BACKGROUND Lupus mesenteric vasculitis(LMV)is a serious condition that may occur as an acute manifestation of gastrointestinal(GI)involvement and is not easily diagnosed by physicians.Delayed diagnosis and treatment o...BACKGROUND Lupus mesenteric vasculitis(LMV)is a serious condition that may occur as an acute manifestation of gastrointestinal(GI)involvement and is not easily diagnosed by physicians.Delayed diagnosis and treatment of LMV may lead to rapid disease progression and can be life threatening.CASE SUMMARY A previously healthy 27-year-old woman presented with abdominal pain following a history of fatigue and consumption of cold water.Laboratory invest-igations,physical examinations,and enhanced abdominal computed tomography(CT)suggested systemic lupus erythematosus complicated by LMV.She received treatments,such as GI decompression,somatostatin,glucocorticoids,and immu-nosuppressants,and was evaluated using color ultrasonography.Twenty days later,the patient reported no stomach discomfort and was able to consume semi-liquid food.Laboratory investigations showed that inflammatory factors decreased to normal levels and complement levels increased slightly.One year after discharged,she recovered with methylprednisolone being tapered to 4 mg per day,mycophenolate mofetil to 0.75 g bid,and hydroxychloroquine to 0.2 g bid;however,only C3 complement level was slightly below the normal level.CONCLUSION Early diagnosis of LMV is essential for successful treatment;this depends on a combination of clinical manifestations,laboratory investigations,and imaging findings.Enhanced CT is preferred,but ultrasonography can be used for prompt screening and follow-up.展开更多
Objective:Although relatively rare,adult immunoglobulin A vasculitis(IgAV)can lead to severe complications and longer hospitalization,and result in poor prognosis,when compared to childhood IgAV.Hence,early identifica...Objective:Although relatively rare,adult immunoglobulin A vasculitis(IgAV)can lead to severe complications and longer hospitalization,and result in poor prognosis,when compared to childhood IgAV.Hence,early identification and prevention for patients prone to develop systemic involvement are essential.The purpose of this study was to explore the correlations of common serological markers with the development of systemic involvement in adult IgAV.Methods:A retrospective analysis was performed for adult IgAV patients,who were hospitalized in Wuhan Union Hospital between January 2016 and December 2019.A total of 259 patients were enrolled,and the pre-treatment serological markers were comprehensively assessed.Results:In the present study,49.0% and 33.2% of patients developed renal and gastrointestinal(GI)involvement,respectively.Furthermore,the elevated levels of white blood cells count,D-Dimer(D-D),C-reactive protein(CRP)and neutrophil granulocyte ratio(NE%)>60% were significantly associated with GI involvement in the univariate analysis,while the decrease in high density lipoprotein level,and the elevated D-D and CRP levels were significantly associated with renal involvement(P<0.05).Moreover,a prediction model that combined multiple markers was established by performing a logistic regression analysis,and this presented a more favorable value of prediction than the individual serological markers.Conclusion:The present study suggests that common serological markers have close correlations with systemic involvement in adult IgAV,and that the establishment of a prediction model for systemic involvement may be helpful in facilitating personalized therapeutic strategies and clinical management for IgAV patients.展开更多
Mastocytosis is a rare and heterogeneous disease characterized by various clinical and biological features that affect different prognoses and treatments.The disease is usually divided into 2 principal categories:cuta...Mastocytosis is a rare and heterogeneous disease characterized by various clinical and biological features that affect different prognoses and treatments.The disease is usually divided into 2 principal categories:cutaneous and systemic disease(SM).Clinical features can be related to mast cell(MC)mediator release or pathological MC infiltration.SM is a disease often hard to identify,and the diagnosis is based on clinical,biological,histological,and molecular criteria with different specialists involved in the patient’s clinical work-up.Among all manifestations of the disease,gastrointestinal(GI)symptoms are common,being present in 14%-85% of patients,and can significantly impair the quality of life.Here we review the data regarding GI involvement in SM,in terms of clinical presentations,histological and endoscopic features,the pathogenesis of GI symptoms,and their treatment.展开更多
BACKGROUND Gastrointestinal involvement in Behçet's disease(GIBD)and Crohn’s disease(CD)are inflammatory diseases sharing a considerable number of similarities.However,different from CD,the operative and pos...BACKGROUND Gastrointestinal involvement in Behçet's disease(GIBD)and Crohn’s disease(CD)are inflammatory diseases sharing a considerable number of similarities.However,different from CD,the operative and postoperative management of GIBD remains largely empirical because of the lack of comprehensive treatment guidelines.AIM To compare surgical patients with GIBD and those with CD in a medical center and identify notable clinical features and effective postoperative treatment for surgical patients with GIBD.METHODS We searched patients diagnosed with CD and GIBD who underwent operations for gastrointestinal complications from 2009 to 2015 at West China Hospital of Sichuan University.A total of 10 surgical patients with GIBD and 106 surgical patients with CD were recruited.Information including demographic data,medication,and operative and postoperative parameters were collected and analyzed.As the incidence of surgical GIBD is low,their detailed medical records were reviewed and compared to previous studies.Moreover,the prognoses of CD and GIBD were evaluated respectively between groups treated with biological and non-biological agents.RESULTS Indication for first surgery was often acute intestinal perforation for GIBDpatients (7/10 vs 0/106, P < 0.001), whereas intestinal fistulae (0/10 vs 44/106, P =0.013) and ileus (0/10 vs 40/106, P = 0.015) were the indications for surgical CDpatients. Approximately 40% of patients with GIBD and 23.6% of patients withCD developed postoperative complications, 50% of patients with GIBD and 38.7%of patients with CD had recurrence postoperatively, and 40% (4/10) of patientswith GIBD and 26.4% (28/106) of patients with CD underwent reoperations. Theaverage period of postoperative recurrence was 7.87 mo in patients with Behçet'sdisease (BD) and 10.43 mo in patients with CD, whereas the mean duration fromfirst surgery to reoperation was 5.75 mo in BD patients and 18.04 mo in CDpatients. Surgical patients with GIBD more often used corticosteroids (6/10 vs7/106, P < 0.001) and thalidomide (7/10 vs 9/106, P < 0.001) postoperatively,whereas surgical patients with CD often used infliximab (27/106), azathioprine,or 6-mercaptopurine (74/106) for maintenance therapy.CONCLUSIONPatients suffering GIBD require surgery mostly under emergency situations,which may be more susceptible to recurrence and reoperation and need moreaggressive postoperative treatment than patients with CD.展开更多
文摘BACKGROUND Lupus mesenteric vasculitis(LMV)is a serious condition that may occur as an acute manifestation of gastrointestinal(GI)involvement and is not easily diagnosed by physicians.Delayed diagnosis and treatment of LMV may lead to rapid disease progression and can be life threatening.CASE SUMMARY A previously healthy 27-year-old woman presented with abdominal pain following a history of fatigue and consumption of cold water.Laboratory invest-igations,physical examinations,and enhanced abdominal computed tomography(CT)suggested systemic lupus erythematosus complicated by LMV.She received treatments,such as GI decompression,somatostatin,glucocorticoids,and immu-nosuppressants,and was evaluated using color ultrasonography.Twenty days later,the patient reported no stomach discomfort and was able to consume semi-liquid food.Laboratory investigations showed that inflammatory factors decreased to normal levels and complement levels increased slightly.One year after discharged,she recovered with methylprednisolone being tapered to 4 mg per day,mycophenolate mofetil to 0.75 g bid,and hydroxychloroquine to 0.2 g bid;however,only C3 complement level was slightly below the normal level.CONCLUSION Early diagnosis of LMV is essential for successful treatment;this depends on a combination of clinical manifestations,laboratory investigations,and imaging findings.Enhanced CT is preferred,but ultrasonography can be used for prompt screening and follow-up.
基金supported by the National Natural Science Foundation of China(No.81772913).
文摘Objective:Although relatively rare,adult immunoglobulin A vasculitis(IgAV)can lead to severe complications and longer hospitalization,and result in poor prognosis,when compared to childhood IgAV.Hence,early identification and prevention for patients prone to develop systemic involvement are essential.The purpose of this study was to explore the correlations of common serological markers with the development of systemic involvement in adult IgAV.Methods:A retrospective analysis was performed for adult IgAV patients,who were hospitalized in Wuhan Union Hospital between January 2016 and December 2019.A total of 259 patients were enrolled,and the pre-treatment serological markers were comprehensively assessed.Results:In the present study,49.0% and 33.2% of patients developed renal and gastrointestinal(GI)involvement,respectively.Furthermore,the elevated levels of white blood cells count,D-Dimer(D-D),C-reactive protein(CRP)and neutrophil granulocyte ratio(NE%)>60% were significantly associated with GI involvement in the univariate analysis,while the decrease in high density lipoprotein level,and the elevated D-D and CRP levels were significantly associated with renal involvement(P<0.05).Moreover,a prediction model that combined multiple markers was established by performing a logistic regression analysis,and this presented a more favorable value of prediction than the individual serological markers.Conclusion:The present study suggests that common serological markers have close correlations with systemic involvement in adult IgAV,and that the establishment of a prediction model for systemic involvement may be helpful in facilitating personalized therapeutic strategies and clinical management for IgAV patients.
文摘Mastocytosis is a rare and heterogeneous disease characterized by various clinical and biological features that affect different prognoses and treatments.The disease is usually divided into 2 principal categories:cutaneous and systemic disease(SM).Clinical features can be related to mast cell(MC)mediator release or pathological MC infiltration.SM is a disease often hard to identify,and the diagnosis is based on clinical,biological,histological,and molecular criteria with different specialists involved in the patient’s clinical work-up.Among all manifestations of the disease,gastrointestinal(GI)symptoms are common,being present in 14%-85% of patients,and can significantly impair the quality of life.Here we review the data regarding GI involvement in SM,in terms of clinical presentations,histological and endoscopic features,the pathogenesis of GI symptoms,and their treatment.
基金National Natural Science Foundation of China,No.81270447.
文摘BACKGROUND Gastrointestinal involvement in Behçet's disease(GIBD)and Crohn’s disease(CD)are inflammatory diseases sharing a considerable number of similarities.However,different from CD,the operative and postoperative management of GIBD remains largely empirical because of the lack of comprehensive treatment guidelines.AIM To compare surgical patients with GIBD and those with CD in a medical center and identify notable clinical features and effective postoperative treatment for surgical patients with GIBD.METHODS We searched patients diagnosed with CD and GIBD who underwent operations for gastrointestinal complications from 2009 to 2015 at West China Hospital of Sichuan University.A total of 10 surgical patients with GIBD and 106 surgical patients with CD were recruited.Information including demographic data,medication,and operative and postoperative parameters were collected and analyzed.As the incidence of surgical GIBD is low,their detailed medical records were reviewed and compared to previous studies.Moreover,the prognoses of CD and GIBD were evaluated respectively between groups treated with biological and non-biological agents.RESULTS Indication for first surgery was often acute intestinal perforation for GIBDpatients (7/10 vs 0/106, P < 0.001), whereas intestinal fistulae (0/10 vs 44/106, P =0.013) and ileus (0/10 vs 40/106, P = 0.015) were the indications for surgical CDpatients. Approximately 40% of patients with GIBD and 23.6% of patients withCD developed postoperative complications, 50% of patients with GIBD and 38.7%of patients with CD had recurrence postoperatively, and 40% (4/10) of patientswith GIBD and 26.4% (28/106) of patients with CD underwent reoperations. Theaverage period of postoperative recurrence was 7.87 mo in patients with Behçet'sdisease (BD) and 10.43 mo in patients with CD, whereas the mean duration fromfirst surgery to reoperation was 5.75 mo in BD patients and 18.04 mo in CDpatients. Surgical patients with GIBD more often used corticosteroids (6/10 vs7/106, P < 0.001) and thalidomide (7/10 vs 9/106, P < 0.001) postoperatively,whereas surgical patients with CD often used infliximab (27/106), azathioprine,or 6-mercaptopurine (74/106) for maintenance therapy.CONCLUSIONPatients suffering GIBD require surgery mostly under emergency situations,which may be more susceptible to recurrence and reoperation and need moreaggressive postoperative treatment than patients with CD.