Syndrome are two types of initial and secondary Primary SS, where there is no satisfactory facilities, while the Secondary SS is associated with rheumatic disease such as SLE, rheumatoid arthritis, RA, scleroderma Scleroderma.
Pathogenesis (Figure 1)
Is still not well understood, but there is a relationship with the factions of the disease tissue (HLA-DR3 / HLA-B8), especially among the white race, as recent studies have focused on the role in the pathogenesis APOPTOSIS.
As a result of damage to cells and blueberry Alguenoah no decrease in the secretion of saliva and tears, resulting in injuries to the cornea and conjunctiva and oral mucosa while the demonstrations outside the gland is due to the presence of antibodies to ANA and RF.
Studies also recalls that incitement prolonged continuous B cells may lead to disruption in the differentiation and maturity, raising the incidence of lymphomas in patients with this syndrome.
Common:
Happens in the USA increased by 0.1 - 4%.
Race: all races.
Gender: Females
Symptoms:
Most common symptoms are dry tears and dry saliva, described by the patient's inability to eat dry as a result of attachment to the roof the mouth and puts the patient's part is always a glass of water, especially at night, leading to nocturnal urination Nocturia, and the patient's hoarseness of the voice and difficulty in speaking for a long time, and getting a Sunni unrest with the change in the sense of taste in these patients may be observed for some fungal infections (Candida) with inflammation of the corners of the mouth and oral pain.
As for dry eyes, appears to the patient as itchy, painful, with a sense of the presence of a foreign body or sand in the eyes may find inflammation of the eyelids, and increase the severity of these symptoms during the day.
The symptoms: the magnitude of the bilateral parotid gland often. (Figure 2)
Other symptoms:
Skin: dry skin - itching - Verwriaat palpable - Raynaud's phenomenon (20% of patients).
Lung: dry respiratory mucosa with dry cough and a slip at the association with interstitial lung disease - bronchitis frequently - of the lung.
Gastrointestinal: difficulty swallowing dry secretions - a sense of heartburn caused by two reasons, first is the gastro-esophageal reflux GERD and the second reduced amount of saliva, which reduces the acid cleaning of the esophagus - stomach pain - diarrhea - and rarely Bankeraas inflammation.
Heart attack: the high tension pneumonia - inflammation of the heart.
Nerve: ill optical nerve - peripheral neuropathy.
Renal: renal tubular acidosis - interstitial nephritis leading to osteomalacia.
Other symptoms Mtalqh to SLE-RA-scleroderma-Idiobathic pulmonary fibrosis
Examination estoppel:
Examination shows the following assets are not required meeting in one patient:
1. Expansion vessels conjunctivitis with reduced amount of tears, where testing is conducted Schirmer, a bar, particularly of paper is placed on the conjunctiva bottom for five minutes, if wet <15 mm of paper in five minutes is normal but less than 5 mm is satisfactory (but test is not specific). (Figure 3)
2. Reduced amount of saliva accumulated under the tongue, sticking tongue with tongue depressor. (Figure 4)
3. Oral Candida (patches of white smudge) - Lack of tongue papillae - cracking of the tongue - oral erythema - Alsuarin inflammation.
4. Magnitude of the bilateral parotid side (unilateral = tumor doubt and thus indicated for biopsy).
5. Arthralgia - signs of inflammation of the joints (If the symmetrical and polyarticular rheumatoid TB Vick and .......) note that one third of patients with rheumatoid arthritis have a SS.
6. Khrakhar bilateral basal side caused by interstitial lung disease.
7. Verwriaat on the lower limbs may ulcerate.
Measure Diagnostic Work Up:
Laboratory:
• thrombocytopenia ← Primary SS, but it may also occur in the. SLE
• defect in white blood cell malignancies ← lymphocytes.
• ← electrophoresis shows the lack of serum albumin and increase the level of total protein.
• Antibodies: we may find all of the positive RF / ANA / antibodies to phospholipids / globulins village, but what distinguishes the SS is the presence of antibodies in particular are:
1. SSA/R0 to 50% of patients and mothers with newborn babies living with Neonatal Lupus Syndrome
2. SSB / La in 40-50% of the Primary SS and 15% of patients with SLE
Note: The levels of these antibodies do not reflect the effectiveness and the severity of the disease.
Radiologically:
• imaging the salivary glands to deny ← narrowing or blockage of the salivary ducts (non-specific).
• Shooting Alomadana of salivary glands to assess ← secretory function of the glands (non-specific).
Other surveys:
1. Sialometry Sialometry (Figure 5)
2. Chemical examination of the saliva shows the rise of both sodium and chlorine ions and Allambrovrin and IgA ......... (All non-specific).
3. Parotid gland biopsy: a test for the development of better diagnosis, but does not always take place only when you doubt the diagnosis and helps in the differential diagnosis for which we find Alarchoid tissue Habibomat Mottagbna and is the most important textile assets of the disease (Figure 6)
- Clusters of lymphocytes and plasma cells are less and Balat.
- The absence of the natural structure of the glands (bilboes) and replace them with lymphocytes.
- These changes include all the salivary glands often.
American-European Consensus Criteria
Diagnosis requires the presence of 4 out of 6 criteria to meet the criteria 5 or 6 and these standards are as follows:
1 - symptoms of in-kind: drought for more than 3 months, foreign body sensation in eyes
2 - Symptoms of oral dryness, swelling of the parotid gland frequently, drink fluids frequently to facilitate swallowing
3 - signs of in-kind: a positive Schirmer test
4 - signs of oral: assets and radiologically satisfactory Sialometry
5 - Parotid biopsy positive
6 - positive antibodies Anti SSB / SSA
As for the Secondary SS Vch_khas when accompanied by a disease of connective tissue disease with symptoms of dry mouth or eye with a positive Criteria 3 - 4 - 5
The application of the above criteria gives the sensitivity in the diagnosis of about 97.2%.
Treatment
Often incidental
1 - dry eyes: Artificial water, soften the atmosphere, avoid medications with anticholinergic effects or anti-histamine, kind of night creams, topical steroids, cyclosporine A, tetracycline, anti-inflammatory systemic and topical Vit A.
2 - dry mouth: a lot of drinking water, drops of lemon to induce the secretion of saliva, periodic follow-up of dentists, treatment of fungal infections (nystatin / fluconazole) and there are studies on the future role of interferon alpha.
3 - Dry skin: moisturizing creams.
4 - arthritis: inflammation Allastairoidah treated with NSAIDs ..
5 - to accompany systemic infections: involved steroids and immune suppression.
Cyclophosphamide may be useful in the treatment of severe events in the SS with the attention that it may help the development of lymphomas.
A recent study showed 2010 that Rituximab improves salivary flow rate significantly as well as the function of lacrimal glands in patients with Primary SS.
The role of surgery in: SS
1 - parotid biopsy for diagnosis.
2 - enlarged lymph node biopsy to rule out lymphoma.
3 - surgical correction of lacrimal duct obstruction with the use of less attention to the possible amount of drugs during anesthesia Colin.
Complications:
1 - complications associated cases: SLE / RA.
2 - parotid gland infections (staphylococcal - streptococci - Streptococcus pneumoniae).
3 - parotid gland tumors, where the severity and magnitude of the observed one-sided.
4 - lymphoma, lymphoma and false.
5 - Neonatal lupus syndrome and the siege of my heart and the Valley of the children of pregnant mothers who have antibodies SSA/R0
6 - Alfosfolipid antibody syndrome.
Warning:
Usually good, but when the association with other diseases, the type of disease-warning facilities
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