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

Female ; Humans ; Hypertension, Pulmonary ; etiology ; Middle Aged ; Sjogren's Syndrome ; complications

Anemia, Hemolytic, Autoimmune ; etiology ; Female ; Humans ; Infant, Newborn ; Male ; Pregnancy ; Pregnancy Complications ; Sjogren's Syndrome ; complications

Adult ; Factor XIII ; antagonists & inhibitors ; Factor XIII Deficiency ; complications ; etiology ; Female ; Humans ; Sjogren's Syndrome ; complications

OBJECTIVE: To explore the effect of hypothyroidism (HT) on the ocular surface status of patients with primary Sjögren's syndrome-related dry eye (pSS-DED). METHODS: The cross-sectional study included 36 patients with pSS-DED who were treated at the dry eye clinic of Peking University Third Hospital from December 2020 to June 2021, of whom 12 were pSS-DED patients combined with HT. In the same period, 24 patients with simple dry eye disease (DED) were served as a control group. All the patients filled out the Ocular Surface Disease Index (OSDI) questionnaire, and performed tear film break-up time (BUT), Schirmer test, tear meniscus height, corneal/conjunctival fluorescein staining, meibomian gland secretion capacity, meibum evaluation and confocal microscope examination. RESULTS: (1) Compared with pSS-DED and simple DED patients, pSS-DED +HT patients had lower average BUT [(2.7±0.8) s], Schirmer test [(4.9±4.8) mm] and tear meniscus height [(0.13±0.03) mm], and the difference was statistically significant (F=12.43, P < 0.01; F=6.96, P < 0.01; F=3.31, P < 0.05). (2) Compared with DED and pSS-DED patients, the meibomian gland secretion capacity and meibomian trait scores of pSS-DED+HT patients were mainly distributed in the high division. There were statistically significant differences in the distribution of secretion capacity of meibomian glands (χ²=10.72, P < 0.05) and meibomian trait assessment scores (χ²=8.34, P < 0.05) among the three groups. (3) Serum total thyroxine and serum free thyroxine levels in the pSS-DED+HT patients showed positive correlation (P < 0.05, P < 0.05) with their BUT (r=0.60, 0.60), Schirmer's test (r=0.64, 0.66) and tear river height (r=0.61, 0.62), independent of lid gland secretory capacity; no significant correlation was found between thyroid-stimulating hormone, anti-thyroglobulin antibody and lid gland secretory capacity. Thyroid hormone, anti-thyroglobulin antibody, and thyroid peroxidase antibody were not found to be significantly correlated with ocular surface status. (4) Compared with pSS-DED, the fiber density of the subbasal nerve plexus in pSS-DED+HT group decreased (t=2.06, P < 0.05), and the curvature score increased (t=2.13, P < 0.05). CONCLUSION The ocular surface condition of pSS-DED patients with HT is worse than that of pSS-DED and DED patients. The main manifestations are that tear secretion, tear film stability, secretory function of the meibomian glands, meibum trait and fiber density of the subbasal nerve plexus decrease while the curvature increases. The mechanism might be related to the decrease in thyroid hormone production.
Cross-Sectional Studies ; Dry Eye Syndromes/etiology* ; Humans ; Hypothyroidism/complications* ; Sjogren's Syndrome/complications* ; Thyroxine

Annular erythema (AE) associated with anti-Ro (SSA) and/or La (SSB) autoantibody in patients with Sjogren syndrome (SS) or with SS/systemic lupus erythematosus overlap syndrome (SS/SLE), has recently been described in Orientals, and it may be a counterpart of annular skin lesion of the subacute cutaneous LE seen mostly in Caucasians. The author examined five Korean AE patients in respect to dinical diversity. In this small-sample study, subtle differences appeared between individual cases regarding the serologic features and the diagnoses of the disease. Among the five cases, four had circulating anti-Ro and anti-La antibodies, and one had only anti-La. Regarding the diagnosis, one was SS/SLE, two were primary SS, and the remaining two were only "AE associated with anti-Ro/La antibody". There seem to be a wide clinical spectrum in the disease expression of AE associated with anti-Ro/La autoantibody than previously thought.
Adolescence ; Adult ; Antibodies, Antinuclear/immunology* ; Case Report ; Erythema/immunology* ; Erythema/etiology ; Female ; Human ; Lupus Erythematosus, Systemic/immunology* ; Lupus Erythematosus, Systemic/complications ; Male ; Sjogren's Syndrome/immunology* ; Sjogren's Syndrome/complications

Adult ; Female ; Humans ; Intestinal Obstruction ; etiology ; Sjogren's Syndrome ; complications ; drug therapy

OBJECTIVETo assess the clinical features of primary Sjgren's syndrome (pSS) in childhood and adult patients to understand the differences between them.

METHODData of 21 childhood and 400 adult patients with definite primary Sjgren's syndrome were analyzed retrospectively.

RESULTSIn adult patients, initial clinical symptoms were various, dry mouth and dry eyes, arthritis and arthralgia, parotid swelling, renal tubular abnormalities (RTA) were more common. Compared with adult patients, RTA, parotid swelling and rash were more common in childhood, pulmonary abnormalities and neurologic system involvement were less common. The frequency of renal tubular abnormalities (52.4%) and rash (47.6%) in childhood pSS were higher than those of adults, but the frequencies of dry eyes (61.0%) and pulmonary interstitial fibrosis (25.2%) in adults pSS were higher than those in childhood (P < 0.01); serum rheumatoid factor (RF) and gamma-globulin were higher in all childhood patients (P < 0.01). Anti-nuclear antibody (ANA), anti-SSA and anti-SSB antibodies, and IgG were significantly higher in childhood cases than in adult patients (P < 0.05 or 0.01).

CONCLUSIONThe major clinical characteristics of childhood pSS cases included: (1) RTA, parotid swelling and rash which appeared earlier, but dry mouth and dry eyes appeared later and were mild. (2) The frequency of pulmonary abnormalities, nervous system involvement and Raynaud's phenomenon were less commonly seen. (3) Positivity of RF, anti-SSA and anti-SSB antibodies, and serum IgG were higher.

Adult ; Autoantibodies ; blood ; Child ; Humans ; Retrospective Studies ; Sjogren's Syndrome ; blood ; complications ; diagnosis

Humans ; Nephritis ; etiology ; therapy ; Prognosis ; Purpura, Schoenlein-Henoch ; complications ; therapy ; Sjogren's Syndrome ; etiology ; therapy

OBJECTIVE: To understand the clinical characteristics about sequence diagnosis and treatment of oral complications in patients with Sjögren's syndrome (SS) through retrospective analysis, and to provide some guidance for clinical work. METHODS: Some SS patients who underwent oral sequence management in the Department of General Dentistry, Peking University School and Hospital of Stomatology from January 2015 to September 2021 were enrolled. For the SS patients included in this study, a comprehensive oral examination was performed, including parotid region examination, oral mucosal exa-mination, dentition examination, dental examination, periodontal examination, unstimulated salivary flow rate, Candida infection and radiological imaging examination. According to the examination results, the patients were given fluoride application, antifungal treatment, root canal therapy, direct filling repair, and indirect repair treatment in sequence and the results recorded. RESULTS: A total of 9 patients with SS, with 4 primary SS patients (pSS) and 5 secondary SS patients (sSS) were enrolled in the study. For all the 9 patients, the average age was (49.2±16.2) years and the median xerostomia duration 5 years. The unstimulated salivary flow rate of the 9 patients was all less than 1 mL/10 min. Eight of the 9 cases was diagnosed as oral Candidiasis, with positive salivary Candida culture result (>200 cfu/mL), and 1 of the 9 cases was not. The average decay, missing, filling teeth (DMFT) was 24.8±4.2; the average decay, missing, filling tooth surfaces (DMFS) was 59.2±21.9, the average incisal caries was 2.5±1.3, and the average number of crown restorations at baseline was 4.5±3.6. All the 9 SS patients were applied with topical fluoride usage, and 8 were prescribed with antifungal treatment. One sSS patient was conducted with filling restoration treatment, one pSS patient was conducted with full mouth rehabilitation, and the remaining 7 patients were conducted with direct filling combined with fixed repair treatment. The average 3.2 full crown restorations in 6 patients had to be removed and restored because of secondary caries, and 3 of the 9 patients underwent implant denture restorations finally. CONCLUSION Management of oral complications in SS patients needs to be carried out in sequence. A comprehensive examination and diagnosis should be carried out first, followed by infection control, and then restoration of oral function at last.
Humans ; Adult ; Middle Aged ; Aged ; Sjogren's Syndrome/complications* ; Retrospective Studies ; Antifungal Agents ; Xerostomia ; Fluorides