A case of Sj?gren's syndrome with valvular diseases

The case of a 61 year old woman with Sj?gren's syndrome with aortic and mitral stenosis is reported. She suffered from rheumatic fever at a young age. Physical and echocardiographic examinations showed findings of mitral and aortic valve stenosis. In addition, she had experienced xerostomia, a gritty sensation in the eyes and Raynaud's phenomenon. Blood examination showed hypergammaglobulinemia, positive rheumatoid factor, antinuclear and anti-Ro (SS-A) antibodies. The diagnosis of Sj?gren's syndrome was confirmed by sialography and biopsy of the labial salivary gland. The combination of valvular disease and Sj?gren's syndrome is rare and the etiological correlation is discussed.     

Is the heart affected in primary Sjögren's syndrome? An echocardiographic study

OBJECTIVE: To evaluate whether patients with primary Sj?gren's syndrome without overt cardiac disease have echocardiographic abnormalities and their relation with clinical and laboratory data. METHODS: One hundred and seven consecutive patients with primary Sj?gren's syndrome and 112 healthy controls, matched for age and gender, underwent complete echocardiographic study. RESULTS: Thirty-two patients had mitral valve regurgitation (p<0.001) whereas tricuspid and aortic valve regurgitation were, also, more frequent in the patient group (p=0.022 and p=0.007 respectively). In multivariate analyses, low C4 levels of complement and age were strong predictors of mitral valve regurgitation whereas age was predictor of aortic valve regurgitation. Tricuspid valve regurgitation was associated with pulmonary hypertension. Clinically silent pericardial effusion, found in 9 patients (p=0.008), was associated with cryoglobulinemia and primary biliary cirrhosis. Twenty-four patients had pulmonary hypertension (p<0.001) whereas hypocomplementemia and cryoglobulinemia were strong predictors of pulmonary artery systolic pressure. The analyses reveal that easy fatigability was associated with pulmonary hypertension and low C4 levels. The patients' left ventricular mass index differed significantly from the controls (108.9+/-17.21 gm(-2) vs. 85.8+/-6.73 gm(-2), p<0.001) and was associated with palpaple purpura and anti-Ro/SSA. From the diastolic function indices only the left ventricular isovolumic relaxation time differed significantly among patients and controls. CONCLUSION: Valvular regurgitation, pericardial effusion, pulmonary hypertension and increased left ventricular mass index occur with disproportionately high frequency in patients with primary Sj?gren's syndrome and no clinically apparent heart disease. Thus echocardiographic studies may need to be performed in these patients especially when palpable purpura, antibody reactivity and low C4 levels are present.     

Cystic lung disease associated with Sjögren's syndrome: 2 cases

INTRODUCTION: Cystic lung disease is characterised on chest iconography by foci of decreased lung density with definable and thinned walls (wall thickness<4 mm) and with length's diameter superior at 1 cm. Cystic lung disease is exceptionally associated with the Sj?gren's syndrome; very few cases have been described. EXEGESIS: We report two cases of cystic lung disease associated with Sj?gren's syndrome, one occurring in a Lupus-Sj?gren's overlapping syndrome, and another revealing primary Sj?gren's syndrome. CONCLUSION: The Sj?gren's syndrome should be recognised as could be associated with Cystic lung disease; and latent Sj?gren's syndrome should be researched in presence of cystic lung lesions.     

Sj?gren's syndrome with multiple bullae and pulmonary nodular amyloidosis

We treated a patient with Sj?gren's syndrome associated with multiple bullae and pulmonary nodular amyloidosis, both of which were identified by open lung biopsy. The mechanism of bullae formation appeared to be narrowing of the airway, as a result of extensive inflammatory cell infiltration to the bronchiolar wall, which acted as the check valve mechanism. We believe this to be the first reported case of Sj?gren's syndrome accompanied by these two pulmonary manifestations.     

Recurrent prosthetic endocarditis in a 21-year old patient with renal transplantation treated with allogeneic homograft

Prosthetic infective endocarditis is a possible complication of implantation of a prosthetic cardiac valve. Without early and effective treatment, it can have fatal consequences. One treatment option is use of an allogeneic cryopreserved homograft. This case report presents a 21-year old patient after kidney transplantation due to hereditary nephrotic syndrome and aortic valve replacement with aortic conduits. After fever was noted in the patient, prosthetic infective endocarditis was diagnosed by echocardiography and also confirmed by CT-3D examination. The cryopreserved aortic homograft was implanted at the Department of Cardiac Surgery. This along with additional conservative management effectively treated the infection. Based on literature data and our own experience, we believe that the treatment of prosthetic endocarditis after aortic valve replacement with cryopreserved homograft can be a method of choice.     

The bicuspid aortic valve

The bicuspid aortic valve affects 1 to 2% of the population and may be complicated by aortic stenosis or aortic insufficiency and infective endocarditis. The bicuspid aortic valve is associated with abnormalities of the aortic wall such as coarctation of the aorta, aortic dissection, and aortic aneurysm. Most patients with a bicuspid aortic valve will develop some complication during life. Individuals with a bicuspid valve may be unaware of its presence and are at risk for unsuspected complications. Aortic wall abnormalities associated with bicuspid aortic valve are due to cystic medial necrosis. This process is associated with increased metalloproteinase activity and apoptosis of vascular smooth muscle cells. The clinical correlates of aortopathy in the bicuspid aortic valve include significant enlargement of the ascending aorta with aneurysm formation and dissection. This process continues after valve replacement. The person with bicuspid aortic valve requires continuous surveillance to treat associated lesions and prevent complications.     

Sj?gren's syndrome in patients with primary biliary cirrhosis

Symptomatology and objective findings of Sj?gren's syndrome were evaluated in 38 consecutive patients with primary biliary cirrhosis. Symptoms of Sj?gren's syndrome were present in 18 (47.4%) patients, but were severe enough to warrant therapy in only four (10.5%). Nineteen patients consented to evaluation for Sj?gren's syndrome, which included Schirmer's I test, measurement of parotid flow rate and serum autoantibodies, labial minor salivary gland biopsy and human leukocyte antigen typing. Histological changes diagnostic of Sj?gren's syndrome were present in five patients (26.3%). All five patients had symptoms of Sj?gren's syndrome and three had abnormal Schirmer's I tests, but none had corneal ulcerations or decreased parotid flow rates. Results of serological tests and human leukocyte antigen typing were not similar to those described in patients with primary Sj?gren's syndrome but were similar to those described in patients with rheumatoid arthritis and Sj?gren's syndrome. These findings indicate that Sj?gren's syndrome associated with primary biliary cirrhosis is a form of secondary Sj?gren's syndrome resembling that associated with rheumatoid arthritis.     

原发性胆汁性肝硬化与干燥综合征20例临床分析

目的：探讨原发性胆汁性肝硬化与干燥综合征的相关性。方法：对20例原发性胆汁性硬化伴或不伴发干燥综合征病例的临床特点,血清学检查,肝脏病理,治疗等进行回顾性分析。结果：原发性胆汁性肝硬化病人临床以黄疸,皮肤瘙痒,肝大,脾大为主要表现,抗线粒体抗体M2亚型为特征性抗体。合并口眼干燥表现的不少见,但不具备原发性干燥综合征的典型表现。治疗上缴素和免疫抑制剂疗效不佳,熊去氧胆碱酸有一定的疗效。结论：原发性胆汁性肝硬化合并的干燥综合征不同于原发性干燥综合征。     

The management of Sjögren's syndrome

Sj?gren's syndrome is a chronic autoimmune disorder, characterized by lymphocytic infiltration and malfunction of the exocrine glands, resulting in dry mouth and eyes. The syndrome can present either alone (primary Sj?gren's syndrome) or in the context of an underlying connective tissue disease (secondary Sj?gren's syndrome). Systemic features, resulting from cutaneous, respiratory, renal, hepatic, neurologic, and vascular involvement, often occur. Two types of primary Sj?gren's syndrome are currently recognized: a benign disease that affects quality of life, and a systemic syndrome associated with increased morbidity and mortality owing to a high risk of malignant transformation, and that requires close follow-up. Ocular involvement, manifested as keratoconjunctivitis sicca, is managed with local and systemic stimulators of tear secretion and supportive surgical procedures. Treatment of oral manifestations includes intense oral hygiene, prevention and treatment of oral infections, use of saliva substitutes, and local and systematic stimulation of salivary secretion. Cholinergic agents, such as pilocarpine and cevimeline, are helpful in patients with residual salivary function, and ciclosporin ocular drops seem to be of some benefit. Systemic immunosuppressives are reserved for treatment of severe extraglandular manifestations of Sj?gren's syndrome. Anti-B-cell therapy is a new potential therapy for the glandular and extraglandular manifestations, such as glomerulonephritis or vasculitis, in addition to the management of lymphoma associated with Sj?gren's syndrome. Induction of oral tolerance and gene-transfer modalities were recently attempted in animal models, with promising results.     

Primary Sj?gren's syndrome in men. Clinical, serologic, and immunogenetic features

Although primary Sj?gren's syndrome is a common rheumatic disorder in women, it is not well recognized in men. This study represents the first report of the clinical, serologic, and immunogenetic features of a group of 36 men with primary Sj?gren's syndrome, which are contrasted with those of a group of 69 women with primary Sj?gren's syndrome. The majority of male patients had extraglandular involvement including articular (78 percent), neurologic (39 percent), inflammatory vascular (25 percent), and lymphoproliferative disorders (17 percent). Although men were at the same risk for the development of extraglandular complications, there were significant serologic and immunogenetic differences. In sharp contrast to women with Sj?gren's syndrome, men with Sj?gren's syndrome were seronegative with respect to the presence of serum rheumatoid factor (p = 0.008) and antibodies to Ro(SS-A) (p = 0.016). The supertypic specificity, MT2 (DRw52), as in women, was strongly associated with primary Sj?gren's syndrome in men when compared with race-matched control subjects (p = 0.0015). In men, however, the frequency of HLA-B8 and HLA-DR3, the most common DR locus specificity observed in women, was not statistically different from that observed in the normal control group.     

Spondylodiscitis and endocarditis caused by S. vestibularis

Streptococcus vestibularis is a recently described member of the viridans group that was first isolated from the vestibular mucosa of the human oral cavity and described as a new species in 1988. It has been rarely associated with human infections. In few papers, it has been reported as a causal agent of systemic infection in immunosupressed adults and in those with other severe underlying diseases, like coronary valve diseases. A 65-year-old woman was admitted to the hospital with complaints of fever for three months, general malaise, effort dyspnea, weight loss, back pain and myalgia. Both native aortic valve endocarditis and spondylodiscitis due to Streptococcus vestibularis were detected. The patient was successfully treated with intravenous potassium penicillin G and gentamicin for six weeks, followed by oral amoxicillin for three months, in addition to aortic valve replacement. In all patients with spondylodiscitis, infective endocarditis should be considered, particularly in patients with heart valve disease history, since spondylodiscitis may be the presenting sign of an infective endocarditis. Cardiac valve replacement surgery should be performed if the course of fever and inflammatory syndrome is unfavorable after appropriate antibiotic treatment. We report the first case with both native aortic valve endocarditis and spondylodiscitis due to Streptococcus vestibularis.     

Increased bronchial responsiveness in primary and secondary Sj?gren's syndrome

We examined one group of 33 patients with primary Sj?gren's syndrome, one group of 17 patients with secondary Sj?gren's syndrome, i.e. associated with other connective tissue diseases, and one group of 14 patients with connective tissue diseases but without Sj?gren's syndrome. In each patient we obtained chest radiographs and measured lung volumes, carbon monoxide diffusing capacity and airway responsiveness to methacholine. We observed no difference in chest radiograph abnormalities, in lung volumes and in carbon monoxide diffusing capacity among the three groups. However, we found a slight but significant increase of bronchial responsiveness in patients with primary and secondary Sj?gren's syndrome compared with patients with connective tissue disorders but without Sj?gren's syndrome. Thus PD20FEV1 methacholine was 1.07 mg (1.2) (geometric mean and GSEM) in primary Sj?gren's syndrome, 0.91 mg (1.4) in secondary Sj?gren's syndrome (NS), and 2.24 mg (1.09) in patients with connective tissue diseases but without Sj?gren's syndrome (t = 2.59 and t = 2.8, both p less than 0.05, vs primary and secondary Sj?gren's syndrome, respectively). These results show that some patients with Sj?gren's syndrome have mild bronchial hyperresponsiveness, which may be related to the specific airway abnormalities of this disease.     

Autoimmune hemolytic anemia in an elderly patient with primary Sjögren's syndrome

Primary Sj?gren's syndrome is an autoimmune disease characterized by lymphocytic infiltration of the salivary glands and lacrimal glands. The histological features of chronic inflammation in primary Sj?gren's syndrome may be associated with B cell hyper-reactivity. This syndrome also has various manifestations associated with other exocrine glands and nonglandular tissues. The hematological abnormalities usually seen in Sj?gren's syndrome are lymphopenia, leucopenia, and thrombocytopenia. Although the direct Coomb's test is often positive, the occurrence of autoimmune hemolytic anemia (AIHA) is rare. Here, we report an elderly patient with primary Sj?gren's syndrome who developed AIHA during the clinical course.     

Intractable infective endocarditis associated with supraaortic stenosis in Williams syndrome: a case report

A 17-year-old man with supravalvular aortic stenosis associated with Williams syndrome was admitted to our hospital for intensive treatment for intractable infective endocarditis. The patient had a history of percutaneous balloon valvuloplasty for aortic stenosis in 1992. He was well until late in 1999, when he had a high temperature after dental work-up. The diagnosis was infective endocarditis but antibiotic therapy was not effective. He was transferred to our clinic. Transthoracic echocardiography demonstrated bicuspid aortic valve, supraaortic stenosis, mitral valve prolapse with severe regurgitation and scattered vegetations on the anterior mitral and aortic valves. In addition, transesophageal echocardiography showed innumerable mobile vegetations located from Valsalva's sinus to the descending aorta. Aortic root and arch replacement with a homograft and mitral valve replacement with an artificial valve were successfully performed to eliminate the infective endocarditis. In the present patient, the flow jet across the supraaortic stenosis seemed to cause a predisposition to severe endocarditis.     

Congenital disorders of the cardiovascular system and their complications in a 21-year-old woman with Turner syndrome--a case report

A 21-year-old woman with Turner syndrome was admitted to hospital because of progressive heart failure. Echocardiography revealed a bicuspid aortic valve with one cusp ruptured due to infective endocarditis and anomalous pulmonary venous drainage. The patient underwent successful aortic valve replacement.     

Budd-Chiari Syndrome in a Patient with Sjögren''s Syndrome

A case of Budd-Chiari syndrome associated with obstruction of the inferior vena cava is reported. The case was complicated by Sj?gren's syndrome and chronic thyroiditis. A marked prolongation of activated partial thromboplastin time, due to circulating anticoagulant, was noted. Interrelation of thrombosis of the inferior vena cava, circulating anticoagulant, and Sj?gren's syndrome is suggested.     

Relationship of Sjögren's syndrome to other connective tissue and autoimmune disorders

Sj?gren's syndrome is a systemic autoimmune disease that frequently presents concomitantly with other systemic connective tissue or organ-specific autoimmune diseases. This association is well described for systemic lupus erythematosus and rheumatoid arthritis. The presence of Sj?gren's syndrome influences the expression of the other autoimmune disease to some degree, for instance by increasing fatigue and lymphoma risk. The etiopathogenic mechanism for the simultaneous or sequential development of multiple autoimmune diseases in one individual is not well understood. Common genetic backgrounds and additional immunogenetic, environmental, or hormonal factors may be responsible for the formation of subsets of autoimmune disease clustering. While the most currently accepted classification criteria (American European Consensus Criteria) designate these cases as secondary Sj?grens syndrome, the terms overlapping or associated Sj?gren's syndrome are frequently used in the literature to describe these cases.     

Osteomalacia revealing Sjögren's syndrome: a case report

INTRODUCTION: The most common renal disease in Sj?gren's syndrome is tubulo-interstitial nephritis, responsible for tubular acidosis in around 20 % of patients. Osteomalacia exceptionally occurs as the first manifestation of a renal tubule disorder due to a Sj?gren's syndrome. EXEGESIS: We report a case of a 20-year-old woman with tubular acidosis induced osteomalacia secondary to primary Sj?gren's syndrome. Improvement was obtained with bicarbonates, vitamin D, calcium and high-dose steroid therapy. CONCLUSION: During Sj?gren's syndrome, osteomalacia can complicate the distal renal tubular acidosis. In spite of the rare cases of osteomalacia revealing Sj?gren's syndrome, this auto-immune disease must appear in the list of the aetiologies of osteomalacia.     

Right-to-left shunting through a patent foramen ovale caused by pulmonary hypertension associated with rheumatoid arthritis and Sj?gren's syndrome: a case report

This case report presents a fifty-four-year-old woman with right-to-left shunt in the atrium. It seemed clinically at first that the shunt was due to atrial septal defect. However, she also had pulmonary disease associated with rheumatoid arthritis and Sj?gren's syndrome. At autopsy atrial septal defect was not evident, but a patent foramen ovale and pulmonary artery disease were observed. This case suggests that pulmonary hypertension secondary to rheumatoid arthritis and Sj?gren's syndrome could lead to right-to-left shunting through a patent foramen ovale.     

Update on Sjögren's syndrome autoimmune epithelitis: from classification to increased neoplasias

Sj?gren's syndrome is a chronic inflammatory process that primarily involves the exocrine glands. Its clinical manifestations range from autoimmune exocrinopathy to extraglandular (systemic) involvement affecting the lungs, kidneys, blood vessels, and muscles; it can occur alone (primary Sj?gren's syndrome) or in association with other autoimmune diseases (secondary Sj?gren's syndrome). In recent years, clinical and laboratory observations have highlighted the central role of the epithelial cell and it has been suggested that the etiological name of the disease should be 'autoimmune epithelitis'. The extraglandular manifestations of the disease are divided in two groups: (1) lung, kidney (interstitial nephritis), and liver involvement as a result of lymphocytic invasion in epithelial tissues; and (2) skin vasculitis, peripheral neuropathy, and glomerulonephritis, with low C4 levels, which is the result of immune complex disease, are associated with increased morbidity and high risk for lymphoma. The diagnosis of the disease is based on the classification criteria, raised by the American-European Study Group and which have been built on the European preliminary classification criteria, developed in 1992. The association of Sj?gren's syndrome with lymphoma is well documented as in approximately 5% of patients the benign autoimmune process is transformed into a lymphoid malignancy. The salivary extranodal marginal zone B-cell lymphomas of the mucosa-associated lymphoid tissue type are the most common lymphoma in Sj?gren's syndrome. These tumors are antigen-stimulated B-cell lymphomas and are characterized by localized stage, indolent clinical course, and recurrence in other extranodal sites. Among the clinical and serological parameters that have been associated with lymphoma development in patients with Sj?gren's syndrome, the presence of palpable purpura, low C4 and mixed monoclonal cryoglobulinemia constitute the main predictive markers; patients displaying these risk factors should be monitored closely. The purpose of this review is to discuss the clinical picture, the diagnostic procedure, and the malignant lymphoproliferation in the disease.