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

Rheumatic diseases are associated various extra-articular manifestations, such as eye, nerve, pericardium, and pleura. The anterior part of the eye has distinguished anatomic structure resembling synovial joints, thus it is a common site of ocular manifestation in rheumatic disease. These changes include sicca syndrome, peripheral ulcerative keratitis, episcleritis, scleritis, and iritis. Some of these findings may be the clues for the diagnosis of the rheumatic diseases, and some ocular manifestations may represent the activity or prognosis of the rheumatic diseases. These ocular complications may leads to severe visual loss. It is crucial to rule out underlying systemic diagnosis in rheumatic disease associated ocular diseases, and when diagnosed, the coordination of the rheumatologist with the ophthalmologist in the treatment is imperative.
Corneal Ulcer ; Diagnosis ; Eye Diseases* ; Iritis ; Joints ; Pericardium ; Pleura ; Prognosis ; Rheumatic Diseases* ; Scleritis ; Sjogren's Syndrome

Sjogren's syndrome is an autoimmune disease that is characterized by keratoconjunctivitis sicca and xerostomia. Small-fiber neuropathy is rare, although neurological manifestations occur in as many as 30% of patients with Sjogren's syndrome. We report herein a patient with Sjogren's syndrome who presented with autonomic neuropathy and painful sensory neuropathy. Primary Sjogren's syndrome should be considered in the differential diagnosis of small-fiber neuropathy.
Autoimmune Diseases ; Diagnosis, Differential ; Humans ; Keratoconjunctivitis Sicca ; Neurologic Manifestations ; Sjogren's Syndrome ; Xerostomia

BACKGROUNDPrimary Sjögren's syndrome (pSS) is one of the autoimmune diseases with high incidence. There were several clinical investigations in Caucasian but seldom in Chinese. The aim of this study was to compare the difference of clinical manifestations, immunological features and prognosis of pSS between Caucasian and Chinese pSS patients.

METHODSFive hundred and seventy-three patients who fulfilled the 2002 international classification (criteria) for pSS from Peking Union Medical College Hospital between 1985 and 2006 were screened retrospectively and compared with other populations.

RESULTS(1) The study consisted of 524 (91%) female and 49 (9%) male patients (female: male = 10.7:1). Mean age at the onset of the disease was (39.0 ± 13.7) years and in 169 (29.5%) patients the disease onset occurred before the age of 30 years. The average duration from disease onset to pSS diagnosis was 48 months (range, 1 - 552 months). It had been shortened during the recent five years. (2) Dry mouth (84.5%) and dry eyes (70.0%) were the most common symptoms, significantly lower than foreign patients (P = 0.000). Two hundred and seventy-two (47.5%) patients presented with rampant caries, 160 (27.9%) with parotidomegaly. The positivity of xerostomia, xerophthalmia and salivary gland biopsy were 91.9%, 94.8% and 90.7%, respectively. (3) Systemic involvement occurred in 91.4% patients. Compared with studies done outside China, higher prevalence of fever 41.0%, myositis 4.9%, pericardial effusion 14.8%, pulmonary involvement 42.3%, renal involvement 33.5%, thyroid involvement 32.7%, pancrease involvement 5.6% (P < 0.01) and lower prevalence of fatigue, lymphadenectasis and Raynaud's phenomenon (P < 0.01) were seen. (4) Risk factors of death include pulmonary artery hypertension, liver damage and interstitial lung disease.

CONCLUSIONSChinese pSS differs significantly from the non-Chinese cases in terms of the age of onset, systemic involvement, autoantibodies and proportional mortality rate. Lung and liver damage were found to be the highest risk factors of the disease prognosis.