Auto-immune hepatitis is a chronic necroinflammatory liver disorder that is characterized by hypergammaglobulinemia, auto-antibodies in serum, and, on histological examination, the presence of periportal hepatitis. Although it can be associated with a number of other auto-immune diseases, Sjogren's syndrome is rarely associated with auto-immune hepatitis. We herein report an unusual case of auto-immune hepatitis associated with primary Sjogren's syndrome. A 39-year-old woman was admitted to our hospital due to jaundice. Laboratory data showed negative viral hepatitis marker, increased serum IgG level, positive anti- nuclear antibody, and an increased rheumatoid factor titer. The patient had no history of taking medications and alcohol. Based on characteristic clinical features, liver biopsy findings, positive Schirmer's test, and salivary scintigraph, she was diagnosed as having auto-immune hepatitis and Sjogren's syndrome. The patient achieved complete remission with steroid monotherapy.
Adult ; Hepatitis, Autoimmune/*complications/diagnosis ; Humans ; Sjogren's Syndrome/*complications/diagnosis

Humans ; Sjogren's Syndrome/diagnosis* ; Castleman Disease/diagnosis* ; Case-Control Studies

Sjogren's syndrome is a chronic autoimmune disorder characterized by lymphocytic infiltration of the lacrimal and salivary glands, leading to dryness of eyes (kerato-conjunctivitis sicca) and mouth (xerostomia). The skin lesions in Sjogren's syndrome are usually manifested as xeroderma, but sometimes appear as annular erythema or vasculitis. Central nervous system symptoms may be presented as one of extraglandular manifestations, though rare in incidence, and need differential diagnosis from multiple sclerosis. We report a case of a 45-year-old woman diagnosed as multiple sclerosis at first but later as neurologic manifestation of primary Sjogren's syndrome, showing signs of multiple sclerosis and cutaneous erythematous lesions.
Case Report ; Diagnosis, Differential ; Erythema/pathology ; Erythema/diagnosis* ; Female ; Human ; Middle Age ; Multiple Sclerosis/pathology ; Multiple Sclerosis/diagnosis* ; Sjogren's Syndrome/pathology ; Sjogren's Syndrome/diagnosis*

Background: Gougerot-Sj\xf6gren syndrome is an autoimmune disorder with two remarkable symptoms such as dry eyes and dry mouth. Objective: To study the symptoms of dry mouth of Gougerot-Sj\xf6gren syndrome after rheumatoid arthritis; To evaluate clinical and subclinical manifestations of dry mouth. Subjects and method: A prospective, descriptive, cross-sectional study included 160 patients with rheumatoid arthritis, who treated at Department of Rheumatology of Bach Mai hospital, from 1998 to 2003. The patients were divided into 2 groups: 60 patients with Gougerot-Sj\xf6gren syndrome and 100 patients without Gougerot-Sj\xf6gren syndrome. Results: The average age of patients with Gougerot-Sj\xf6gren syndrome was 54.55 \xb1 10.91 years. The mean time of having dry mouth was 8.65 \xb1 8.39 months. Clinical manifestations of dry mouth were sensation of dry mouth (90%), lost of saliva (80%), drink a lot of water while eating (58.3%), enlargement of parotid glands (15%). Degrees of dry mouth were mild and moderate (36.7%), severe (53.3%). 93.3% of patients decreased total salivary flow.82% of patients had 3 and 4 anatomopathologic stages according to Chilsom classification. Conclusion: Incidence of symptoms of dry mouth was higher significantly in the rheumatoid arthritis patients with Gougerot-Sj\xf6gren syndrome than those without Gougerot-Sj\xf6gren syndrome.
Arthritis ; Rheumatoid/ pathology ; diagnosis ; Sjogren's Syndrome/ pathology ; diagnosis ; Xerostomia/ pathology ; diagnosis

Acquired renal tubular disorder can be observed in various disease processes, especially autoimmune diseases. Gitelman syndrome is an autosomal recessive disease characterized by hypokalemic metabolic alkalosis, hypomagnesemia, and hypocalciuria. This disorder is caused by mutation in the SLC12A3 gene, which encodes the thiazide - ensitive NaCl cotransporter (NCCT). Acquired Gitelman syndrome has been reported and the majority has been associated with Sjogren's syndrome. The presence of circulating auto - antibodies to NCCT was suggested as a mechanism of acquired Gitelman syndrome. Treatment of acquired Gitelman syndrome was done with supplements of potassium and magnesium and prednisone was effective in some cases. Acquired Gitelman syndrome should be included in the differential diagnosis of renal involvement in patients with autoimmune diseases, especially Sjogren's syndrome.
Alkalosis ; Antibodies ; Autoimmune Diseases ; Diagnosis, Differential ; Gitelman Syndrome ; Humans ; Magnesium ; Potassium ; Prednisone ; Sjogren's Syndrome

No abstract available.
Adult ; Aged ; *Edema ; Female ; Human ; Male ; Sjogren's Syndrome/*diagnosis/physiopathology ; Synovitis/*diagnosis/physiopathology

Background: The minor salivary gland biopsy is an important diagnostic criterion of Gougerot-Sj\xf6gren syndrome. Objective: To describe histopathological characteristics of minor salivary gland of Gougerot-Sj\xf6gren syndrome combined to rheumatoid arthritis. Subjects and method: A prospective, descriptive, cross-sectional study included 108 patients with rheumatoid arthritis, who treated at Department of Rheumatology of Bach Mai hospital, from 1998 to 2003. The patients were divided into 2 groups: 50 patients with Gougerot-Sj\xf6gren syndrome and 58 patients without Gougerot-Sj\xf6gren syndrome. Results: Characteristics of minor salivary gland biopsy were lymphocytic infiltration of minor salivary glands (96%) with various distribution: periductal (86%), periacinic (72%), perivascular (36%). 78% of cases were plasmocytic infiltration, with periductal distribution (70%). 82% of cases were 3, 4 stages according to Chilsom clsssification. 67.5% of cases had ductal abnormality, glandular atrophy (62%), vascular edema (58%). Conclusion: Incidence of 3, 4 stages according to Chilsom classification was 82% in rheumatoid arthritis patients with Gougerot-Sj\xf6gren syndrome.
Arthritis ; Rheumatoid/ pathology ; diagnosis ; Sjogren's Syndrome/ pathology ; diagnosis ; Salivary Glands ; Minor/ pathology

Sjogren's syndrome is a chronic autoimmune exocrinopathy that destroys salivary and lacrimal gland tissue. We report an unusual case of this disease in a 54-year-old woman who presented with multiple and bilateral parotid cystic masses. The multiple, small, bead-like cysts were clearly evident in the computed tomography sections in this patient, a visible reminder that this may be the initial presentation in a patient with Sjogren's syndrome. As the case illustrates, Sjogren's syndrome should be included in the differential diagnosis of multiple and bilateral cystic parotid lesions.
Diagnosis, Differential ; Female ; Humans ; Lacrimal Apparatus ; Middle Aged ; Parotid Gland ; Rare Diseases ; Sjogren's Syndrome*

Sjogren's syndrome is a chronic autoimmune exocrinopathy that destroys salivary and lacrimal gland tissue. We report an unusual case of this disease in a 54-year-old woman who presented with multiple and bilateral parotid cystic masses. The multiple, small, bead-like cysts were clearly evident in the computed tomography sections in this patient, a visible reminder that this may be the initial presentation in a patient with Sjogren's syndrome. As the case illustrates, Sjogren's syndrome should be included in the differential diagnosis of multiple and bilateral cystic parotid lesions.
Diagnosis, Differential ; Female ; Humans ; Lacrimal Apparatus ; Middle Aged ; Parotid Gland ; Rare Diseases ; Sjogren's Syndrome*

OBJECTIVE: The purpose of this study is to evaluate the diagnostic performance of minor salivary gland biopsy (MSGB) for patients with primary Sjogren's syndrome (pSS). METHODS: We have conducted a search from Medline, Embase, and Cochrane Library databases, and performed a meta-analysis on the diagnostic accuracy of MSGB in pSS patients. RESULTS: A total of eight studies, including 583 pSS and 627 non-pSS patients, were available for the meta-analysis. The pooled sensitivity and specificity of MSGB were 75.7% (95% confidence interval [CI], 72.0~79.1) and 90.7% (88.1~92.9), respectively. The positive likelihood ratio, negative likelihood ratio, and diagnostic odds ratio were 9.475 (4.051~22.16), 0.266 (0.208~0.340), and 38.92 (19.12~72.21), respectively. The area under the curve was 0.901 and the Q*; index was 0.902, indicating a high diagnostic accuracy. Some between-study heterogeneity was found in the meta-analyses; however, there was no evidence of a threshold effect (Spearman correlation coefficient= 0.419; p=0.301). Meta-regression showed that the study quality, sample size, study design, and diagnostic criteria were not sources of heterogeneity, and subgroup meta-analyses did not change the overall diagnostic accuracy. CONCLUSION: Our meta-analysis of published studies demonstrates that MSGB has a high diagnostic accuracy and may play an important role in the diagnosis of pSS.
Biopsy* ; Diagnosis ; Humans ; Odds Ratio ; Population Characteristics ; Salivary Glands, Minor* ; Sample Size ; Sensitivity and Specificity ; Sjogren's Syndrome*