Sjögren's syndrome is a chronic autoimmune disease with unclear etiology. From the point of etiology, Chinese medicine (CM) theory holds that pathological products like dry toxin, blood stasis are produced in the pathological process. They are both pathologic results and pathogenic factors for its further development. So pathological products are also named as second pathogenic factors. In this article, the concept of second pathogenic factors was sorted and defined. Main second pathogenic factors of Sjögren's syndrome were pinpointed, and their modern medical bases were analyzed. Authors came to a conclusion that clearing away second pathogenic factors is a key point in treating Sjögren's syndrome.
Humans ; Sjogren's Syndrome ; pathology

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

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 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*

Sjogren's syndrome (SS) is an autoimmune disorder in which lymphocytes infiltrate the exocrine glands, resulting in the development of sicca symptoms. Lymphocytes may also invade various other organs and cause diverse symptoms. Interstitial pneumonia has been observed frequently in SS patients. Typically, the pneumonia responds well to systemic steroids, and fatal cases are rare. We experienced a case of lymphocytic pneumonia accompanied by SS and treated with cyclophosphamide pulse therapy, and we present details of the case herein.
Adult ; Humans ; Lung/*pathology ; Lung Diseases, Interstitial/drug therapy/*pathology ; Lymphocytes/*pathology ; Male ; Plasma Cells/pathology ; Sjogren's Syndrome/*pathology

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.

Sjogren's syndrome (SS) is an autoimmune disease characterized by a lymphocytic infiltration of the salivary and lacrimal glands leading to a progressive destruction of these glands due to the production of autoantibodies. This disorder is either isolated (primary SS) or associated with other systemic diseases (secondary SS). The occurrence of B-cell non-Hodgkin's lymphoma (NHL) represents the major complication in the evolution of SS patients. The risk of developing NHL, which is equivalent for both primary and secondary SS, was estimated to be 44 times greater than that observed in a comparable normal population. NHLs in SS patients occur preferentially in the salivary glands and in other mucosa-associated lymphoid tissues (MALT). However, it can also occur in the lymph nodes or bone marrow. We documented a case of low-grade B-cell lymphoma of MALT in the right eyelid and primary biliary cirrhosis (PBC) of a patient with SS. To the best of our knowledge, this is the first case reported in Korea.
Case Report ; Eyelid Neoplasms/pathology ; Eyelid Neoplasms/etiology* ; Female ; Human ; Liver Cirrhosis, Biliary/pathology ; Liver Cirrhosis, Biliary/complications* ; Lymphoma, Mucosa-Associated Lymphoid Tissue/pathology ; Lymphoma, Mucosa-Associated Lymphoid Tissue/etiology* ; Middle Age ; Sjogren's Syndrome/pathology ; Sjogren's Syndrome/complications*

OBJECTIVE: To evaluate the diagnostic performances of salivary gland ultrasonography(SGUS)in Sjögren's syndrome(SS). METHODS: A total of 246 patients with dry mouth and/or eyes who were treated in the outpatient department and inpatient department of Rheumatology and Immunology Department of the Ninth People's Hospital, Shanghai Jiaotong University School of Medicine from December 2019 to January 2022 were collected. All patients received SGUS examination and scored by 2019 outcome measures in rheumatology clinical trial (OMERACT)ultrasonic scoring system.Their general information, unstimulated saliva flow rate(USFR), Schirmer test and serological test results were recorded. In the study, 193 cases had lip gland biopsy. The 2016 American College of Rheumatology(ACR)/ European League Against Rheumatism(EULAR)classification criteria were adopted as the diagnostic standard of SS. χ² test was used to compare the difference of salivary gland ultrasonic scores between the two groups. The receiver operating characteristic(ROC) curve was used to evaluate the accuracy of SGUS in diagnosing SS, and the disease characteristics of SGUS positive group and negative group in the SS patients were compared. RESULTS: A total of 175 patients were SS group according to the ACR/EULAR classification, and the remaining 71 patients were non-SS group.There was no significant difference in age [(54.2±11.8) years vs. (53.4±14.9) years, P=0.705] and female (94.4% vs.93.1%, P=1.000) between SS and non-pSS groups. A total of 109 patients were SGUS positive (≥ 2 points), of whom 104 patients met the SS diagnosis and 5 patients did not meet the SS diagnosis. The positive rate of SGUS in SS group was significantly higher than that in non-SS group (59.4% vs. 7.0%, P < 0.001). The accuracy of 2019 OMERACT ultrasonic scoring system to predict ACR/EULAR classification was good, with an area under the curve of 0.762 (95%CI 0.701-0.823). The absolute agreement between the SGUS outcome and ACR-EULAR classification was 69.1%(170/246), with a sensiti-vity of 59.4%(104/175), specificity of 93%(66/71), positive predictive value of 95.4%(104/109) and negative predictive value of 48.2% (66/137). A total of 81 patients were positive SGUS combined with anti-SSA antibody, 100% (81/81) fulfilled the ACR-EULAR criteria, 85 patients were negative SGUS and anti SSA antibody, and 60 patients(70.6%, 60/85) did not fulfil the ACR-EULAR criteria. SGUS positive group had higher antinuclear antibody(ANA) positive rate(83.1% vs. 98.1%, P < 0.001) in the patients with SS. CONCLUSION The OMERACT ultrasonic scoring system has high diagnostic value in SS. The combination of SGUS and anti-SSA antibody can improve the diagnostic value.
Humans ; Female ; Adult ; Middle Aged ; Aged ; Sjogren's Syndrome/diagnostic imaging* ; Sensitivity and Specificity ; China ; Salivary Glands/pathology* ; Ultrasonography/methods*

We report a 46-year-old woman with primary biliary cirrhosis (PBC) presenting with Sjogren's syndrome and systemic mononuclear inflammatory vasculopathy. Biopsy specimens of sural nerve showed findings consistent with vasculitic neuropathy. Perivascular inflammatory mononuclear cell infiltration was observed on muscle biopsy specimen. The findings of abdominal computed tomography and brain magnetic resonance imaging were suggestive of vasculitis. Clinical manifestations and radiologic findings were improved after high dose prednisolone therapy.
Biopsy, Needle ; Case Report ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Human ; Liver Cirrhosis, Biliary/diagnosis+ACo- ; Liver Cirrhosis, Biliary/complications+ACo- ; Middle Age ; Prednisone/administration +ACY- dosage ; Sjogren's Syndrome/pathology ; Sjogren's Syndrome/diagnosis+ACo- ; Sjogren's Syndrome/complications ; Sural Nerve/pathology ; Treatment Outcome ; Vasculitis/pathology ; Vasculitis/drug therapy ; Vasculitis/diagnosis+ACo- ; Vasculitis/complications

Biopsy ; Female ; Glomerular Basement Membrane ; metabolism ; Hashimoto Disease ; metabolism ; pathology ; Humans ; Kidney Diseases ; metabolism ; pathology ; Middle Aged ; Proteinuria ; metabolism ; pathology ; Sjogren's Syndrome ; metabolism ; pathology