This case study aims to contribute to the literature in order to highlight the importance of this collaboration between medical specialties. A female patient R.N.N. F, age 66, from the city of Manaus, with a previous ...This case study aims to contribute to the literature in order to highlight the importance of this collaboration between medical specialties. A female patient R.N.N. F, age 66, from the city of Manaus, with a previous diagnosis of Sjogren’s syndrome in regular follow-up by the Rheumatology team at the Araujo Lima outpatient clinic and referred to the Ophthalmology sector for complementary evaluation related to visual discomfort. The fundoscopy performed in the patient was within normal limits, but the symptoms experienced by her proved to be an important clinical finding, which has ratified the need for regular and multidisciplinary follow-up. This report unequivocally demonstrates that even in the face of tests considered within the expected limits for a given population, the clinical presentation can be specific and particular for each analyzed individual. Early screening exams should contemplate the patient in a holistic and individualized way whenever possible.展开更多
Sjogren’s syndrome is a chronic autoimmune exocrinopathy associated with dry eyes and dry mouth as major clinical manifestations. It is characterized by lymphocytic infiltration of lacrimal and salivary glands and au...Sjogren’s syndrome is a chronic autoimmune exocrinopathy associated with dry eyes and dry mouth as major clinical manifestations. It is characterized by lymphocytic infiltration of lacrimal and salivary glands and autoantibody production, especially anti-Ro (or SSA) and anti-La (or SSB). Lymphoproliferative disorders are a feature of Sjogren’s syndrome, and can be considered an extraglandular manifestation of the disease. Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a rare form of lymphadenitis. It is reported more often in young adult women with localized lymphadenopathy (usually cervical), fever, rashes, and leukopenia. It is a self-limiting disease with resolution within 1 - 4 months in almost all patients. Sjogren’s syndrome has been reported in patients with other systemic diseases including SLE and lymphomas. Here we present a patient with Kikuchi-Fujimoto disease who developed Sjogren’s Syndrome 8 years after her diagnosis of Kikuchi-Fujimoto disease.展开更多
BACKGROUND Crohn’s disease(CD)is a chronic inflammatory bowel disorder that progresses to bowel damage(BD)over time.An image-based index,the Lémann index(LI),has been developed to measure cumulative BD.AIM To ch...BACKGROUND Crohn’s disease(CD)is a chronic inflammatory bowel disorder that progresses to bowel damage(BD)over time.An image-based index,the Lémann index(LI),has been developed to measure cumulative BD.AIM To characterize the long-term progression of BD in CD based on changes in the LI and to determine risk factors for long-term progression.METHODS This was a single-center longitudinal cohort study.Patients who had participated in prospective studies on the accuracy of magnetic resonance imaging using endoscopy as a gold standard and who had a follow-up of at least 5 years were reevaluated after 5-12 years.RESULTS Seventy-two patients were included.LI increased in 38 patients(52.8%),remained unchanged in 9 patients(12.5%),and decreased in 25 patients(34.7%).The small bowel score and surgery subscale significantly increased(P=0.002 and P=0.001,respectively),whereas the fistulizing subscale significantly decreased(P=0.001).Baseline parameters associated with BD progression were ileal location(P=0.026),CD phenotype[stricturing,fistulizing,or both(P=0.007,P=0.006,and P=0.035,respectively)],disease duration>10 years(P=0.019),and baseline LI stricturing score(P=0.049).No correlation was observed between BD progression and baseline clinical activity,biological markers,or severity of endoscopic lesions.CONCLUSION BD,as assessed by the LI,progressed in half of the patients with CD over a period of 5-12 years.The main determinants of BD progression were ileal location,stricturing/fistulizing phenotype,and disease duration.展开更多
Mesenteric Panniculitis is a benign fibro-inflammatory process involving adipose tissue of the mesentery. It is characterised by fat necrosis, chronic inflammation and fibrosis, causing thickening and shortening of th...Mesenteric Panniculitis is a benign fibro-inflammatory process involving adipose tissue of the mesentery. It is characterised by fat necrosis, chronic inflammation and fibrosis, causing thickening and shortening of the mesentery. Patients may present with localised abdominal pain, abdominal mass, intestinal obstruction and ischaemic colitis. We report a case of mesenteric panniculitis causing abdominal pain in a patient with active Primary Sjogren’s Syndrome. The rarity of this case makes it of interest. We review the current literature on mesenteric panniculitis and its association with connective tissue disease and inflammatory conditions. A 64-year-old Caucasian male presented in 1994 with dry mouth. A diagnosis of Primary Sjogren’s Syndrome (PSS) was made on salivary gland biopsy. In 2010 he presented with an exacerbation of his eye symptoms, muscle pain and fatigue. He complained of abdominal pain and night sweating, but denied any weight loss or change in bowel habit. There was no significant past medical history other than PSS. On examination he had a small right submandibular node and mild synovitis at the right proximal interphalangeal joint and carpometacarpal joint. Examination of the abdomen showed marked umbilical tenderness but no organomegally. Blood tests at this time showed an active inflammation: CRP of 61 (NR 5 mg/L), ESR 39 (NR 20 mm/s), strongly positive ENA Ro and La. IgG was elevated at 18.6 (NR 5.8-15.4), Complement was low at 0.17 (NR 0.18-0.6). An abdominal ultrasound scan demonstrated a 6 × 3 ×3 cmarea of diffuse homogenous fat encasing some mesenteric vessels in the area of focal tenderness. CT abdomen and pelvis showed oedematous mesenteric fat and lymph nodes in the jejunal small bowel mesentery, consistent with mesenteric panniculitis. Laparoscopic biopsy was discussed with the surgical team, but was felt not indicated as risk outweighed potential benefit. The patient was treated with a 9-week reducing course of oral steroids. His abdominal symptoms resolved although CT abdomen showed little improvement in mesenteric panniculitis. A review of the literature suggests that currently there is no standard treatment and management should be guided by patient symptoms. Mesenteric Panniculitis is rare;as a result evidence for treatment is limited to individual case reports. There is no clear link between symptom improvement and radiological resolution of mesenteric panniculitis. It has, therefore been suggested that follow-up imaging should be limited to those with persistent symptoms. Overall the prognosis for mesenteric panniculitis is good, up to half of patients do not require treatment, and recurrence of symptoms is uncommon.展开更多
AIM:To establish the frequency and clinical features of connective tissue diseases(CTDs)in a cohort of Chinese patients with primary biliary cirrhosis(PBC).METHODS:Three-hundred and twenty-two Chinese PBC patients wer...AIM:To establish the frequency and clinical features of connective tissue diseases(CTDs)in a cohort of Chinese patients with primary biliary cirrhosis(PBC).METHODS:Three-hundred and twenty-two Chinese PBC patients were screened for the presence of CTD,and the systemic involvement was assessed.The differences in clinical features and laboratory findings between PBC patients with and without CTD were documented.The diversity of incidence of CTDs in PBC of different countries and areas was discussed.For the comparison of normally distributed data,Student’s t test was used,while non-parametric test(Wilcoxon test)for the non-normally distributed data and 2×2χ2or Fisher’s exact tests for the ratio.RESULTS:One-hundred and fifty(46.6%)PBC patients had one or more CTDs.The most common CTD was Sj gren’s syndrome(SS,121 cases,36.2%).There were nine cases of systemic sclerosis(SSc,2.8%),12of systemic lupus erythematosus(SLE,3.7%),nine of rheumatoid arthritis(RA,2.8%),and 10 of polymyositis(PM,3.1%)in this cohort.Compared to patients with PBC only,the PBC+SS patients were more likely to have fever and elevated erythrocyte sedimentation rate(ESR),higher serum immunoglobulin G(IgG)levels and more frequent rheumatoid factor(RF)and interstitial lung disease(ILD)incidences;PBC+SSc patients had higher frequency of ILD;PBC+SLE patients had lower white blood cell(WBC)count,hemoglobin(Hb),platelet count,γ-glutamyl transpeptidase and immunoglobulin M levels,but higher frequency of renal involvement;PBC+RA patients had lower Hb,higher serum IgG,alkaline phosphatase,faster ESR and a higher ratio of RF positivity;PBC+PM patients had higher WBC count and a tendency towards myocardial involvement.CONCLUSION:Besides the common liver manifestation of PBC,systemic involvement and overlaps with other CTDs are not infrequent in Chinese patients.When overlapping with other CTDs,PBC patients manifested some special clinical and laboratory features which may have effect on the prognosis.展开更多
Interstitial lung disease(ILD) is an important mani-festation of collagen vascular diseases. It is a common feature of scleroderma, and also occurs in dermatomyositis and polymyositis, mixed connective tissue disease,...Interstitial lung disease(ILD) is an important mani-festation of collagen vascular diseases. It is a common feature of scleroderma, and also occurs in dermatomyositis and polymyositis, mixed connective tissue disease, Sjogren's syndrome, rheumatoid arthritis, systemic lupus erythematosus, and Antineutrophil cytoplasmic antibody-associated vasculitis. When present, it is associated with increased morbidity and mortality, thus making early diagnosis important. In fact, in many patients, ILD may be the first manifestation of a collagen vascular disease. The most common symptoms are cough and dyspnea. The diagnosis is made based on pulmonary function tests showing restrictive lung disease and impaired oxygen diffusion and chest imaging showing ground glass infiltrates, interstitial thickening, and/or fibrosis. The most common histologic finding on lung biopsy is non-specific interstitial pneumonia, though organizing pneumonia and usual interstitial pneumonia may also be seen. Treatment is focused on addressing the underlying collagen vascular disease with immunosuppression, either with corticosteroids or a steroid-sparing agent such as cyclophosphamide, azathioprine, or mycophenolate, although the optimal agent and duration of therapy is not known. There are few clinical trials to guide therapy that focus specifically on the progression of ILD. The exception is in the case of scleroderma-associated ILD, where cyclophosphamide has been shown to be effective.展开更多
Because inflammation plays a key role in the pathogenesis of dry eye disease and Sjogren's syndrome, topical anti-inflammatory agents such as corticosteroids and cyclosporine A have been used to treat inflammation...Because inflammation plays a key role in the pathogenesis of dry eye disease and Sjogren's syndrome, topical anti-inflammatory agents such as corticosteroids and cyclosporine A have been used to treat inflammation of the ocular surface and lacrimal gland. Systemic biological agents that target specific immune molecules or cells such as tumor necrosis factor(TNF)-α, interferone-α, interleukin(IL)-1, IL-6, or B cells have been used in an attempt to treat Sjogren's syndrome. However, the efficacy of systemic biological agents, other than B-cell targeting agents, has not yet been confirmed in Sjogren's syndrome. Several studies have recently evaluated the efficacy of topical administration of biological agents targeting cytokines in the treatment of dry eye disease. Topical blockade of IL-1 by using IL-1 receptor antagonist could ameliorate clinical signs and inflammation of experimental dry eye. Using a mouse model of desiccating stress-induced dry eye, we have demonstrated that topical application of a TNF-α blocking agent, infliximab, could improve tear production and ocular surface irregularity, decrease inflammatory cytokines and Th-1 CD4+ cells on the ocular surface, and increase gobletcell density in the conjunctiva. Although controversy still remains, the use of topical biological agents targeting inflammatory cytokines may be a promising therapy for human dry eye disease.展开更多
Objective: To explore the change of intestinal microecology in patients with primary Sjogren’s syndrome(p SS) and correlation with disease activity, and also discuss the therapy effect of Yangyin Yiqi Huoxue Recipe(...Objective: To explore the change of intestinal microecology in patients with primary Sjogren’s syndrome(p SS) and correlation with disease activity, and also discuss the therapy effect of Yangyin Yiqi Huoxue Recipe(养阴益气活血方, YYHD). Methods: Sixteen p SS patients were enrolled in the present study, who received 3-month treatment of YYHR, 200 mL orally twice daily. Their pre-and post-test ESSDAI scores, erythrocyte sedimentation rate(ESR) and serum immunoglobulin G(IgG) levels were measured respectively. The 16 Sr DNA metagenomic sequencing was used to detect and analyze the abundance and diversity of intestinal bacteria flora and the proportion of bacteria at the levels of phylum, family, and genus, in comparision with those of 6 healthy subjects in the control group. Results: The abundance and diversity of intestinal bacteria flora in pSS patients were lower than those of healthy subjects(P<0.05). After the treatment with YYHD, patients’ ESSDAI score and levels of IgG and ESR have decreased significantly(P<0.05). At the phylum level, the proportions of Actinobacteria, Firmicutes, Fusobacteria and Proteobacteria have reduced sharply, while the proportions of Bacteroidetes, Teneriquetes and Candidate-division-TM7 have increased significantly by treatment(all P<0.05). At the classification level, such treatment has caused a significant decrease in the proportions of Bacteroidaceae, Ruminococcaceae, Veillonellaceae, and Enterobacteriacea(all P<0.05), but a significant increase in the proportion of Lachnospiraceae(P<0.05). At the genus level, the treatment has significantly decreased the proportions of Bifidobacterium, Bacteroides, Escherichia-Shigella, Faecalibacterium and Prevotella(all P<0.05), but significantly increased the proportion of Clostridia(P<0.05), close to the levels of healthy subjects(P>0.05). Conclusions: There exists an imbalance of intestinal microecology in p SS patients, which can be improved through the treatment with YYHD. Besides, such treatment can also improve the disease activity and adjust the diversity of intestinal bacteria flora, the composition and the abundance of intestinal flora.展开更多
Objective： To observe the effect of Chinese herbal medicine for nourishing yin, supplementing qi, and activating blood on the expression of interferen- γ （IFN-γ,）/interleukin-4 （IL-4） in peripheral blood and di...Objective： To observe the effect of Chinese herbal medicine for nourishing yin, supplementing qi, and activating blood on the expression of interferen- γ （IFN-γ,）/interleukin-4 （IL-4） in peripheral blood and disease activity in primary Sjogren＇s syndrome （pSS） patients, and to study the relationship between the immune balance of Th1/Th2 and the disease activity. Methods： A total of 66 pSS patients were randomized with tossing coins method into two groups： the integrative therapy group （34 cases） and the control group （32 cases）; and 28 healthy subjects were taken as the normal group. The integrative therapy group was treated by Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood combined with hydroxychloroquine sulfate tablets and the control group was treated with hydroxychloroquine sulfate tablets. The treatment course was 3 months for both groups. The levels of serum immunoglobulin G （IgG）, erythrocyte sedimentation rate （ESR）, IFN-γand IL-4 in peripheral blood were measured before and after treatment. Results： Compared with the normal group, the levels of IgG, ESR, IFN- γ and IL-4 were significantly increased in pSS patients （P〈0.05）. Remarkably, after 3 months of treatment, these levels were dramatically decreased in both the integrative therapy group and the control group, although still higher than the normal group. The levels of IgG, ESR, IFN- and IL-4 in the integrative therapy group were lower than the control group and the same group before treatment （P〈0.05）. The ratio of IFN-γ/IL-4 also significantly decreased after treatment. Moreover, the level of IFN- γ, and the ratio of IFN- γ/IL-4 in the integrative theraphy group were significantly lower than the control group （P〈0.05）. For all patients the ratio of IFN-γ/IL-4 before and after treatment was positive correlated with the levels of IgG and ESR. Conclusion： Chinese herbal medicine for nourishing yin, supplementing qi, and activating blood can alleviate the disease activity of pSS by regulating the immune balance of Th1/Th2.展开更多
ABSTRACT Objective： To investigate the effect of Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood on the reproductive endocrine-immune network and its mechanisms in patients with pr...ABSTRACT Objective： To investigate the effect of Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood on the reproductive endocrine-immune network and its mechanisms in patients with primary Sjogren＇s syndrome （pSS）. Methods： Seventy pSS patients were randomly assigned to two groups using a randomized digital table： the integrative therapy group （36 cases） and the control group （34 cases）. Thirty healthy subjects were taken as a normal group. The control group was treated with hydroxychloroquine sulfate tablets alone, and the integrative therapy group was treated by Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood combined with hydroxychloroquine sulfate tablets. The treatment course was 6 months for both groups. Before and after treatment, serum estradiol （E2）, testosterone （T）, luteinizing hormone （LH）, prolactin （PRL） by radioimmunoassay and immunoglobulin （IgG） by immunodiffusion, erythrocyte sedimentation rate （ESR） by Westergren, interferon-γ （IFN-γ） and interleukin-4 （IL-4） by enzyme linked immunosorbent assay were determined. Results： E2 and T levels in all patients were lower than those of normal subjects before treatment （P〈0.05） and were increased significantly after 6-month treatment （P〈0.05）. ESR, FSH, LH, IgG, IFN - γ, IL - 4 and ratios of E2/T, and IFN --γ/IL in the patients were higher than those of normal subjects before the treatments （P〈0.05）, and were reduced significantly after the treatments （P〈0.05）. The T and IFN -γ levels and E2/T ratio in the patients treated with integrative therapy were reduced significantly compared with the control group （P〈0.05）. However, the PRL levels before and after treatment were not significantly changed in the two groups （P〉0.05）. The ratios of E2/T and IFN -γ/IL-4, and levels of IgG and ESR were positively correlated before and after treatment （P〈0.05）. Conclusions： The ratios of E2/T and IFN - γ/ IL-4 might be used as indicators of pSS activity. Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood combined with Western medicine could improve the therapeutic effect by regulating the reproductive endocrine-immune network in pSS patients.展开更多
Primary Sjogren’s syndrome(p SS)is a chronic autoimmune disease involving exocrine glands.Current studies have found that the occurrence of the disease is closely related to genetic,environmental and neuroendocrine f...Primary Sjogren’s syndrome(p SS)is a chronic autoimmune disease involving exocrine glands.Current studies have found that the occurrence of the disease is closely related to genetic,environmental and neuroendocrine factors,as well as abnormal activation of T and B lymphocytes.The etiology and pathogenesis of p SS is complex,and there is a lack of specific targeted drugs.Traditional Chinese medicines(TCMs)have been comprehensively investigated for their treatment effects on p SS.Through a systematic review of the literature,we summarized the TCMs used to treat p SS,and find that there are four major ways that TCMs are used,including upregulation of aquaporin proteins,suppression of cell apoptosis,suppression of the abnormal activation of B lymphocytes and suppression of the abnormal activation of T lymphocytes(balancing T helper type[Th]1/Th 2& Th17/Treg and suppressing follicular helper T[Tfh]cells).However,there are not enough data about the active constituents,quality control,pharmacokinetics,toxicity and modern preparations of these TCMs;therefore,more investigations are needed.This paper highlights the importance of TCMs for treating p SS and provides guidance for future investigations.展开更多
文摘This case study aims to contribute to the literature in order to highlight the importance of this collaboration between medical specialties. A female patient R.N.N. F, age 66, from the city of Manaus, with a previous diagnosis of Sjogren’s syndrome in regular follow-up by the Rheumatology team at the Araujo Lima outpatient clinic and referred to the Ophthalmology sector for complementary evaluation related to visual discomfort. The fundoscopy performed in the patient was within normal limits, but the symptoms experienced by her proved to be an important clinical finding, which has ratified the need for regular and multidisciplinary follow-up. This report unequivocally demonstrates that even in the face of tests considered within the expected limits for a given population, the clinical presentation can be specific and particular for each analyzed individual. Early screening exams should contemplate the patient in a holistic and individualized way whenever possible.
文摘Sjogren’s syndrome is a chronic autoimmune exocrinopathy associated with dry eyes and dry mouth as major clinical manifestations. It is characterized by lymphocytic infiltration of lacrimal and salivary glands and autoantibody production, especially anti-Ro (or SSA) and anti-La (or SSB). Lymphoproliferative disorders are a feature of Sjogren’s syndrome, and can be considered an extraglandular manifestation of the disease. Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a rare form of lymphadenitis. It is reported more often in young adult women with localized lymphadenopathy (usually cervical), fever, rashes, and leukopenia. It is a self-limiting disease with resolution within 1 - 4 months in almost all patients. Sjogren’s syndrome has been reported in patients with other systemic diseases including SLE and lymphomas. Here we present a patient with Kikuchi-Fujimoto disease who developed Sjogren’s Syndrome 8 years after her diagnosis of Kikuchi-Fujimoto disease.
基金Supported by the Helmsley Charitable Trust Grant,No.2015PG-IBD005.
文摘BACKGROUND Crohn’s disease(CD)is a chronic inflammatory bowel disorder that progresses to bowel damage(BD)over time.An image-based index,the Lémann index(LI),has been developed to measure cumulative BD.AIM To characterize the long-term progression of BD in CD based on changes in the LI and to determine risk factors for long-term progression.METHODS This was a single-center longitudinal cohort study.Patients who had participated in prospective studies on the accuracy of magnetic resonance imaging using endoscopy as a gold standard and who had a follow-up of at least 5 years were reevaluated after 5-12 years.RESULTS Seventy-two patients were included.LI increased in 38 patients(52.8%),remained unchanged in 9 patients(12.5%),and decreased in 25 patients(34.7%).The small bowel score and surgery subscale significantly increased(P=0.002 and P=0.001,respectively),whereas the fistulizing subscale significantly decreased(P=0.001).Baseline parameters associated with BD progression were ileal location(P=0.026),CD phenotype[stricturing,fistulizing,or both(P=0.007,P=0.006,and P=0.035,respectively)],disease duration>10 years(P=0.019),and baseline LI stricturing score(P=0.049).No correlation was observed between BD progression and baseline clinical activity,biological markers,or severity of endoscopic lesions.CONCLUSION BD,as assessed by the LI,progressed in half of the patients with CD over a period of 5-12 years.The main determinants of BD progression were ileal location,stricturing/fistulizing phenotype,and disease duration.
文摘Mesenteric Panniculitis is a benign fibro-inflammatory process involving adipose tissue of the mesentery. It is characterised by fat necrosis, chronic inflammation and fibrosis, causing thickening and shortening of the mesentery. Patients may present with localised abdominal pain, abdominal mass, intestinal obstruction and ischaemic colitis. We report a case of mesenteric panniculitis causing abdominal pain in a patient with active Primary Sjogren’s Syndrome. The rarity of this case makes it of interest. We review the current literature on mesenteric panniculitis and its association with connective tissue disease and inflammatory conditions. A 64-year-old Caucasian male presented in 1994 with dry mouth. A diagnosis of Primary Sjogren’s Syndrome (PSS) was made on salivary gland biopsy. In 2010 he presented with an exacerbation of his eye symptoms, muscle pain and fatigue. He complained of abdominal pain and night sweating, but denied any weight loss or change in bowel habit. There was no significant past medical history other than PSS. On examination he had a small right submandibular node and mild synovitis at the right proximal interphalangeal joint and carpometacarpal joint. Examination of the abdomen showed marked umbilical tenderness but no organomegally. Blood tests at this time showed an active inflammation: CRP of 61 (NR 5 mg/L), ESR 39 (NR 20 mm/s), strongly positive ENA Ro and La. IgG was elevated at 18.6 (NR 5.8-15.4), Complement was low at 0.17 (NR 0.18-0.6). An abdominal ultrasound scan demonstrated a 6 × 3 ×3 cmarea of diffuse homogenous fat encasing some mesenteric vessels in the area of focal tenderness. CT abdomen and pelvis showed oedematous mesenteric fat and lymph nodes in the jejunal small bowel mesentery, consistent with mesenteric panniculitis. Laparoscopic biopsy was discussed with the surgical team, but was felt not indicated as risk outweighed potential benefit. The patient was treated with a 9-week reducing course of oral steroids. His abdominal symptoms resolved although CT abdomen showed little improvement in mesenteric panniculitis. A review of the literature suggests that currently there is no standard treatment and management should be guided by patient symptoms. Mesenteric Panniculitis is rare;as a result evidence for treatment is limited to individual case reports. There is no clear link between symptom improvement and radiological resolution of mesenteric panniculitis. It has, therefore been suggested that follow-up imaging should be limited to those with persistent symptoms. Overall the prognosis for mesenteric panniculitis is good, up to half of patients do not require treatment, and recurrence of symptoms is uncommon.
基金Supported by Grants from the Research Special Fund for Public Welfare Industry of Health,No.201202004the National Major Scientific and Technological Special Project for"Significant New Drugs Development",No.2012ZX09303006-002the National High Technology Research and Development Program of China,No.2011AA020111
文摘AIM:To establish the frequency and clinical features of connective tissue diseases(CTDs)in a cohort of Chinese patients with primary biliary cirrhosis(PBC).METHODS:Three-hundred and twenty-two Chinese PBC patients were screened for the presence of CTD,and the systemic involvement was assessed.The differences in clinical features and laboratory findings between PBC patients with and without CTD were documented.The diversity of incidence of CTDs in PBC of different countries and areas was discussed.For the comparison of normally distributed data,Student’s t test was used,while non-parametric test(Wilcoxon test)for the non-normally distributed data and 2×2χ2or Fisher’s exact tests for the ratio.RESULTS:One-hundred and fifty(46.6%)PBC patients had one or more CTDs.The most common CTD was Sj gren’s syndrome(SS,121 cases,36.2%).There were nine cases of systemic sclerosis(SSc,2.8%),12of systemic lupus erythematosus(SLE,3.7%),nine of rheumatoid arthritis(RA,2.8%),and 10 of polymyositis(PM,3.1%)in this cohort.Compared to patients with PBC only,the PBC+SS patients were more likely to have fever and elevated erythrocyte sedimentation rate(ESR),higher serum immunoglobulin G(IgG)levels and more frequent rheumatoid factor(RF)and interstitial lung disease(ILD)incidences;PBC+SSc patients had higher frequency of ILD;PBC+SLE patients had lower white blood cell(WBC)count,hemoglobin(Hb),platelet count,γ-glutamyl transpeptidase and immunoglobulin M levels,but higher frequency of renal involvement;PBC+RA patients had lower Hb,higher serum IgG,alkaline phosphatase,faster ESR and a higher ratio of RF positivity;PBC+PM patients had higher WBC count and a tendency towards myocardial involvement.CONCLUSION:Besides the common liver manifestation of PBC,systemic involvement and overlaps with other CTDs are not infrequent in Chinese patients.When overlapping with other CTDs,PBC patients manifested some special clinical and laboratory features which may have effect on the prognosis.
文摘Interstitial lung disease(ILD) is an important mani-festation of collagen vascular diseases. It is a common feature of scleroderma, and also occurs in dermatomyositis and polymyositis, mixed connective tissue disease, Sjogren's syndrome, rheumatoid arthritis, systemic lupus erythematosus, and Antineutrophil cytoplasmic antibody-associated vasculitis. When present, it is associated with increased morbidity and mortality, thus making early diagnosis important. In fact, in many patients, ILD may be the first manifestation of a collagen vascular disease. The most common symptoms are cough and dyspnea. The diagnosis is made based on pulmonary function tests showing restrictive lung disease and impaired oxygen diffusion and chest imaging showing ground glass infiltrates, interstitial thickening, and/or fibrosis. The most common histologic finding on lung biopsy is non-specific interstitial pneumonia, though organizing pneumonia and usual interstitial pneumonia may also be seen. Treatment is focused on addressing the underlying collagen vascular disease with immunosuppression, either with corticosteroids or a steroid-sparing agent such as cyclophosphamide, azathioprine, or mycophenolate, although the optimal agent and duration of therapy is not known. There are few clinical trials to guide therapy that focus specifically on the progression of ILD. The exception is in the case of scleroderma-associated ILD, where cyclophosphamide has been shown to be effective.
基金Supported by The Chonnam Natinal University Hospital Biomedical Research Institute(CRI 11076-21 and 13906-22)Forest Science and Technology Projects,No.S121313L050100provided by Korea Forest Service
文摘Because inflammation plays a key role in the pathogenesis of dry eye disease and Sjogren's syndrome, topical anti-inflammatory agents such as corticosteroids and cyclosporine A have been used to treat inflammation of the ocular surface and lacrimal gland. Systemic biological agents that target specific immune molecules or cells such as tumor necrosis factor(TNF)-α, interferone-α, interleukin(IL)-1, IL-6, or B cells have been used in an attempt to treat Sjogren's syndrome. However, the efficacy of systemic biological agents, other than B-cell targeting agents, has not yet been confirmed in Sjogren's syndrome. Several studies have recently evaluated the efficacy of topical administration of biological agents targeting cytokines in the treatment of dry eye disease. Topical blockade of IL-1 by using IL-1 receptor antagonist could ameliorate clinical signs and inflammation of experimental dry eye. Using a mouse model of desiccating stress-induced dry eye, we have demonstrated that topical application of a TNF-α blocking agent, infliximab, could improve tear production and ocular surface irregularity, decrease inflammatory cytokines and Th-1 CD4+ cells on the ocular surface, and increase gobletcell density in the conjunctiva. Although controversy still remains, the use of topical biological agents targeting inflammatory cytokines may be a promising therapy for human dry eye disease.
基金Supported by National Natural Science Foundation of China(No.81473604)Natural Science Foundation of Zhejiang Province,China(No.LY19H270013)
文摘Objective: To explore the change of intestinal microecology in patients with primary Sjogren’s syndrome(p SS) and correlation with disease activity, and also discuss the therapy effect of Yangyin Yiqi Huoxue Recipe(养阴益气活血方, YYHD). Methods: Sixteen p SS patients were enrolled in the present study, who received 3-month treatment of YYHR, 200 mL orally twice daily. Their pre-and post-test ESSDAI scores, erythrocyte sedimentation rate(ESR) and serum immunoglobulin G(IgG) levels were measured respectively. The 16 Sr DNA metagenomic sequencing was used to detect and analyze the abundance and diversity of intestinal bacteria flora and the proportion of bacteria at the levels of phylum, family, and genus, in comparision with those of 6 healthy subjects in the control group. Results: The abundance and diversity of intestinal bacteria flora in pSS patients were lower than those of healthy subjects(P<0.05). After the treatment with YYHD, patients’ ESSDAI score and levels of IgG and ESR have decreased significantly(P<0.05). At the phylum level, the proportions of Actinobacteria, Firmicutes, Fusobacteria and Proteobacteria have reduced sharply, while the proportions of Bacteroidetes, Teneriquetes and Candidate-division-TM7 have increased significantly by treatment(all P<0.05). At the classification level, such treatment has caused a significant decrease in the proportions of Bacteroidaceae, Ruminococcaceae, Veillonellaceae, and Enterobacteriacea(all P<0.05), but a significant increase in the proportion of Lachnospiraceae(P<0.05). At the genus level, the treatment has significantly decreased the proportions of Bifidobacterium, Bacteroides, Escherichia-Shigella, Faecalibacterium and Prevotella(all P<0.05), but significantly increased the proportion of Clostridia(P<0.05), close to the levels of healthy subjects(P>0.05). Conclusions: There exists an imbalance of intestinal microecology in p SS patients, which can be improved through the treatment with YYHD. Besides, such treatment can also improve the disease activity and adjust the diversity of intestinal bacteria flora, the composition and the abundance of intestinal flora.
文摘Objective： To observe the effect of Chinese herbal medicine for nourishing yin, supplementing qi, and activating blood on the expression of interferen- γ （IFN-γ,）/interleukin-4 （IL-4） in peripheral blood and disease activity in primary Sjogren＇s syndrome （pSS） patients, and to study the relationship between the immune balance of Th1/Th2 and the disease activity. Methods： A total of 66 pSS patients were randomized with tossing coins method into two groups： the integrative therapy group （34 cases） and the control group （32 cases）; and 28 healthy subjects were taken as the normal group. The integrative therapy group was treated by Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood combined with hydroxychloroquine sulfate tablets and the control group was treated with hydroxychloroquine sulfate tablets. The treatment course was 3 months for both groups. The levels of serum immunoglobulin G （IgG）, erythrocyte sedimentation rate （ESR）, IFN-γand IL-4 in peripheral blood were measured before and after treatment. Results： Compared with the normal group, the levels of IgG, ESR, IFN- γ and IL-4 were significantly increased in pSS patients （P〈0.05）. Remarkably, after 3 months of treatment, these levels were dramatically decreased in both the integrative therapy group and the control group, although still higher than the normal group. The levels of IgG, ESR, IFN- and IL-4 in the integrative therapy group were lower than the control group and the same group before treatment （P〈0.05）. The ratio of IFN-γ/IL-4 also significantly decreased after treatment. Moreover, the level of IFN- γ, and the ratio of IFN- γ/IL-4 in the integrative theraphy group were significantly lower than the control group （P〈0.05）. For all patients the ratio of IFN-γ/IL-4 before and after treatment was positive correlated with the levels of IgG and ESR. Conclusion： Chinese herbal medicine for nourishing yin, supplementing qi, and activating blood can alleviate the disease activity of pSS by regulating the immune balance of Th1/Th2.
基金Supported by the National Natural Science Foundation of China(No.81473604)
文摘ABSTRACT Objective： To investigate the effect of Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood on the reproductive endocrine-immune network and its mechanisms in patients with primary Sjogren＇s syndrome （pSS）. Methods： Seventy pSS patients were randomly assigned to two groups using a randomized digital table： the integrative therapy group （36 cases） and the control group （34 cases）. Thirty healthy subjects were taken as a normal group. The control group was treated with hydroxychloroquine sulfate tablets alone, and the integrative therapy group was treated by Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood combined with hydroxychloroquine sulfate tablets. The treatment course was 6 months for both groups. Before and after treatment, serum estradiol （E2）, testosterone （T）, luteinizing hormone （LH）, prolactin （PRL） by radioimmunoassay and immunoglobulin （IgG） by immunodiffusion, erythrocyte sedimentation rate （ESR） by Westergren, interferon-γ （IFN-γ） and interleukin-4 （IL-4） by enzyme linked immunosorbent assay were determined. Results： E2 and T levels in all patients were lower than those of normal subjects before treatment （P〈0.05） and were increased significantly after 6-month treatment （P〈0.05）. ESR, FSH, LH, IgG, IFN - γ, IL - 4 and ratios of E2/T, and IFN --γ/IL in the patients were higher than those of normal subjects before the treatments （P〈0.05）, and were reduced significantly after the treatments （P〈0.05）. The T and IFN -γ levels and E2/T ratio in the patients treated with integrative therapy were reduced significantly compared with the control group （P〈0.05）. However, the PRL levels before and after treatment were not significantly changed in the two groups （P〉0.05）. The ratios of E2/T and IFN -γ/IL-4, and levels of IgG and ESR were positively correlated before and after treatment （P〈0.05）. Conclusions： The ratios of E2/T and IFN - γ/ IL-4 might be used as indicators of pSS activity. Chinese herbal medicines for nourishing yin, supplementing qi, and activating blood combined with Western medicine could improve the therapeutic effect by regulating the reproductive endocrine-immune network in pSS patients.
基金supported by the National Science and Technology Major Project of the Ministry of Science and Technology of China(No.2018ZX09721004-009-002)Xinglin Scholar Research Promotion Project of Chengdu University of TCM(No.BSH2018006)。
文摘Primary Sjogren’s syndrome(p SS)is a chronic autoimmune disease involving exocrine glands.Current studies have found that the occurrence of the disease is closely related to genetic,environmental and neuroendocrine factors,as well as abnormal activation of T and B lymphocytes.The etiology and pathogenesis of p SS is complex,and there is a lack of specific targeted drugs.Traditional Chinese medicines(TCMs)have been comprehensively investigated for their treatment effects on p SS.Through a systematic review of the literature,we summarized the TCMs used to treat p SS,and find that there are four major ways that TCMs are used,including upregulation of aquaporin proteins,suppression of cell apoptosis,suppression of the abnormal activation of B lymphocytes and suppression of the abnormal activation of T lymphocytes(balancing T helper type[Th]1/Th 2& Th17/Treg and suppressing follicular helper T[Tfh]cells).However,there are not enough data about the active constituents,quality control,pharmacokinetics,toxicity and modern preparations of these TCMs;therefore,more investigations are needed.This paper highlights the importance of TCMs for treating p SS and provides guidance for future investigations.
