and secondary infection.
Improvement of symptoms
1. It is difficult to reduce dry mouth, so stop smoking, drinking alcohol and avoid taking drugs that cause dry mouth, such as atropine. Keep your mouth clean and rinse your mouth regularly to reduce the possibility of dental caries and oral secondary infections. Overseas, parasympathetic acetylcholine stimulants such as pilocarpine tablets and similar products can be taken to stimulate the secretion of undamaged glands in salivary glands to improve dry mouth symptoms. They have certain efficacy but also more adverse reactions such as sweating and frequent urination.
2, dry keratoconjunctivitis can be given artificial tear drops to reduce dry eye symptoms and prevent corneal damage. Some eye creams can also be used to protect the cornea. Some people abroad use autologous serum to treat the eye drops.
3, muscle, joint pain available non-steroidal anti-inflammatory drugs.
4, hypokalemia: correction of hypokalemic paralytic episodes can be used intravenous potassium supplementation (potassium chloride), to be stable after the change to oral potassium salt solution or tablets, some patients need to take lifelong, to prevent the reoccurrence of hypokalemia. Most patients can still live and work normally after the correction of hypokalemia.
Systemic damage should be treated according to the severity of the damaged organ. Adrenocorticotropic hormone should be given in the same doses as in other connective tissue diseases in cases of combined neurological, glomerulonephritis, interstitial lung lesions, liver damage, low blood cells, especially low platelets, and myositis. Immunosuppressive agents such as cyclophosphamide and azathioprine may be used in combination with other drugs for rapidly progressive disease. Combination chemotherapy is recommended for those with malignant lymphoma in an aggressive and timely manner.
Prognosis
The prognosis of this disease is good. Most of the patients with visceral damage can be controlled to achieve remission after appropriate treatment, but the disease can recur after stopping treatment. Among the visceral damage, those with progressive pulmonary fibrosis, central neuropathy, glomerular damage with renal insufficiency, and malignant lymphoma have a poor prognosis, while the rest of the systemic damage are mostly in remission and even resume daily life and work after appropriate treatment.