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Objective:To investigate the clinical features of patients with coexisting connective tissue disease (CTD) and sarcoisosis and to avoid misdiagnosis and mistreatment.Methods:To analyze the clinical manifestations, laboratory data, imaging and pathological features of patients with CTD combined with sarcoidosis in Peking Union Medical College Hospital from January 1985 to December 2021.Results:There were 17 patients with CTD(including 10 SS, 2 DM, 2 PBC, 1 SLE, 1 RA and 1 UCTD), combined with sarcoidosis, with a mean age of (55±10) years old and the ratio of male-to-female was 1:16. Eight patients were diagnosed as CTD before sarcoidosis, while 3 patients after sarcoidosis. The other 6 patients were diagnosed with the two diseases almost simultaneously. Lymphadenopathy(12/17), pulmonary nodules (8/17), subcutaneous nodules (4/17), rash (4/17) and blurred vision (1/17) were the main manifestations of patients with the onset of nodular disease. Nine patients were treated based on the presentation of sardoisis and 5 patients for CTD; 3 patients were treated for both diseases at the same time. All 17 patients discharged with improvement after treatment.Conclusion:When sarcoidosis do coexists with CTD, occult CTD might occur. It is important to investigate specific manifestations including pathological features of sarcoidosis and differentiate it from CTD.
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Objective:To analyze the risk factors of patients with ankylosing spondylitis (AS) combined with premature coronary atherosclerotic heart disease (PCAD).Methods:A total of 74 patients with AS and coronary atherosclerotic heart disease (CAD) in Peking Union Medical College Hospital from January 1983 to July 2021 were enrolled. According to the age of onset of coronary heart disease, the 74 patients were divided into PCAD group and NPCAD (non-premature coronary heart disease) group. T test and Chi square test were used to analyze the data of the two groups, the risk factors for AS-PCAD were analyzed by multivariate Logistic regression. Results:① There were 37 cases in the PCAD group and 37 cases in the NPCAD group. In the PCAD group, there were 28 men and 9 women; wherease all were men in the NPCAD group. The difference was statistically significant ( χ2=10.25, P=0.001). ② Compared with the NPCAD group, the age of AS-PCAD group was younger [(23±10) years vs (29±12) years, t=-2.28, P=0.026], and the course from AS to CAD was shorter [(25±10) years vs (34±13) years, t=-3.00, P=0.004], hemoglobin (Hb) level was lower [(122±23) g/L vs(132±18) g/L, t=2.10, P=0.039], rate of anemia was higher [38.5%(14/37) vs 16.2%(6/37), χ2=4.39, P=0.037]. Proportion of increased C-reactive protein (CRP) was higher [65.5%(19/29) vs 35.5%(11/31), χ2=5.41, P=0.019]. ③ Juvenile onset AS (JoAS)[ OR(95% CI)=3.45(1.31, 9.10), P=0.012] and high levels of CRP [ OR (95% CI)=3.68 (1.44, 9.40), P=0.006] might berisk factors of AS-PCAD by multiple logisctic regression analysis. Conclusion:Patients with AS have a higher probability of PCAD, especially in those patients with JoAS, persistent inflammation and anemia. It is necessary to be alert to the risk of PCAD and early screening.
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Objective:To describe a series of systemic sclerosis (SSc) patients with the combination of scleroderma renal crisis (SRC) and pulmonary arterial hypertension (PAH).Methods:The medical records of 472 SSc patients in Peking Union Medical College Hospital between January 2012 and October 2020 were reviewed and a retrospective analysis of the characteristics of patients with SRC and PAH among SSc patients was conducted.Results:Thirteen patients suffered from SRC and PAH in the SSc patients, 1 case was limited cutaneous SSc, and 12 cases were diffuse cutaneous SSc. Five patients had renal crisis before pulmonary arterial hypertension, 4 patients had pulmonary arterial hypertension before the occurrence of renal crisis, and the remaining 4 patients were found at the same time. Among them, 11 patients had Raynaud's phenomenon, 7 had gastrointestinal bleeding, 6 had pulmonary edema and 3 had telangiectasias. Twelve cases were positive for anti-nuclear antibodies and 4 cases were positive for anti-Scl-70 antibodies. N-terminal pro-brain natriuretic peptide (NT-proBNP)>1 400 ng/L in 11 patients. Two patients had thrombotic microangiopathy (TMA). Among the 13 patients, 3 patients died during hospitalization, 2 patients were lost to follow-up, and 2 patients died within 5 years of follow-up. Six patients survived, and 1 of the 4 patients with regular dialysis were discharged from dialysis.Conclusion:In patients with scleroderma, SRC can occur earlier, later than, or at the same time with SSc-PAH. Patients may have a higher incidence of gastrointestinal bleeding and higher level of NT-proBNP. PDE5i or ERAs may be beneficial.
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BACKGROUND@#Perioperative treatment has become an increasingly important aspect of the management of patients with non-small cell lung cancer (NSCLC). Small-scale clinical studies performed in recent years have shown improvements in the major pathological remission rate after neoadjuvant therapy, suggesting that it will soon become an important part of NSCLC treatment. Nevertheless, neoadjuvant immunotherapy may be accompanied by serious adverse reactions that lead to delay or cancelation of surgery, additional illness, and even death, and have therefore attracted much attention. The purpose of the clinical recommendations is to form a diagnosis and treatment plan suitable for the current domestic medical situation for the immune-related adverse event (irAE).@*METHODS@#This recommendation is composed of experts in thoracic surgery, oncologists, thoracic medicine and irAE related departments (gastroenterology, respirology, cardiology, infectious medicine, hematology, endocrinology, rheumatology, neurology, dermatology, emergency section) to jointly complete the formulation. Experts make full reference to the irAE guidelines, large-scale clinical research data published by thoracic surgery, and the clinical experience of domestic doctors and publicly published cases, and repeated discussions in multiple disciplines to form this recommendation for perioperative irAE.@*RESULTS@#This clinical recommendation covers the whole process of prevention, evaluation, examination, treatment and monitoring related to irAE, so as to guide the clinical work comprehensively and effectively.@*CONCLUSIONS@#Perioperative irAE management is an important part of immune perioperative treatment of lung cancer. With the continuous development of immune perioperative treatment, more research is needed in the future to optimize the diagnosis and treatment of perioperative irAE.
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Primary biliary cholangitis is a chronic autoimmune cholestatic disease with a progressive course. This disease is not rare in China, but standardized diagnosis and treatment for primary biliary cholangitis are insufficient. Based on the evidence and guidelines from China and other countries, Rheumatology Branch of Chinese Medical Association developed the recommendations of diagnosis and treatment for primary biliary cholangitis in China. The aim is to help clinicians recognize clinical characters, therapeutic selection and prognosis judgement of primary biliary cholangitis, which will contribute to make diagnosis in time, to select treatment properly and to manage follow-up scientifically.
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Objective:To investigate the distribution and clinical significance of subtypes of antimitochondrial antibodies (AMA)-M2, M4, M9 in primary biliary cholangitis (PBC).Methods:A total of 1 367 patients were detected with AMA-M2, M4, M9 in Peking Union Medical College Hospital (PUMCH) from Jan 2014 to Dec 2019 and the clinical parameters were collected. The distribution patterns of AMA subtypes in different groups were analyzed and the diagnostic sensitivity and specificity of AMA subtypes in PBC were calculated. Chi-square test was used for statistical analysis.Results:In 1 367 patients, 236 of whom were positive for AMA subtypes. The positivity of AMA subtypes in female was significantly higher than in male (20.34% vs 9.41%, χ2=23.792, P<0.01). In addition, the positivity of AMA subtypes was significantly higher in 30-65 years old patients than in patients younger than 30 years old or older than 65 years old [(20.00%(193/965) vs 10.97%(17/155) vs 10.53%(26/247), χ2=17.209, P<0.01]. 110 patients with positive AMA subtypes were diagnosed with PBC. The diagnostic sensitivity and specificity of AMA-M2 were both desirable [94.64%(106/112) and 92.35%(1 159/1 255)]. Although the specificity of AMA-M4 was as high as 99.12%(1 244/1 255), its sensitivity was very low [15.18%(17/122)]. Combined detection of different AMA subtypes could not improve the diagnostic sensitivity and specificity significantly. Diseases other than PBC can be positive for AMA subtypes, predominantly for AMA-M2. Conclusion:Female and 30-65 years old patients were more frequently positive for AMA subtypes. AMA-M2 was the most valuable AMA subtype for diagnosing PBC.
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Thrombocytopenia is the main clinical manifestation or common complication of multiple diseases, but there is still a lack of systematic understanding of pathogenesis, underlying diseases and treatment strategies of thrombocytopenia. Based on evidence-based medicine, this consensus summarizes seven aspects related to thrombocytopenia, including definition, epidemiology, pathogenesis, clinical manifestations, laboratory examination, diagnosis and treatment. This consensus provides an important reference for the diagnosis and treatment of thrombocytopenia.
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Objective:To explore the efficacy and safety of anti-tumor necrosis factor alpha (TNFα) monoclonal antibodies (mAbs) for severe/refractory vasculo-Behcet′s disease (BD).Method:The clinical data of severe/refractory vasculo-BD patients treated with anti-TNFα mAbs were retrospectively analyzed. Response of anti TNFα mAbs was analyzed. The dosage changes of glucocorticoid, the level of erythrocyte sedimentation rate (ESR) and hypersensitive C-reactive protein (hsCRP) before and after treatment were recorded, as well as side effects.Result:Sixteen patients were enrolled. Arterial lesions were reported in 12 patients, including 9 with arterial aneurysm, 6 with arterial dilation, 2 with stenosis and 2 with occlusion. Seven patients presented venous thrombosis, including lower extremity veins ( n=6), cerebral venous sinus ( n=2) and inferior vena cava system ( n=2). Two cases had both arterial and venous involvement. Before the application of TNFα mAbs, all 16 patients failed to response to prednisone or its equivalent dose of 40 (7.5-90) mg/d in combination with cyclophosphamide, methotrexate, thalidomide or azathioprine for median 4 (0-156) months. After a mean duration of treatment for (17.1±6.5) months, 15 patients achieved complete remission and 1 patient achieved partial remission. Three patients received surgery without any postoperative complications. After using anti TNFα mAbs, the dosage of prednisone [5(0-12.5)mg/d vs. 40(7.5-90)mg/d, P<0.01], ESR [(7.3±4.6) mm/1h vs. (33.5±26.7) mm/1h, P<0.01] and hsCRP [1.9(0.2-11.4) mg/L vs. 24.3(0.4-113.9) mg/L, P<0.01] were significantly decreased. Side effects were observed in 2 patients. One developed pulmonary infection 12 months after adalimumab with conventional treatment. Another patient had allergy to infliximab then switched to adalimumab. Conclusion:In combination with corticosteroids and immunosuppressants, anti-TNF α mAbs are effective and well-tolerated in severe/refractory vasculo-BD, with a favorable steroid -sparing effect and rare postoperative complications.
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To analyze the perceptions of cardiovascular specialists about Behcet′s disease and its cardiovascular lesions in Beijing Anzhen Hospital, Capital Medical University. A survey using questionnaires was conducted among cardiovascular specialists in the hospital, the differences among groups were analyzed with χ 2 analyses. Less than half of the cardiovascular specialists were familiar with the diagnostic criteria of Behcet′s disease (32.6%), and its skin lesions, as acne-like rash (41.3%), erythema nodosum (42.0%), acupuncture response (47.8%). The knowledge of its cardiovascular lesions was too superficial, especially in the awareness of heart conduction block. Most of the specialists preferred to use low dose of glucocorticoids before operation in their practice. To improve the knowledge and awareness of Behcet′s disease and its cardiovascular lesions is of great significance for the early diagnosis, improvement of prognosis, and reduction of postoperative complications.
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Objective:To observe the efficacy of mycophenolate mofetil (MMF) combined with ursodeoxycholic acid (UDCA) for the treatment of primary biliary cholangitis (PBC) with incomplete response to UDCA monotherapy.Methods:This is an open label study. Combination therapy of MMF (0.75-1.5 g/d) and UDCA (13-15 mg·kg -1·d -1) were applied to PBC patients with incomplete response to UDCA alone according to Barcelona Criteria. The expected observation duration of treatment were at least 12 months. The levels of serum alanine amiotransferase (ALT), aspartate transaminase (AST), total bilirubin (TBil), albumin (Alb), alkaline phosphatase (ALP), γ-glutamyltranspeptidase (GGT), immunoglobulin (Ig)M, IgG, IgA were mea-sured at different time points before and after treatment. The biochemical response rates were evaluated with reference to formerly reported criteria including Barcelona criteria and so on. Paired student's t-test and wilcoxon matched-pairs signed rank test were used to compare differences between groups before and after treatment. Mann-Whitney U test was used to compare differences between the two independent groups with abnormal distribution. A two-sided P<0.05 was considered statistically significant. Results:Seventeen of 29 patients included were treated with combination therapy for at least 1 year. The levels of serum ALP were significantly decreased after 3 months [(369±184) U/L vs (309±148) U/L, t=2.149, P=0.045] but not afterwards. GGT levels were significantly decreased at 6 months [266.5(205.5, 414.0) U/L vs 217.5(173.8, 391.8) U/L, Z=-2.334, P=0.018] and the significance continued to 1 year. IgM, IgG and IgA levels had a significant decrease at 3, 6 and 12 months but not AST, Tbil or Alb levels. The biochemical response rates were between 9.5%-23.5%. Conclusion:Overall, the biochemical response rate of combination therapy of MMF and UDCA is low in incomplete responders to UDCA alone. Early recognition of incomplete responders is of great im-portance and high quality researches are needed to confirm the effectiveness of the combined therapies.
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Objective:To study the patterns of tocilizumab (TCZ) use, its efficacy and safety in patients with rheumatoid arthritis (RA) in routine clinical practice.Methods:A total of 407 patients with RA were enrolled from 23 centers and treated with TCZ within 8 weeks prior to the enrollment visit, and were followed for 6-month. The patterns of TCZ treatment at 6 months, the effectiveness and safety outcomes were recorded. Statistical analysis was performed using SAS version 9.4.Results:A total of 396 patients were included for analysis, in which 330 (83.3%) patients received TCZ combined with conventional synthetic disease-modifying antirheumatic drugs (csDMARDs), and 16.7%(66/396) received TCZ monotherapy. At baseline, TCZ was initiated in 56.6%(224/396) and 9.6%(38/396) of patients after failure of DMARDs and other biological agents (bDMARDs) respectively. During the 6-month follow-up period, the mean frequency of TCZ administration was (3.7±1.6), the mean TCZ dosage was (7.4±1.2) mg/kg, and the mean interval between doses was (40±13) days. 120(25.8%) patients were on TCZ treatment at the end of the study. Improvements in disease activity, systemic symptoms and patient report outcomes were observed at the end of the study. 22.7%(90/396) patients experienced at least one treatment related adverse event, and 8 patients experienced at least one serious adverse event.Conclusion:This study demonstrates that TCZ treatment is effective in patients with RA when being treated for 6 months with an acceptable safety profile. The duration of TCZ treatment needs to be extended.
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Objective:The aim of this study was to compare the efficacy and safety of iguratimod (IGU) or leflnomide (LEF) in combination with methotrexate (MTX) in patients with active rheumatoid arthritis (RA).Methods:This was a multicenter, randomized, double-blinded, double dummy and controlled clinical trial. Patients with moderate or high active RA were randomized in a 1∶1 ratio to receive IGU+MTX (Group A) or LEF+MTX (Group B) treatment. The efficacy and safety were assessed at week 12, 24 and 52, respectively. The primary endpoint was the American Colleague of Rheumatology 20 (ACR20) response rates at the 52th week. Pearson chi square test and two-way Analysis of Variance (ANOVA) were used to compare the improve- ment of ACR20 and DAS28 at 52 weeks. Pearson chi square test or Fisher exact probability test were used to compare the ACR 20 and ACR70 rate between the two groups after treatment. The measurement data of the two groups were compared by independent sample t-test or nonparametric test. Results:A total of 240 RA patients were enrolled in the present study. As a result, 84.1% and 81.0% of patients achieved ACR20 criteria at the 52th week in Group A and Group B, respectively ( χ2=0.35, P=0.56). And the ACR50/70 response rates, disease activity score 28 (DAS28), simplified disease activity index (SDAI) and the absolute decrease of DAS28 from baseline were not statistically different between the two groups at week 12, 24 and 52. The rates of adverse events were lower in Group A than those in Group B (60.0% vs 79.0%, P<0.01). The elevations of glutamic pyruvic transaminase/glutamic oxalacetic transaminase levels, concomitant use of hepatinica and white blood cell decrease were more common in Group B ( P<0.05). Conclusion:IGU in combination with MTX is an efficacious and safe treatment regimen, which is comparable in efficacy in control active RA but superior in safety to LEF combined with MTX.
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Objective:To discuss the clinical features and treatment of 19 patients with granulomatosis with polyangiitis (GPA) complicated with hypertrophic cranial pachymeningitis (HCP).Methods:The clinical features of 19 patients diagnosed with GPA complicated with HCP in Peking Union Medical College Hospital were retrospectively analyzed.Results:Among the 315 patients with GPA, 19 (12 males, 7 females, with) were diagnosed with HCP at Peking Union Medical College Hospital. The median age was 57 (19-64) years. In the neurological manifestations per se, all patients had headache, 16 patients had cerebral involvement, which included 8 cases at the frontal area, 8 cases at the temporal area, 8 cases at the skull base area (4 cases with parasellar involvements including 3 cases with cavernous sinus involvement and 2 cases with orbital involvement), 6 cases of tentorium involvement, 2 cases of cerebral palsy, 1 case of calvarium, 1 case of occipital, and 1 case with combined spinal pachymeningitis, respectively. In systemic manifestations, 10 patients had fever, 8 patients had weight lose, 4 patients had lung involvement, 3 patients had kidney involvement, 16 patients had nasosinusitis, 10 patients had tympanitis, and 16 patients had localized GPA. The laboratory tests showed that 15 patients had positive anti-neutrophil cytoplasmic antibodies (ANCA), including 8 cases with positive proteinase 3 (PR3)-ANCA and 6 cases with positive myeloperoxidase (MPO)-ANCA. Sixteen patients had lumbar puncture examination, 9 cases had elevated cerebrospinal pressure, 10 cases had elevated level of protein in cerebrospinal fluid. Nineteen patients were treated with glucocorticoids (12 patients accepted pulse therapy) and immunosuppressive agents. Twelve patients were treated with intrathecal injections of dexamethasone combined with or without Methotrexate (MTX). All 19 patients were improved.Conclusion:HCP, as a rare but serious manifestation of GPA, is not rare in active cases and should be intensively treated.
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Objective:To investigate the relationship of Castleman disease (CD) and connective tissue disease (CTD).Methods:Clinical records and laboratory data of 11 patientsdiagnosed with CD and CTD were collected and retrospectively analyzed. All patients were diagnosed at Peking Union Medical College Hospital.Results:① The proportion of CD associated with or mimicking CTD was 5.67% (11/194) in all CD patients during the same period. The average age of these cases at the diagnosis was (51±17) years and the ratio of male to female was 6∶5. ② Lymphadenopathy (10/11), fever (8/11), serosal effusion (6/11), arthralgia (5/11), alopecia (2/11), Raynaud phenomenon (1/11) and photosensitivity (1/11) were the common clinical manifest- ations that could mimic CTD. ③ Elevated ESR (11/11), hypoalbuminemia (11/11), elevated CRP (10/11), elevated IgG (7/11), proteinuria (5/11), hematuria (5/11) and positive ANA(5/11) were commonly found in the patients' laboratory tests. ④ CD was inclined to mimic systemic lupus erythematosus(SLE)(5/11), IgG4-related disease(IgG4-RD)(2/11) and adult onset Still's disease(AOSD)(2/11), as well as 2 cases were associated with Sj?gren's Syndrome(SS)(2/11). ⑤All cases were ultimately diagnosed as multicentric CD, the pathologic subtypes were plasma cell variant (10/11) and mixed(1/11) respectively.Conclusion:CD maybe overlapped with or mimic a variety of clinical manifestations, such as fever, serosal effusion, arthralgia and proteinuria which could mimic CTD. Early biopsy is helpful for the diagnosis and to avoid misdiagnosis and mistreatment.
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To analyze the perceptions of cardiovascular specialists about Behcet′s disease and its cardiovascular lesions in Beijing Anzhen Hospital, Capital Medical University. A survey using questionnaires was conducted among cardiovascular specialists in the hospital, the differences among groups were analyzed with χ2 analyses. Less than half of the cardiovascular specialists were familiar with the diagnostic criteria of Behcet′s disease (32.6%), and its skin lesions, as acne-like rash (41.3%), erythema nodosum (42.0%), acupuncture response (47.8%). The knowledge of its cardiovascular lesions was too superficial, especially in the awareness of heart conduction block. Most of the specialists preferred to use low dose of glucocorticoids before operation in their practice. To improve the knowledge and awareness of Behcet′s disease and its cardiovascular lesions is of great significance for the early diagnosis, improvement of prognosis, and reduction of postoperative complications.
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Objective@#To investigate the clinical characteristics of polyarteritis nodosa (PAN) patients with renal involvement.@*Methods@#PAN patients admitted to the department of rheumatology, department of pediatrics, department of nephrology, general internal medicine department and department of vascular surgery at Peking Union Medical College Hospital from June 2012 to August 2018 were enrolled in this study and were divided into two groups according to renal involvement or not. The clinical characteristics were analyzed.@*Results@#A total of 94 PAN patients were finally enrolled and 57 (60.64%) presented kidney manifestation. The mean age of onset was (37.76±17.40) years old and the interval from onset to diagnosis was 10 (0 to 240) months. Forty patients were misdiagnosed once or more times. In patients with renal involvement, 9 cases suffered from renal ischemia or infarction, 31 with microscopic haematuria, 26 with proteinuria, renal artery or its branch involved in 17 cases, renal vein thrombosis in 1 case, 4 cases with pyeloureterectasis, one case with renal fascia thickening, 33 cases with impaired renal function (serum creatinine>84 μmol/L) including creatinine>140 μmol/L in 10 patients. Renal artery branch stenosis was the most common presentation [9 cases (52.94%)] of renal vascular involvement, other abnormalities including nodular dilatation [4 cases (23.53%)], occlusion [3 cases (17.65%)]. There were significant differences (P<0.05) in the PAN patients with and without renal involvement in the following: age of onset [(33.72±16.13) years vs. (43.97±17.66) years, t2=2.901, P=0.005], weight loss(≥4kg since PAN onset) [25(43.86%) vs. 7(18.92%), χ2=6.216, P=0.013], elevation of diastolic blood pressure [22(38.60%) vs. 7(18.92%), χ2=4.072, P=0.044], acromegaly gangrene [18(31.58%) vs. 21(56.76%), χ2=5.859, P=0.015], and gastrointestinal artery involvement [20(35.09%) vs. 6(1.22%), χ2=3.993, P=0.046]. Laboratory parameters and the application of glucocorticoid and cyclophosphamide therapies were similar in two groups (all P>0.05).@*Conclusion@#Young PAN patients are more likely to be associated with renal involvement, especially gastrointestinal arteries.
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Objective To investigate the clinical characteristics of polyarteritis nodosa (PAN) patients with renal involvement. Methods PAN patients admitted to the department of rheumatology, department of pediatrics, department of nephrology, general internal medicine department and department of vascular surgery at Peking Union Medical College Hospital from June 2012 to August 2018 were enrolled in this study and were divided into two groups according to renal involvement or not. The clinical characteristics were analyzed. Results A total of 94 PAN patients were finally enrolled and 57 (60.64%) presented kidney manifestation. The mean age of onset was (37.76±17.40) years old and the interval from onset to diagnosis was 10 (0 to 240) months. Forty patients were misdiagnosed once or more times. In patients with renal involvement, 9 cases suffered from renal ischemia or infarction, 31 with microscopic haematuria, 26 with proteinuria, renal artery or its branch involved in 17 cases, renal vein thrombosis in 1 case, 4 cases with pyeloureterectasis, one case with renal fascia thickening, 33 cases with impaired renal function (serum creatinine>84 μmol/L) including creatinine>140 μmol/L in 10 patients. Renal artery branch stenosis was the most common presentation [9 cases (52.94%)] of renal vascular involvement, other abnormalities including nodular dilatation [4 cases (23.53%)], occlusion [3 cases (17.65%)]. There were significant differences (P<0.05) in the PAN patients with and without renal involvement in the following: age of onset [(33.72±16.13) years vs. (43.97±17.66)years, t2=2.901, P=0.005], weight loss(≥4kg since PAN onset) [25(43.86%) vs. 7(18.92%), χ2=6.216, P=0.013], elevation of diastolic blood pressure [22(38.60%) vs. 7 (18.92%), χ2=4.072, P=0.044], acromegaly gangrene [18(31.58%) vs. 21(56.76%), χ2=5.859, P=0.015], and gastrointestinal artery involvement [20(35.09%) vs. 6(1.22%), χ2=3.993, P=0.046]. Laboratory parameters and the application of glucocorticoid and cyclophosphamide therapies were similar in two groups (all P>0.05). Conclusion Young PAN patients are more likely to be associated with renal involvement, especially gastrointestinal arteries.
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Three patients diagnosed as amyloidosis secondary to ankylosing spondylitis (AS) in Peking Union Medical College Hospital are reported.All 3 cases had renal abnormalities,2 cases were complicated with cardiac abnormalities,1 case was complicated with gastrointestinal abnormalities and 1 case was complicated with skin moss-like changes.Eleven cases of AS with amyloidosis were retrieved from Wanfang database and Pubmed database from 1997 to 2017.In total 14 cases,the male and female ratio was 5:2,the mean age of AS onset was (29.6± 10.5)years,and the mean age of amyloidosis diagnosed was(44.0± 10.5)years.Eight patients presented with edema and severe proteinuria,2 cases showed diarrhea,2 cases showed dyspnea,while 2 cases had no obvious symptoms.Thirteen cases had renal abnormalities and 1 case had only heart involvement.Of the 13 patients with renal involvement,2 cases were complicated with cardiac abnormalities,2 cases complicated with gastrointestinal abnormalities,2 cases complicated with skin abnormalities.The mean ESR was(83.7±22.6)mm/1 h,C reactive protein was(89±59)mg/L at onset.After the diagnosis of amyloidosis,9 patients were treated with TNF-α inhibitors,3 cases received symptomatic therapy,1 case underwent renal transplantation and 1 case were treated with peritoneal dialysis.In 9 patients treated with TNF-α inhibitors,8 cases were relieved and 1 case lost follow-up.Three patients who received symptomatic treatment all died,while patients receiving kidney transplantation and peritoneal dialysis were in stable condition.The study indicates that secondary amyloidosis often occurs in young male AS patients who have long course and poorly controlled disease,the kidney is the most common affected organ.TNF-α inhibitors may be effective for treatment of amyloidosis secondary to AS.
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Objective To explore the influence of prozone effect on anti-nuclear antibodies (ANA) testing by indirect immunofluorescence assay (IIFA).Methods The samples with high titer of ANA (≥1∶1 000) were selected from 880fresh serum samples, and were subsequently diluted in 1∶100, 1∶1 000and 1∶10 000ratio.Prozone effect was defined as fluorescence intensity from 1∶1 000dilution was stronger than that from1∶100dilution.The samples with prozone effect were determined manually or by Sprinter XL and EUROPattern.The samples with prozone effect were further characterized by combinations of fluorescence patterns, fluorescence intensities and autoantibody specificities.Results A total of 880samples were tested.Importantly, 34samples displayed prozone effect (3.86％in total and 29.57％in samples with ANA≥1∶1 000).Interestingly, prozone effect was identified by manual detection as well as by Sprinter XL with similar fluorescence patterns and fluorescence intensities.Notably, EUROPattern can only select the central area for identification.Among all samples with prozone effect, 74.42％samples exhibited fluorescence intensities of≥1∶10 000.Speckled pattern was the most prevalent fluorescence patterns in samples with prozone effect (46.51％).In addition, anti-RNP antibodies (62.79％) were the most popular autoantibodies in samples with prozone effect, followed by anti-dsDNA antibodies (51.16％) and anti-SSA antibodies (51.16％).Conclusion Prozone effect was present in ANA testing, especially in samples with high titers, resulting in underestimating the titers.The study highlighted that special attention should be paid to the prozone effect in clinical practice.
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Objective The aim of this study was to compare the short-term and long-term prognostic significance of dif-ferent systemic inflammatory scores in patients with gastric cancer:neutrophil lymphocyte ratio(NLR) and platelet lymphocyte ratio ( PLR). Methods The clinical data of 240 patients with gastric cancer who underwent radical surgery were retrospectively analyzed. The relationship between NLR,PLR,glasgow prognostic score( GPS) and clinicopathological characteristics and perioperative compli-cations were compared. Survival analysis was performed using Kaplan-Meier survival analysis. The Log-rank methods were used to test the difference significance,and the multivariate analysis was performed using the Cox regression risk model. Results Patients in the high NLR and PLR groups were older,had a high GPS,deep tumor infiltration,more lymph node metastasis,and a late TNM stage (P<0. 05). The rate of positive margin in the high NLR group was higher(P<0. 05). Patients in the high NLR and PLR groups re-quired significantly higher transfusion rates than those in the low NLR and PLR groups(P<0. 05),and the high NLR group had more postoperative complications(P<0. 05). Univariate analysis showed that age,GPS,tumor location,tumor infiltration depth,lymph node metastasis status,TNM stage,vascular tumor thrombus,nerve infiltration,NLR and PLR were associated with postoperative survival of gastric cancer patients(P<0. 05). The overall survival(OS)in high NLR and high PLR groups was lower than that of low NLR and low PLR groups(P=0. 018 and P<0. 001). Cox regression analysis showed GPS and lymph node metastasis were independent prog-nostic factors of OS(P<0. 001 and P=0. 002). Conclusion Preoperative systemic inflammatory scores NLR and PLR are prognos-tic factors affecting the preoperative clinical outcomes of gastric cancer. It is recommended to be used in combination with other prog-nostic indicators for routine use in the prognosis of patients undergoing radical gastrectomy.