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

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

OBJECTIVE: To investigate the clinical manifestations and laboratory indicators of anti-Sjögren's-syndrome-related antigen A (SSA) antibody associated fetal cardiac disease. METHODS: Pregnant women hospitalized at Peking University People's Hospital from January 2013 to July 2023 were included. Eleven patients with anti-SSA antibody positive were eventually diagnosed with fetal cardiac di-sease. And patients with anti-SSA antibody positive without fetal cardiac disease were selected as controls. Clinical manifestations, laboratory indications and drug usage were compared between the two groups. RESULTS: Among these 11 patients, congenital heart block was confirmed in seven, which was the most common manifestations of fetal cardiac malformation. The proportion of the patients diagnosed with autoimmune disease before pregnancy in fetal cardiac malformation group was significantly lower than that in the control group (P=0.032), while most of the patients in the fetal cardiac malformation group received immune-related examinations for the first time because of this time's fetal cardiac diagnosis. While most of the patients in the control group received routine examinations because of autoimmune diseases diagnosed before pregnancy. During pregnancy, the white blood cell level [(9.29±2.58)×10⁹/L vs. (7.10±1.90×10⁹/L, t=3.052, P=0.004], erythrocyte sedimentation rate [(49.50 (48.00, 51.00) mm/h vs. 23.00 (15.00, 30.25) mm/h, Z=-2.251, P=0.024], IgA level [3.46 (2.30, 5.06) g/L vs. 2.13 (1.77, 2.77) g/L, Z=-2.181, P=0.029], and antinuclear antibody (ANA) titers [1∶320 (1∶160, 1∶320) vs. 1∶80 (1∶40, 1∶160), Z=-3.022, P=0.003] were significantly higher in fetal cardiac malformation group than in the control group. The proportion of positive anti-SSB antibody during pregnancy did not show a statistically significant difference between the two groups (37.5% vs. 7.7%, P=0.053). There was no significant difference in hydroxychloroquine dosage and initiation time between the two groups. The dosage of prednisone in the second and third trimesters was significantly higher in the cardiac malformation group than that in the control group, but there was no significant difference in the first trimester. CONCLUSION Fetal cardiac disease is rare in pregnant women with anti-SSA antibody. White blood cell, erythrocyte sedimentation rate, IgA, the titer of ANA positivity were higher in the fetal heart disease group during pregnancy. Since congenital heart block is difficult to reverse, its prevention and monitoring are more important than remedial treatment.
Humans ; Female ; Pregnancy ; Sjogren's Syndrome/complications* ; Autoimmune Diseases ; Heart Block/diagnosis* ; Autoantibodies ; Antibodies, Antinuclear ; Immunoglobulin A

Sjögren's syndrome(SS)is a chronic autoimmune disease that affects exocrine glands, especially salivary and lacrimal glands. The main clinical manifestations are dry mouth and dry eyes, but also multi-organ and multi-system can be involved. Cold agglutinin disease(CAD)is an autoimmune disease characterized by red blood cell agglutination in the blood vessels of extremities caused by cold agglutinin at low temperature, resulting in skin microcirculation disturbance, or hemolytic anemia. Cold agglutinin disease is divided into two categories, primary cold agglutinin disease and secondary cold agglutinin disease. Primary cold agglutinin disease is characterized with cold agglutinin titer of 1 ∶4 000 or more and positive Coomb's test. However, the Coomb's test is not necessarily positive and the cold agglutinin titer is between 1 ∶32 and 1 ∶4 000 in secondary cold agglutinin disease. Here, we reported an elderly patient admitted to hospital due to fever. He was diagnosed with respiratory infection, but he showed incompletely response to the anti-infection treatment. Further laboratory tests showed the patient with positive ANA and anti-SSA antibodies. Additionally, the patient complained that he had dry mouth and dry eyes for 1 year. Schirmer test and salivate gland imaging finally confirmed the diagnosis Sjogren's syndrome. During the hospital stay, the blood clots were found in the anticoagulant tubes. Hemolytic anemia was considered as the patient had anemia with elevated reticulocytes and indirect bilirubin. In addition, further examination showed positive cold agglutination test with a titer of 1 ∶1 024, and cold agglutinin disease was an important type of cold-resistant autoimmune hemolytic anemia. Furthermore, the patient developed cyanosis after ice incubating at the tip of the nose. Hence, the patient was diagnosed as CAD and he was successfully treated with glucocorticoids instead of anti-infection treatments. Hence, the patient was diagnosed with SS combined with secondary CAD. SS combined CAD are rarely reported, and they are both autoimmune diseases. The abnormal function of B lymphocytes and the production of autoantibodies might be the common pathogenesis of them. Cold agglutinin disease can lead to severe hemolytic anemia, even life-threatening. In clinical practice, timely recognizing and dealing with CAD might promote the prognosis of the patient.
Male ; Humans ; Aged ; Anemia, Hemolytic, Autoimmune/diagnosis* ; Sjogren's Syndrome/diagnosis* ; Anemia, Hemolytic/complications* ; Dry Eye Syndromes/complications* ; Autoantibodies

Both systemic lupus erythematosus and Sjogren's syndrome are autoimmune diseases. Almost all organs can be involved but the gall bladder is an unusual site. We report a 39-year-old woman with systemic lupus erythematosus and Sjogren's syndrome presenting with acute acalculous cholecystitis. It is a very rare complication and, in the literature review, surgical interventions are mostly applied for treatment. In our case, high dose corticosteroid was tried and clinical manifestations and radiologic findings were improved.
Acute Disease ; Adult ; Case Report ; Cholecystitis/*complications/diagnosis/drug therapy ; Female ; Human ; Lupus Erythematosus, Systemic/*complications/diagnosis ; Methylprednisolone/therapeutic use ; Sjogren's Syndrome/*complications/diagnosis ; Tomography, X-Ray Computed

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

Central nervous system involvement in primary Sjögren's syndrome (pSS) is less common and usually presents as white matter lesions, neuromyelitis optica spectrum disorder (NMOSD), or transverse myelitis. NMOSD is an immune-mediated inflammatory demyelinating disease of the central nervous system with a high rate of relapse and significant disability. Studies have shown that patients with pSS combined with NMOSD have more severe symptoms and poorer prognosis. Here, we present a case of critical illness in pregnancy-associated NMOSD combined with Sjögren's syndrome. The patient was a 30-year-old pregnant woman with a history of Sjögren's syndrome who was diagnosed with NMOSD. She received combination therapy with steroids, intravenous immunoglobulin (IVIG), and hydroxychloroquine during pregnancy, resulting in partial resolution of numbness below the waist. However, due to irregular medication adherence outside the hospital setting, she developed weakness in her right lower limb accompanied by inability to move it, while her left lower limb still had some mobility but occasional numbness along with urinary and fecal incontinence. Ten days later, she was admitted to the emergency department where an emergency cesarean section was performed to deliver a healthy baby boy. However, her condition worsened postpartum as she developed high fever accompanied by bilateral lower limb paralysis and weakness along with loss of voluntary control over urination and defecation. The patient underwent ano-ther course of treatment consisting of steroids and IVIG; however there was limited improvement in symptoms observed after this intervention. Following administration of rituximab for the first time, the patient developed urinary tract infection which was successfully managed before continuing regular infusions. In later stages the patient could walk slightly with a limp and regained control over urination and defecation, allowing her to resume normal activities. This case suggests that combination therapy with steroids, IVIG, and hydroxychloroquine should be considered for the patients with pregnancy-associated NMOSD combined with Sjögren's syndrome. Rituximab can significantly improve symptoms such as postpartum paralysis in patients with NMOSD, however, there may be a risk of infection associated with its use.
Adult ; Female ; Humans ; Pregnancy ; Cesarean Section/adverse effects* ; Critical Illness ; Hydroxychloroquine/therapeutic use* ; Hypesthesia/complications* ; Immunoglobulins, Intravenous/therapeutic use* ; Inflammation/complications* ; Neuromyelitis Optica/diagnosis* ; Paralysis/complications* ; Pregnancy Complications/therapy* ; Rituximab/therapeutic use* ; Sjogren's Syndrome/complications* ; Steroids/therapeutic use* ; Vision Disorders

Sjogren's syndrome (SS) describes xeropthalmia and xerostomia due to lymphocytic infiltrates of lacrimal and salivary glands. SS may occur alone (primary SS) or in association with several other autoimmune diseases (secondary SS). The clinical features involve a wide variety of organs, including skin, eyes, oral cavity and salivary glands, and systems, including nervous, musculoskeletal, genitourinary and vascular. Sicca symptoms can be found in a number of other disorders including rheumatoid arthritis, systemic lupus erythematosus, scleroderma, primary biliary cirrhosis, and other rheumatic disorders.
Eye Diseases/etiology ; History of Medicine, 20th Cent. ; Human ; Mouth Diseases/etiology ; *Sjogren's Syndrome/complications/diagnosis/history/physiopathology/therapy ; Skin Diseases/etiology ; Sweden

Adolescent ; Adult ; Anemia ; drug therapy ; etiology ; Diagnosis, Differential ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Humans ; Male ; Medicine, Chinese Traditional ; Phytotherapy ; Sjogren's Syndrome ; complications ; drug therapy

Remitting seronegative symmetric synovitis with pitting edema (RS3 PE) syndrome is characterized by symmetrical and acute synovitis, pitting edema, the absence of rheumatoid factor, increased acute phase reactants, lack of bony erosions on radiography, and benign and short clinical course. Half of all patients with Sjogren's syndrome experience arthritis during the disease course. We here describe the first case of Sjogren's syndrome presenting as RS3PE. She had swelling in knees, ankles, and wrists. After then the swelling spread to her lower legs, feet, face, and both hands. She was admitted to another hospital and was suspected of lupus or rheumatoid arthritis. Three months later, she had dry mouth and had lower lip biopsy. She was admitted to this hospital due to development of swelling in face and lower legs for 3 days. On physical examination, she had pitting edema in both hands and feet dorsum. Laboratory test showed elevated erythrocyte sedimentation rate, positivity of rheumatoid factor, anti-nuclear antibody, and anti-Ro antibody. There was no erosion in the hands radiography. Schirmer's test and lip biopsy was compatible with Sjogren's syndrome. She was diagnosed RS3 PE and Sjogren's syndrome. She was begun with prednisolone and her symptoms improved gradually.
Adult ; Antibodies, Antinuclear/biosynthesis ; Arthritis/complications ; Biopsy ; Blood Sedimentation ; Diagnosis, Differential ; Edema/*diagnosis ; Female ; Human ; Lymphocytes/pathology ; Prednisolone/therapeutic use ; Rheumatoid Factor/biosynthesis ; Salivary Glands/pathology ; Sjogren's Syndrome/*diagnosis ; Syndrome ; Synovitis/*diagnosis