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Background: Cevimeline hydrochloride is a cholinergic agent with muscarinic agonist activity prominently affecting the M1 and M3 receptors prevalent in exocrine glands. We evaluated the safety and efficacy of cevimeline in the treatment of xerostomia in patients with Sjogren syndrome. Methods: Seventy-five patients with Sjogren syndrome and associated salivary gland dysfunction were enrolled in a double-blind, randomized, placebo-controlled trial at 8 university- and office-based outpatient clinical facilities in the United States. Eligible study participants were randomized to receive 30 mg of cevimeline 3 times daily, 60 mg of cevimeline 3 times daily, or placebo for 6 weeks. Subjective responses were determined using global patient evaluation and visual analog scales. Salivary flow was measured objectively. Results: Sixty-one participants completed the study. Patients in both cevimeline groups had significant improvements in dry mouth, as indicated by symptoms, salivary flow, and use of artificial saliva, compared with the placebo group. The drug was generally well tolerated, with expected adverse events resulting from the drug's muscarinic agonist action. Fourteen patients withdrew from the study because of adverse events, the most frequent being nausea. Conclusions: Therapy with cevimeline, 30 mg 3 times daily, seems to be well tolerated and to provide substantive relief of xerostomia symptoms. Although both dosages of cevimeline provided symptomatic improvement, 60 mg 3 times daily was associated with an increase in the occurrence of adverse events, particularly gastrointestinal tract disorders. Use of 30 mg of cevimeline provides a new option for the treatment of xerostomia in Sjogren syndrome.

Although Sjogren's syndrome can affect the parasympathetic system, this does not cause eye dryness in most patients. Sjogren's syndrome is an autoimmune disease that affects the nervous system in about one-fourth of all patients. In a study of 41 patients, 15% had abnormal parasympathetic function.

Background: Ear involvement is not unusual in autoimmune diseases, but few data on this problem in the Sjogren syndrome are available. Objectives: To determine whether the incidence of hearing loss is increased in patients with the Sjogren syndrome and to determine what factors might be involved in the pathogenesis of Sjogren syndrome-related hearing loss. Design: Cross-sectional study. Setting: Secondary referral center in Italy. Patients: 30 women with the Sjogren syndrome were evaluated for evidence of audiovestibular disorder. Their results were compared with those of 40 age-matched healthy women. Measurements: Evaluation techniques included pure tone audiometry thresholds at octave frequencies of 125 Hz to 8000 Hz, tympanometry, and the stapedial reflex test in both ears. The presence of vasculitis, the Raynaud phenomenon, nervous system involvement, and serum anticardiolipin antibodies was recorded. Results: 14 patients with the Sjogren syndrome (46% [95% Cl, 28% to 66%]) had sensorineural hearing loss. Only one control (2.5% [Cl, 0.06% to 13%]) had a similar hearing impairment (P [is less than] 0.001). Nine of the 14 patients who had the Sjogren syndrome and sensorineural hearing loss (64% [Cl, 36% to 87%]) had anticardiolipin antibodies compared with only 3 controls (18% [Cl, 4% to 45%]) (P = 0.02). Conclusion: The high prevalence of hearing loss in the Sjogren syndrome supports the value of doing audiometric studies before excluding cranial nerve involvement in this disease. The correlation of sensorineural hearing loss with anticardiolipin antibodies must be investigated further., There may be a relationship between anticardiolipin antibodies and hearing loss in patients with Sjogren syndrome. Sjogren syndrome is an autoimmune disease. A study of 30 women with Sjogren syndrome and 40 healthy women found that 14 of the women with Sjogren syndrome had hearing loss compared to only one of the healthy women. Nine of the 14 women with Sjogren's and hearing loss had anticardiolipin antibodies, which are autoantibodies. Other autoimmune diseases such as lupus and rheumatoid arthritis are also associated with hearing loss.

It appears that among patients with definite primary Sjogren's syndrome (DSS) or probable SS (PSS) many have silent and symptom-free heart conditions. Doctors evaluated echocardiograms and pressure measurements of 54 patients with DSS, 10 patients with PSS and 21 healthy volunteers (the control group). The pericardium, or sac that surrounds the heart, was thickened in 33% of the 64 study patients and none of the control group. Study patients had higher calculated lung pressure values and lower diastolic blood pressure measurements than the control group.

Patients with both systemic sclerosis (SSc) and Sjogren's syndrome (SS) may be more likely to experience bronchial spasms. SSc is a system-wide hardening of connective tissue fibers. SS is a disorder that reduces the output of the salivary glands or tear ducts and is associated with a connective tissue disease such as rheumatoid arthritis. Researchers chemically stimulated the bronchial passages of 32 patients with SSc alone, 57 with SS alone, 24 with both SS and SSc, and 61 healthy volunteers. The chemical stimulation produced bronchial spasms in 50% of the patients with both SS and SSc, 42.2% of patients with SS alone, 25% of patients with SSc alone, and 6.5% of the healthy volunteers. Patients with both the limited variation of SSc and SS experienced more bronchial spasms than patients with a more system-wide variation of SSc.

The development of lung fibrosis in systemic sclerosis (SSc) or bronchial hyper-responsiveness in primary Sjogren's syndrome (pSS) may not be caused by phenotypical activation of alveolar helper/inducers or by suppressor/cytotoxic T lymphocytes and NK (natural killer) cells. A study of 13 SSc patients and 11 women with pSS analyzed cell populations obtained through bronchoalveolar lavage (BAL) by using two-color flow cytometric (FACS) analysis. FACS analysis revealed that patients with SSc had three times as many CD14 molecules on alveolar macrophages. Macrophage activation in the lungs of SSc patients may contribute to respiratory insufficiency by destroying endothelial tissue and promoting lung fibrosis. Women with pSS may not experience pulmonary fibrosis, but they may develop bronchial hyper-responsiveness and disease of the small airways.

Mothers who deliver children with congenital heart block are likely to develop a rheumatic disease even if they have no symptoms during pregnancy. The appearance of congenital heart block in infants has been associated with mothers who have autoimmune diseases. A group of 57 women, 52 of whom had already delivered children with congenital heart block and 5 pregnant with children with the abnormality, were followed for an average of 5 years to determine the long-term effects on both mother and child. Twenty-three of these women showed no symptoms of a rheumatic disease during pregnancy, but others had systemic lupus erythematosus, Sjogren syndrome or an undifferentiated autoimmune syndrome. Eleven women developed symptoms in the first few years following the birth of their children. None of the mothers who were diagnosed with an undifferentiated autoimmune syndrome during or before pregnancy developed new manifestations of the disease during a median follow-up of 4.8 years. The mortality rate for the 55 children with congenital heart block was 31% (17), with most surviving children requiring pacemakers. The risk of a subsequent child being born with congenital heart block appears low.

A 59-year-old woman with Sjogren's syndrome had an anterior mediastinal tumor. The tumor had epithelium-lined thymic cysts. Histologically, centrocyte-like (CCL) cells were present as clusters intermingling with small lymphocytes and plasma cells, invaded the epithelium, and formed characteristic lymphoepithelial lesions; the tumor was identified as malignant lymphoma arising in mucosa-associated lymphoid tissue (MALT). Within the tumor, trapped Hassall's corpuscles were recognized. Immunohistochemical staining demonstrated monotypic cytoplasmic kappa light chains in a small portion of the CCL cells. Furthermore, Southern blot hybridization studies showed rearrangements of immunoglobulin heavy chain, immunoglobulin kappa light chain, and T-cell receptor beta genes. The findings are consistent with thymic low-grade B-cell MALT lymphoma. Cancer 1992; 69:1347-1355.

In autoimmune disorders the immune system does not recognize certain cells of the body as part of itself and inappropriately responds to them as foreign cells. Sjogren's syndrome is an autoimmune disorder that is characterized by chronic inflammation with infiltration of sweat, salivary, and similar glands by lymphocytes (immune cells important in fighting infection). Other organ systems, especially the lungs, may be also be involved. Recurrent infections of the upper and lower respiratory tract, ranging from colds to pneumonia, are a major cause of illness in patients with this disease. Changes in levels of granulocytes (white blood cells and immune cells containing granular packets) may underlie the increased tendency toward infection. To better understand this possibility, the functions of neutrophils (white blood cells that engulf and kill invading organisms) obtained from 23 patients with Sjogren's syndrome were evaluated and compared with neutrophils from 35 healthy control subjects. Neutrophil levels were similar in both groups. The most important neutrophil activities were similar in both groups, but other activities including adherence (the ability to attach to blood vessel walls) and opsonic activity (aids engulfment of bacteria) were reduced, especially in patients with greater propensities to bacterial infection. The report suggests that changes in neutrophils, especially decreased adherence, may be partly responsible for the greater frequency of bacterial infections in patients with Sjogren's syndrome. (Consumer Summary produced by Reliance Medical Information, Inc.)

Sjogren's syndrome (SS) is a condition that affects the salivary glands and lacrimal glands (glands that make tears), reduces the production of saliva and tears, and causes mucous membranes to become dry. It is seen in postmenopausal women, and is often associated with rheumatoid arthritis, dental caries and Raynaud's phenomenon (a condition that affects blood vessels in the hands and feet). Many people who develop SS also develop some form of gastritis (inflammation of the mucous membranes that line the stomach). Previous studies have indicated that chronic atrophic gastritis (CAG) occurs in more than 65 percent of patients with SS. It is believed that the incidence of CAG increases with age. To investigate the relationship between SS and CAG, 104 subjects without SS (controls) and 44 female patients with SS were examined. Thirty-eight of the patients had abdominal pain and six had either weight loss, loss of appetite or anemia. Twenty-one of the patients were taking anti-inflammatory drugs or corticosteroids. Biopsy samples were taken from three different regions of the stomach (the antrum, the corpus, and the region between the antrum and corpus) and examined for the presence of CAG. The incidence of CAG was substantially higher in patients with SS than in those who did not have SS. Fifty percent of the patients with SS had CAG in the antrum and in the region between the antrum and corpus, while 40 percent had CAG in the corpus region of the stomach. Patients with SS and CAG had less acid in their stomachs than subjects who did not have SS and CAG (several types of atrophic gastritis are associated with reduced stomach acid secretion). It is concluded that CAG is more common in patients with SS than in the general population, and that its occurrence varies with age. (Consumer Summary produced by Reliance Medical Information, Inc.)

Sjogren's syndrome is one of the autoimmune inflammatory diseases, in which the body produces antibodies against its own tissues, and it chiefly affects exocrine glands such as sweat and mucus glands. The disease may occur alone, as primary Sjogren's syndrome, or as secondary Sjogren's syndrome in association with other autoimmune diseases. Primary Sjogren's syndrome may involve the lungs or other organs, and lung problems may be varied and include airway obstruction. Many patients with the disorder may have symptoms similar to those of bronchial hyperreactivity (BHR), whether related to poor glandular secretion of mucus or to actual lung involvement. Bronchial hyperreactivity, a hyperreactive constriction of the airway, may occur in affected people following exposure to smoke, cold, and exercise, as well as particular compounds such as histamine. Methacholine is a drug similar to the hormone which normally causes bronchial constriction and fluid secretion, and lung hyperresponsiveness to methacholine has been used as a test for BHR. The effect of methacholine in 21 patients (20 female) with primary Sjogren's syndrome, aged 22 to 78 years, was evaluated. Of 20 patients tested, 12 had BHR, one severely and five moderately. Of 21 healthy subjects also tested, only two had mild BHR. Nine of the 21 patients had dry coughs and a sensation of foreign bodies in the windpipe. Six had difficulty breathing on exertion, and two had asthma, while two had pleuritis (inflammation of the membrane between the lungs and rib cage) and five frequently had respiratory infections. Evaluation of lung function indicated that 16 patients had breathing problems which were varied in terms of severity. This study indicates that BHR correlates well with lung symptoms, and is more often found in patients with problems in the smaller airways, where oxygen is moved into the bloodstream. BHR may be related to mucus dryness. BHR testing may uncover patients with Sjogren's syndrome at risk for disease of small airways. The study suggests that Sjogren's syndrome should be suspected in patients with a chronic cough and other lung symptoms but whose lab tests are not compatible with a diagnosis of asthma. (Consumer Summary produced by Reliance Medical Information, Inc.)

A 48-year-old man with a history of Sjogren syndrome was admitted to a hospital when he developed a sudden swelling of his left leg. Deep vein thrombosis was suspected and he was given an anticoagulant. This condition is characterized by the formation of blood clots in the legs. He developed various medical conditions while hospitalized, was treated successfully, and discharged. Three months later, he was hospitalized again for swelling of his left leg. A mass was also felt in his abdomen. A biopsy of the mass revealed that he had lymphoma, which was the cause of his swollen leg.

Routine dental examinations and dental hygiene may be important to the overall health of patients with Sjogren's syndrome associated with rheumatoid arthritis (RA). An elderly patient with Sjogren's syndrome and RA developed a large abscess in the left thigh. Laboratory tests on the pus removed from the abscess indicated an infection by an organism typically found in the mouth called Peptostreptococcus. An examination and X-rays of the patient's mouth revealed gum abscesses and cavities in all five teeth.

Sjogren's syndrome is a disorder characterized by rheumatoid arthritis (inflammatory joint disease), xerostomia (dryness of the mouth), and keratoconjunctivitis sicca (congestion and dryness of the conjunctiva, the membrane lining the eye and lids). This syndrome usually develops in postmenopausal women and is thought to be a disease of collagen, a structural fiber-like protein. Patients with Sjogren's syndrome may develop myositis, inflammation of the muscle tissue. A case is described of a 65-year-old woman with Sjogren's syndrome and myositis, who responded to treatment with the drug cyclophosphamide, which was given intravenously in pulse doses. The patient developed inflammation, pain, and weakness of the muscle three years after the onset of symptoms of keratoconjunctivitis sicca. Microscopic tissue examination of the muscle revealed several abnormalities, including muscle tissue death, ingestion of muscle fibers by phagocytic cells, and some degree of muscle regeneration. Treatment with methylprednisolone and cyclophosphamide resulted in reduction of symptoms, as indicated by clinical, biochemical, and tissue tests. (Consumer Summary produced by Reliance Medical Information, Inc.)

Sjogren's syndrome is a chronic systemic inflammatory disorder characterized by dryness of mouth, eyes, and other membranes, and by joint inflammation. Some features associated with other autoimmune disorders, in which the immune system attacks the body's own tissues, may also occur. A case is described of a 37-year-old woman who had membrane dryness and low blood levels of autoantibodies, but no joint disease. Two years later, she developed a mild polyarthritis (inflammation involving multiple joints), and blood levels of autoantibodies were higher. Later that year, the arthritis became active, and several different drugs were tried, some ineffective and others causing side effects. Sulphasalazine was started about one year later, since arthritis was still active. Three weeks after starting this therapy, the woman was admitted to the emergency room with a three-day history of headache, nausea, and vomiting and with severe knee and leg pain. The patient had mild neck stiffness, aversion to light, moderate lymph node swelling, and a rash on the trunk and adjacent extremities. Straight leg raising was restricted and painful, a sign of meningitis. Antibiotics were started, with rapid improvement, and sulphasalazine was discontinued. No organism causing the meningitis was identified. Two weeks after discharge, she seemed well, although tired. As joints were painful, sulphasalazine was restarted, and she experienced fever, severe pain, headache, and rash within eight hours. Straight leg raising was painful, and the neck was slightly rigid. Response to antibiotics and painkillers was slow. After review, it was suggested that both episodes of meningitis were caused by a hypersensitivity reaction to sulphasalazine rather than an organism. She improved markedly when steroids were begun and antibiotics were discontinued. She remained well three months after discharge and was taking steroids for the arthritis. The report suggests that as the frequency of sulphasalazine use increases, the possibility of occurrence of meningitis or related nerve toxicity should be considered. (Consumer Summary produced by Reliance Medical Information, Inc.)

A 32-year-old women presented with elevated, purpuric macules on her legs, dryness of the eyes and mouth, and vaginal dryness. Her leg vasculitis did not respond to compression therapy, and [...]

Primary Sjogren's syndrome is an autoimmune disorder; these disorders occur when the immune system attacks natural body tissues and substances. The syndrome affects women after menopause and is characterized by xerostomia (dry mouth), rheumatoid arthritis, and dry eyes with possible loss of vision. Research has recently found that Sjogren's syndrome can also have adverse effects on the central nervous system and the blood vessels. A case is described of a 48-year-old woman with Sjogren's syndrome who developed auricular chondritis, or inflammation of the cartilage of the ears. Microscopic examination showed characteristics of relapsing polychondritis, which is the inflammation of cartilage tissue at several sites including the ears, nose, joints, bronchi, and trachea. The patient also had diffuse proliferative glomerulonephritis or inflammation of the kidney, resulting in rapid deterioration of kidney function. She was treated with the corticosteroid prednisone, and at one-year follow-up, showed improvement in kidney function and no inflammation or deformity of the ear. This case is unusual because of the simultaneous occurrence of both ear chondritis and diffuse proliferative glomerulonephritis in a patient with Sjogren's syndrome. It demonstrates that Sjogren's syndrome can affect many body systems, including some that were not previously thought to be vulnerable to this disease. (Consumer Summary produced by Reliance Medical Information, Inc.)