Enthesitis in patients with psoriatic arthritis: A nationwide data from the Chinese Registry of Psoriatic Arthritis (CREPAR) / 中华医学杂志(英文版)

BACKGROUND@#The clinical features of enthesitis in patients with psoriatic arthritis (PsA) have been reported in some Western countries, but data in China are very limited. This study aimed to describe the characteristics of enthesitis in Chinese patients with PsA and compared them with those in other cohorts.@*METHODS@#Patients with PsA enrolled in the Chinese Registry of Psoriatic Arthritis (CREPAR) (December 2018 to June 2021) were included. Data including demographics, clinical characteristics, disease activity measures, and treatment were collected at enrollment. Enthesitis was assessed by the Spondyloarthritis Research Consortium of Canada (SPARCC), Maastricht ankylosing spondylitis enthesitis score (MASES), and Leeds enthesitis index (LEI) indices. A multivariable logistic model was used to identify factors related to enthesitis. We also compared our results with those of other cohorts.@*RESULTS@#In total, 1074 PsA patients were included, 308 (28.7%) of whom had enthesitis. The average number of enthesitis was 3.3 ± 2.8 (range: 1.0-18.0). More than half of the patients (165, 53.6%) had one or two tender entheseal sites. Patients with enthesitis had an earlier age of onset for both psoriasis and arthritis, reported a higher proportion of PsA duration over 5 years, and had a higher percentage of axial involvement and greater disease activity. Multivariable logistic regression showed that axial involvement (odds ratio [OR] 2.21, 95% confidence interval [CI], 1.59-3.08; P <0.001), psoriasis area and severity index (PASI) (OR: 1.03, 95% CI: 1.01-1.04; P = 0.002), and disease activity score 28-C reactive protein (DAS28-CRP) (OR: 1.25, 95% CI: 1.01-1.55; P = 0.037) were associated with enthesitis. Compared with the results of other studies, Chinese patients with enthesitis had a younger age, lower body mass index (BMI), a higher rate of positive human leukocyte antigen (HLA)-B27, more frequent dactylitis, and a higher proportion of conventional synthetic disease-modifying antirheumatic drugs' (csDMARDs) use.@*CONCLUSIONS@#Enthesitis is a common condition among Chinese patients with PsA. It is important to evaluate entheses in both peripheral and axial sites.

Clinical characteristics of 37 antiphospholipid syndrome patients complicated by autoimmune hemolytic anemia / 中华内科杂志

Objective:We sought to investigate the clinical characteristics and risk factors of antiphospholipid syndrome (APS) complicated by autoimmune hemolytic anemia (AIHA).Methods:Retrospective anaysis.Three hundred fifteen consecutive patients with APS were enrolled at the Department of Rheumatology of Peking Union Medical College Hospital between May 2017 to May 2021, and their clinical manifestations[including initial symptoms, time interval between APS onset and diagnosis, systemic lupus erythematosus(SLE), thrombotic events, obstetric morbidity, and extra-criteria manifestations] and laboratory test results[including blood routine, antiphospholipid antibodies(aPLs), blood lipid profile, homocysteine, anti-nuclear antibody profile, immunoglobulin levels, and complement levels] were collected. Then, univariate and multivariate logistic regression analyses were performed. Clinical features and risk factors were analyzed using univariable and multivariable logistic regression analysis.Results:Among 315 APS patients, 37 cases (11.7%) were complicated by AIHA, and AIHA was the first manifestation or co-occurrence. The median time interval between APS onset and diagnosis was 12 months. The proportion of SLE in APS patients combined with AIHA was higher than that in APS patients without AIHA[62.2%(23/37) vs. 19.4%(54/278), P<0.001]. There was no significant difference in the proportions of thrombosis and pregnancy morbidity between the two groups. In terms of extra-criteria manifestations, APS patients with AIHA had a significantly ( P<0.05) greater risk of thrombocytopenia ( OR=6.19, 95% CI 2.81-13.65) and higher proportions of hypocomplementemia, a positive lupus anticoagulant (LA) result, double aPLs positivity[i.e., any two of the following antibodies were positive: LA, anticardilolipin antibody(aCL), and anti-β2 glycoprotein Ⅰ(β2GPⅠ)], and triple aPLs positivity (i.e., LA, aCL, and anti-β2GPⅠ antibodies were all positive). Multivariate logistic regression analysis showed that SLE ( OR=3.46,95% CI 1.60-7.48), thrombocytopenia ( OR=2.56,95% CI 1.15-5.67), and hypocomplementemia ( OR=4.29,95% CI 2.03-9.04) were independent risk factors for the complication of APS. In the primary APS subgroup, multivariate logistic regression analysis showed that livedo reticularis ( OR=10.51,95%CI 1.06-103.78), thrombocytopenia ( OR=3.77, 95% CI 1.23-11.57), and hypocomplementemia ( OR=5.92,95% CI 1.95-17.95) were independent risk factors for the complication of APS. Conclusions:AIHA is not rare in APS patients; moreover, it occurs more frequently in APS secondary to SLE and is more likely to present with a variety of extra-criteria manifestations. Patients with AIHA should be promptly tested for antiphospholipid antibody profiles and alerted to the possibility of thrombotic events.

Sex disparities in clinical characteristics of Chinese patients with systemic sclerosis / 中华内科杂志

Objective:To evaluate the differences in clinical characteristics between different genders of Chinese patients with systemic sclerosis(SSc).Methods:The data of SSc patients registered in Chinese Rheumatism Data Center between August 2008 and June 2020 were retrospectively analyzed.Results:A total of 1 844 patients with SSc were enrolled in the study. The ratio of males to females was 289 to 1 555. The onset age was (48.6±13.7) years in males and (45.5±13.1) years in females( P<0.001). Male patients represented shorter disease duration [2.0(0.0, 4.0)years vs.3.0(1.0, 7.0) years, P<0.001],higher proportion of diffuse cutaneous SSc (dcSSc) [63.0% (182/289)vs.44.2%(688/1 555), P<0.001]. Although more man patients experienced smoking [47.4%(137/289) vs. 1.7%(27/1 555), P<0.001] and exposure to harmful environments [7.6%(22/289) vs. 2.1%(33/1 555), P<0.001], there was no statistically significant difference in interstitial lung disease between male and female patients [69.3%(181/261) vs. 74.5%(1 085/1 457), P=0.084].Otherwise, Raynaud′s phenomenon [87.7% (1 364/1 555) vs.75.4%(218/289), P<0.001], arthritis [11.1%(173/1 555) vs.6.9%(20/289), P=0.032], gastroesophageal reflux disease [22.0%(342/ 1 555) vs.13.1%(38/289), P=0.001], and leucopoenia [10.7(161/1 511)% vs. 6.1%(17/279), P=0.019] were more common in female patients, but finger ulcer was less common [22.5%(350/1 555) vs. 30.4%(88/289), P=0.004]. Antinuclear antibody(ANA) positivity rate [85.6%(1 310/1 531) vs. 78.6%(221/281), P=0.003], anti-RNP antibody positivity rate [23.1%(342/1 479) vs.14.0%(38/271), P=0.001], anti-SSA antibody positivity rate [28.2%(419/1 487) vs.13.9%(38/274), P<0.001] were higher in female patients. Physician′s global assessment(PGA) scores [1.4 (1.0, 2.0) vs. 1.0 (0.3, 1.6), P<0.001] and modified Rodnan Skin Score(mRSS) [18.0 (9.5, 28.0) vs. 14.0 (5.0, 28.0), P=0.003] were higher in males. Conclusion:Even though male SSc patients account for a small proportion, more extensive skin involvement, finger ulcers and higher PGA are manifested in males. Physicians need pay attention to these clinical disparities between different genders in SSc.

The 492nd case: recurrent thrombosis, thrombocytopenia / 中华内科杂志

A 43-year-old female patient was admitted with recurrent thrombosis for more than 2 years and thrombocytopenia for more than 1 year. Both arterial and venous thromboses developed especially at rare sites even during anticoagulation therapy such as cerebral venous sinus thrombosis. Antinuclear antibody, anti-ENA antibody and antiphospholipid antibody were all negative. Platelet count elevated to normal after high dose glucocorticoid and intravenous immunoglobulin (IVIG). Immune thrombocytopenia was suspected. When 4 grade thrombocytopenia recurred, intravenous heparin, rituximab 600 mg, IVIG and eltrombopag were administrated. After 3 weeks, thrombocytopenia did not improve, and new thrombosis developed instead. Screening of thrombophilia related genes revealed PROS1 gene heterozygous mutation and MTHFR TT genotype. Low amount of serum IgG κ monoclonal protein was detected. Heparin-induced thrombocytopenia was differentiated and excluded. Finally, serum negative antiphospholipid syndrome was considered the most likely diagnosis. Dexamethasone 20 mg/day × 4 days combined with sirolimus 2 mg/day was prescribed. The patient was discharged with low molecular weight heparin. At one month, her headache was greatly relieved. The platelet count raised to 20-30×10 9/L, and no new thrombosis or bleeding was reported.

Analysis of clinical manifestations at different age of onset in systemic sclerosis / 中华风湿病学杂志

Objective:To investigate the clinical and laboratory characteristics of systemic sclerosis (SSc) patients at different age of onset.Methods:Data of SSc patients with onset age ≥18 years old who were registered in the Peking Union Medical College Hospital and Chinese Rheumatism Data Center from August 2008 to June 2020 were included. Patients were divided into 3 groups by the age of onset according to the age segmentation of the World Health Organization. Counting variables were presented as frequency (percentage). Quantitative results were presented as mean±standard deviation, or median, inter quartile range. Differences between groups were analyzed by analysis of variance, the Mann-Whitney test or the chi-square test, depen-ding on the distribution of the variables.Results:Six hundred and eighty-two SSc patients were included. Accor-ding to the age of onset, they were divided into three groups: youth group (18-44 years old), middle-aged group (45-59 years old) and elderly group (over 60 years old). There were 361 patients in the youth group,245 patients in the middle age group and 76 patients in the elderly group. The mean age of onset was (43.8±12.1) years. The variables with significant different among the groups were as the following: left ventricular diastolic dysfunction [14.0%(14/100), 38.8%(39/98), 65.4%(17/26); χ2=30.756, P<0.001]; cardiac arrhythmias [1.9% (7/361), 3.7% (9/361), 7.9% (6/76), χ2=7.38, P=0.024), Raynaud's phenomenon [94.7% (342/361), 89.4%(219/245), 89.5%(68/76), χ2=6.73, P=0.035], loss of finger pad substance [36.9%(133/360), 25.4% (62/244), 18.4% (14/76), χ2=15.184, P=0.001]; digital ulcer [31.0% (112/361), 23.0% (56/244), 15.8% (12/76), χ2=9.86, P=0.007]; arthritis [16.3%(59/361), 13.5%(33/245), 5.3%(4/76), χ2=6.49, P=0.039], digital contracture [11.6%(42/361), 5.7%(14/245), 9.2%(7/76), χ2=6.10, P=0.047]; positive anti-RNP antibody [32.3% (116/359), 20.7% (50/241), 17.3% (13/75), χ2=14.06, P=0.001]; and positive anti-centromere antibody [8.9% (32/351), 18.4%(45/239), 23.7%(18/76), χ2=17.78, P<0.001] were significantly different between the young age group and elder group. Conclusion:The predominant age of disease onset of SSc is middle and young age. Elder onset SSc patients are more likely to have left ventricular diastolic dysfunction, and young onset patients are more likely to have microvascular lesions, which needs more attentions by clinicians.

Sudden chest pain, painful swelling left lower limb and malar erythema / 中华风湿病学杂志

Objective:To improve the understanding of the relationship between antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE).Methods:The clinical characteristics and process of diagnosis and treatment of a case was reported and analyzed. This patient was initially diagnosed as antiphospholipid syndrome and later developed new skin lesion and positive anti-dsDNA antibody, which made the diagnosis of systemic lupus erythematosus.Results:A 15-year-old girl suffered acute pulmonary embolism, lower extremity deep vein thrombosis, and high titer of anti-phospholipid antibody, but negative for other autoantibodies. So primary antiphospholipid syndrome was diagnosed. Symptoms were improved after thrombolysis and anticoa-gulation treatment. During the follow-up period, the patient developed malar erythema, lymphocytopenia, proteinuria, positive ANA, anti-dsDNA antibody, and reduced complement level. So she was diagnosed with systemic lupus erythematosus. After glucocorticoid pulse therapy and immunosuppressants treatment, the symptoms were relieved and lupus disease activity was decreased.Conclusion:A few primary APS patients can progress into SLE. Patients with risk factors such as a younger age of onset, positive ANA and positive Coomb's test results should be closely followed up.

Recommendations for the diagnosis and treatment of connective tissue disease associated pulmonary arterial hypertension in China / 中华内科杂志

Pulmonary arterial hypertension (PAH) is a clinicopathological syndrome caused by the increase of pulmonary artery, and it is the most serious complication of connective tissue disease (CTD). In recent years, a lot of progress has been made in the diagnosis, treatment and evaluation of PAH. Chinese Rheumatology Association formulated this recommendation on the basis of current experience and guidelines, in order to promote early screening, early diagnosis and early intervention of CTD-PAH, as well as patient follow-up and management, to improve the prognosis of CTD-PAH patients.

Recommendations for management of antiphospholipid syndrome in China / 中华内科杂志

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder with vascular, obstetric, and hematological manifestations associated with thrombotic and inflammatory mechanisms orchestrated by antiphospholipid (aPLs) antibodies. Current clinical practice in APS is highly variable duo to lack of high quality of evidence. Here, Chinese Rheumatology Association developed recommendations for management of APS in China. The recommendations cover the early diagnosis, disease evaluation, thrombotic risk assessment, and treatment.

The 490th case: arthralgia, amenorrhea, aphasia / 中华内科杂志

A 25-year-old woman was admitted to Peking Union Medical Hospital presented with arthralgia for 5 years, amenorrhea for 16 months, and speech disorder for 3 months. This patient has been afflicted by intermittent pain in metacarpophalangeal and proximal interphalangeal joints of both hands for 5 years. Her menstruation has been irregular 1 year ago and rapidly progressed to amenorrhea. Laboratory tests revealed postmenopausal sex hormones levels (estradiol<5 ng/L, follicle-stimulating hormone 62.5 IU/L, luteinizing hormone 58.71 IU/L) and no antral follicles were seen in gynecologic ultrasound. She was diagnosed with premature ovarian failure and treated with hormone replacement therapy, still with no ovulation. Numbness and weakness of right arm has recurrently occurred to her 4 months ago, and persistent weakness of right limbs combined with motor speech disorder occurred 1 month later. Magnetic resonance angiography was suggestive of ischemic stroke. Hormone replacement therapy was discontinued. Comprehensive laboratory tests revealed positive anti-dsDNA, anti-SSA/SSB, anticardiolipin and anti-β 2GPⅠ antibodies. Systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS) was diagnosed. Since no drug with gonadal toxicity had been applied to the patient before, her amenorrhea was considered to be due to autoimmune oophoritis secondary to SLE. After treated with high-dose glucocorticoid, mycophenolate mofetil and hydroxychloroquine for 4 months, her menstruation recurred and regularly occurred till now. In some cases, amenorrhea in SLE patient might be resulted from autoimmune oophoritis associated with lupus flare, instead of use of drug with gonadal toxicity.

Clinical characteristics and pregnancy outcomes of non-criteria obstetric antiphospholipid syndrome / 中华内科杂志

Objective:To study clinical characteristics and pregnancy outcomes under anti-coagulation therapy of non-criteria obstetric antiphospholipid syndrome.Methods:Patients suspected of obstetric antiphospholipid syndrome(OAPS) were recruited through Chinese Rheumatism Data Center from 2015 to 2019 consecutively. Patients fulfilling 2006 Sydney revised antiphospholipid syndrome criteria were classified as OAPS. Patients fulfilling definition of non-criteria OAPS(NCOAPS) by expert consensus on diagnosis and management of obstetric antiphospholipid syndrome of China were classified as NCOAPS. Clinical characteristics and laboratory results of two groups were compared. Live birth rates and pregnancy outcomes under anti-coagulation therapy were studied.Results:A total of 88 patients were enrolled, including 56 patients (63.6%) as OAPS, 32(36.4%) as NCOAPS. Live births were only reached in 16.1% (9/56) in OAPS patients and 12.5%(4/32) in NCOAPS. Fetal losses after 10 weeks of gestation and pre-eclampsia before 34 weeks were more common in OAPS group compared to NCOAPS group [78.6%(44/56) vs. 18.8%(6/32), P<0.001; 25.0%(14/56) vs. 3.1%(1/32), P=0.020, respectively]. After enrollment, 15 pregnancies were recorded in OAPS, 10 in NCOAPS, all of whom were treated with low-dose aspirin (LDA) combined with low-molecular weight heparin (LMWH). Live birth rates saw dramatic improvements compared to baseline levels in OAPS [16.1% (9/56) vs. 11/15] along with NCOAPS [12.5% (4/32) vs. 7/10]. Conclusion:Though NCOAPS and OAPS patients differ in antiphospholipid antibody spectrum and pattern of pregnancy morbidities, both groups benefit from LDA combined with LWMH treatment, as live birth rates improve. Non-criteria OAPS patients are recommended to receive anti-coagulation therapy during pregnancy.

Clinical characteristics and risk factors for mortality analysis of 94 cases of rheumatic diseases with pneumomediastinum / 中华风湿病学杂志

Objective:To analyze the clinical characteristics and risk factors of rheumatic diseases complicated with Pneumomediastinum (PnM).Methods:A retrospective analysis of 94 inpatients with rheumatic diseases associated PnM from Peking Union Medical College Hospital and Shanxi Bethune Hospital between January 1998 and October 2018 was carried out. Patients were divided into idiopathic inflammatory myopathies (IIM) and the non-IIM group. Clinical features, laboratory examinations and treatment were compared between the two groups. Cox proportional hazard model was used to investigate the risk factors for prognosis.Results:A total of 94 patients were included in the study, with an average age of (45±14) years. Forty-five (48%) of them were male. There were 62 patients in the IIM group and the other 32 were in the non-IIM group. Sixty-nine patients had predisposing factors before PnM. Severe cough and assisted mechanical ventilation were the most common causes. Compared with the non-IIM group, the incidence of digital vasculitis (29% vs 6%, χ2=6.540, P=0.008), arthritis (60% vs 28%, χ2=8.409, P=0.004), interstitial lung disease (ILD)(98% vs 78%, χ2=11.129, P=0.002) were higher in the IIM group, treatment with cyclophosphamide was higher in the IIM group ( χ2=4.458, P=0.035). There was non-significant difference in mortality between the two groups during hospitalization (50% vs 59%) and 6 months after PnM (64% vs72%) ( P>0.05). Pulmonary infection was the only risk factor for poor prognosis [ HR=3.131, 95% CI (1.025, 9.561), P=0.045], in which bacteria infection was the most common cause (65/75, 86.7%). Conclusion:PnM is a severe complication of rheumatic diseases. To get a good prognosis, rheumatol-ogists should balance the benefit and risk of infection of immunosuppressive therapy.

Clinical characteristics in 40 patients with longitudinally extensive transverse myelitis and connective tissue disease / 中华内科杂志

Objective:Longitudinally extensive transverse myelitis (LETM) could be seen in patients with connective tissue disease (CTD), especially systemic lupus erythematosus (SLE) or primary Sj?gren′s syndrome (pSS). Some patients are combined with neuromyelitis optica spectrum disorders (NMOSD)(termed CTD-LETM-NMOSD) while others without (termed CTD-LETM-non-NMOSD). The aim of this study is to compare the clinical characteristics of CTD-LETM-NMOSD patients to CTD-LETM-non-NMOSD patients.Methods:We retrospectively collected data from 40 CTD patients with LETM who were admitted to the Department of Neurology or Rheumatology at Peking Union Medical College Hospital from Jan, 2006 to Dec, 2016. They were divided into CTD-LETM-NMOSD and CTD-LETM-non-NMOSD two groups. Demographic characteristics, clinical and laboratory features were obtained from the database. Relapse rates and clinical outcome were analyzed by Kaplan-Meier method.Results:Among 40 patients with CTD, 28 (70.0%) were NMOSD while 12 (30.0%) were not. The positivity rates of anti-SSA, antibodies to aquaporin-4 (anti-AQP4) were significantly higher in patients with NMOSD than those in patients with non-NMOSD ( P<0.05). Age, gender, clinical features, disease duration, anti-double-stranded DNA antibody, anti-ribosomal P antibody, antiphospholipid antibodies, expanded disability status scale (EDSS) scores, and magnetic resonance imaging (MRI) features were all comparable between two groups. CTD-NMOSD patients had significantly higher disease relapse rate (75.0% vs. 3/12, P<0.01). Conclusion:Anti-SSA and anti-AQP4 positivity is associated with NMOSD and higher relapse rates, which suggests that NMOSD in CTD-LETM patients may represent distinct characteristics and pathogenesis from patients with CTD-LETM-non NMOSD.

Use of new diagnostic criteria for reclassification of polyarteritis nodosa / 中华内科杂志

With the recognition of antineutrophil cytoplasmic antibodies (ANCA)-related vasculitis and widespread vaccination against viral hepatitis B, the prevalence of polyarteritis nodosa (PAN) varied considerably. In our study, patients diagnosed as polyarteritis nodasa (PAN)based on the 1990 American College of Rheumatology(ACR) criteria were reclassified using 2007 European Medicines Agency(EMA) algorithm modified by 2012 Chapel Hill Consensus Conference(CHCC) definitions, aiming to evaluate the new classification criteria for the diagnosis of PAN. A total of 113 PAN patients admitted to Peking Union Medical College Hospital from January 2002 to December 2018 were retrospectively analyzed, who were classified into three subtypes including 9 patients with cutaneous, 80 with classic and 24 Hepatitis B virus (HBV) associated PAN. All patients were reclassified according to 2007 EMA algorithm using CHCC 2012 definitions. As a result, 7 patients were diagnosed as microscopic polyangiitis(MPA) and 19 patients with unclassified vasculitis based on the new classification criteria. The diagnostic rate of PAN was gradually declined as the classification criteria of vasculitides was update. However, there are quite a few PAN patients in China, whom rheumatologists should pay attention to the early diagnosis and treatment.

The 476th case: skin rash, edema, thrombocytopenia and anemia / 中华内科杂志

The 21-year-old male patient was admitted to the Department of Rheumatology and Immunology at Peking Union Medical College Hospital with chief complaints of "skin rash for 1 year and edema for 2 months". He was diagnosed with systemic lupus erythematosus (SLE) with renal, cardiac and hematological involvement. Remission was not achieved after glucocorticoid pulse treatment. The patient experienced oliguria, malignant hypertension, accompanied by thrombocytopenia and low serum complements, and elevated lactate dehydrogenase and serum creatinine. Schistocytes were seen in the peripheral blood smear. Thrombotic microangiopathy (TMA) secondary to SLE was diagnosed. Though plasma exchange was partially effective, TMA could not be controlled yet. The activity of serum von Willebrand factor -cleaving protease (ADAMTS 13) was 100%, and ADAMTS 13 inhibitor was negative. Finally, remission of the disease was achieved after second glucocorticoid pulse therapy and rituximab treatment. At the 3-month follow-up, the patient′s condition was stable with mild anemia and normal platelet count. Patients with TMA secondary to SLE are heterogenous, while normal ADAMT 13 activity indicates poor prognosis. Early and aggressive treatment is important for disease control, and plasma exchange is helpful as a supportive care.

The 482nd case: vomiting, paroxysmal dizziness and anhidrosis / 中华内科杂志

A 31-year-old woman was admitted to Peking Union Medical College Hospital presented with intermittent vomiting and abdominal pain for 2 years, and recurrence with paroxysmal dizziness for 1 month. This patient was diagnosed with systemic lupus erythematosus (SLE) 2 years ago with involvement of gastrointestinal and urinary tracts. One month ago, repeated vomiting and nausea recurred. No laboratory and imaging abnormalities were found in central nervous system and gastrointestinal evaluation. Orthostatic hypotension and fluctuation of blood pressure were recorded during hospitalization. Combined with sexual dysfunction, left adie pupil, anhidrosis and abnormal sympathetic skin response, autonomic nerve dysfunction related to SLE was diagnosed. After treated with pulse glucocorticoids and intravenous immunoglobulin, the patient′s symptoms improved remarkably. Orthostatic hypotension in SLE patients may link to autonomic nerve dysfunction.

The 476th case: skin rash, edema, thrombocytopenia and anemia / 中华内科杂志

The 21-year-old male patient was admitted to the Department of Rheumatology and Immunology at Peking Union Medical College Hospital with chief complaints of "skin rash for 1 year and edema for 2 months". He was diagnosed with systemic lupus erythematosus (SLE) with renal, cardiac and hematological involvement. Remission was not achieved after glucocorticoid pulse treatment. The patient experienced oliguria, malignant hypertension, accompanied by thrombocytopenia and low serum complements, and elevated lactate dehydrogenase and serum creatinine. Schistocytes were seen in the peripheral blood smear. Thrombotic microangiopathy (TMA) secondary to SLE was diagnosed. Though plasma exchange was partially effective, TMA could not be controlled yet. The activity of serum von Willebrand factor -cleaving protease (ADAMTS 13) was 100%, and ADAMTS 13 inhibitor was negative. Finally, remission of the disease was achieved after second glucocorticoid pulse therapy and rituximab treatment. At the 3-month follow-up, the patient′s condition was stable with mild anemia and normal platelet count. Patients with TMA secondary to SLE are heterogenous, while normal ADAMT 13 activity indicates poor prognosis. Early and aggressive treatment is important for disease control, and plasma exchange is helpful as a supportive care.

Clinical features and risk factors of portal vein thrombosis in 28 patients with antiphospholipid syndrome / 中华内科杂志

Objective@#Portal vein thrombosis (PVT) is a rare and severe clinical manifestation of antiphospholipid syndrome (APS), as well as a predictor of poor prognosis. This study was conducted to explore the clinical features and risk factors of PVT in APS patients.@*Methods@#A total of 123 APS patients diagnosed from 2012 to 2019 were retrospectively enrolled. The diagnosis of PVT was made according to the 2009 American College of Liver Diseases (AASLD) criteria. Clinical and laboratory data were collected. A multivariate (MV) logistic regression model was constructed using a stepwise forward selection procedure among those candidate univariables with P values<0.10.@*Results@#A total of 28 cases with PVT, and 95 control cases without PVT were finally enrolled.The 28 APS-PVT patients included 5 males and 23 females with age range from 17 to 63 years. Clinical manifestations included acute thrombosis in 8 patients, chronic thrombosis in 16, and 4 with portal vein spongiform. As to the involved vessels, single portal vein thrombosis was seen in 20 patients, portal combined with superior mesenteric vein (SMV) and splenic vein in one patient, portal plus SMV in 4 and only SMV in 3 patients. Other manifestations were portal hypertension (16/28), esophageal varices (13/28), spleen infarction (7/28) and gastrointestinal bleeding (4/28). Two antiphospholipid antibodies were positive in 13 cases. Triple positive antibodies were seen in 7 cases. Multivariate logistic regression analysis showed that disease duration less than 0.5 years (OR=72.74, 95%CI 7.50-705.45, P<0.001), hypoalbuminemia (OR=356.45, 95%CI 19.19-6 620.14, P<0.001), and elevated erythrocyte sedimentation rate (ESR)/C-reactive protein (CRP) (OR=14.41, 95%CI 1.49-139.20, P<0.001) were independent risk factors for PVT in APS.@*Conclusion@#PVT is usually misdiagnosed due to insidious onset. Short disease duration, hypoalbuminemia and elevated ESR/CRP are risk factors for PVT in APS. Better understanding, early diagnosis and treatment will improve the clinical outcome.

The 469th case: multiple cutaneous abscesses, pelvic mass, and lung cavities / 中华内科杂志

A 28-year-old woman with multiple abscesses for 2 month and fever for 1 month was admitted in Peking Union Medical College Hospital.The skin abscesses gradually developed at skull,face,abdominal wall and pelvis.Laboratory examinations related to inflammatory reactions were strongly high including erythrocyte sedimentation rate 99 mm/1 h,C-reactive protein160.28 mg/L and ferritin 1 584 μg/L.Multiple nodules and cavities were detected in lungs.And vesico-cervical fistula was found during exploratory laparotomy.The pathological tests of abdominal tissues demonstrated necrosis and granuloma.Evidence of infectious diseases was not definite.Positive anti-proteinase 3 (PR3) antibody confirmed the diagnosis of granulomatosis with polyangiitis.After treated with glucocorticoid and immunosuppressants,the patient's symptoms improved remarkably.This case suggested that systemic vasculitis should be considered as a differential diagnosis of multiple abscesses which are not explained by infections.

The clinical characteristics of antiphospholipid syndrome associated with chronic thromboembolic pulmonary hypertension / 中华内科杂志

Objective To analyze the clinical characteristics of antiphospholipid syndrome (APS) patients with chronic thromboembolic pulmonary hypertension (CTEPH).Methods A total of 22 APS patients with CTEPH were enrolled in our study,who were admitted in Peking Union Medical College Hospital from January 2012 to August 2018.Diagnoses were confirmed by computed tomographic pulmonary angiography (CTPA),or pulmonary angiography.Demographic characteristics,clinical manifestations,laboratory tests,therapy,World Health Organization (WHO) functional class were retrospectively collected.Results There were 15 females and 7 males with a median age of 29-year-old.Chest pain (6 cases),dyspnea on exertion (22 cases),cough (6 cases) and hemoptysis (9 cases) were the most common clinical manifestations.Lupus anticoagulant (LA),anticardiolipin (ACL) antibodies and anti-beta 2 glycoprotein Ⅰ (anti-β2 GP Ⅰ) antibodies were all positive in 12 patients,two of three antibodies positive in 5 patients,only one positive in 5 patients.The WHO functional classes were Ⅱ-Ⅳ before treatment.Anticoagulants were administrated in all patients.After multidisciplinary evaluation,9 patients underwent pulmonary thromboendarterectomy (PTE),who all had a good outcome.Symptoms in eleven over thirteen patients with only anticoagulants improved.Three patients developed cardiac deterioration while other 3 patients died of right heart failure during follow-up.Conclusion Pulmonary embolism is one of the most common thrombotic events in APS patients.It is important to recognize symptoms and signs related to pulmonary embolism and start anticoagulation as soon as possible.Standard anticoagulation improves symptoms but can't reverse the process of pulmonary hypertension.Some patients may benefit from PTE after anticoagulation and multidisciplinary evaluation.

The neurological manifestations in 52 patients with primary Sj?gren′s syndrome / 中华内科杂志

Objective To summarize the neurological manifestations in patients with primary Sj?gren′s syndrome (pSS). Methods A total of 68 patients were diagnosed as pSS in neurology department of Peking Union Medical College Hospital from March 2014 to February 2018, among whom sixteen cases were excluded due to modified final diagnoses of primary neurological diseases. Therefore 52 pSS patients with neurological involvement were enrolled and retrospectively analyzed. They were divided into two groups as extensive group in which both central and peripheral nervous system were involved, non?extensive group in which either central or peripheral nervous system was involved. Results Neurological manifestations were presented as primary symptoms in 98.1%(51/52) patients, while 35 had neurological involvement as their only extraglandular manifestations. Thirteen cases were in extensive group. The other 39 in non?extensive group including 22 cases with only peripheral nervous system involved and 17 cases with only single central nervous system involved. Compared to non?extensive group, the proportion of woman patients [13/13 vs.71.8% (28/39),P＝0.047], serum IgG level [17.73(11.11,22.41)g/L vs. 11.49(9.58,13.40)g/L, P＝0.017] and positive rates of oligoclonal band (OB) in cerebral spinal fluid (CSF) [7/13 vs. 22.6%(7/31), P＝0.042)] were significantly higher in extensive involvement group. Conclusions Neurological manifestations in pSS patients could be extensive, both central and peripheral nervous system might be associated. Female patients, high serum IgG level and positive OB in CSF are risk factors of extensive neurological involvement, suggesting that the immune system may be generally over?stimulated.