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1.
Zhonghua Nei Ke Za Zhi ; 62(9): 1102-1113, 2023 Sep 01.
Artigo em Chinês | MEDLINE | ID: mdl-37650184

RESUMO

Objective: To investigate the clinical characteristics of patients with rheumatic diseases and abnormal liver function, as well as determine the proportion and severity of liver function abnormalities. Methods: Cross-sectional study. Data were collected from patients registered in the Chinese Rheumatism Date Center from 2011 to 2021. The rheumatic diseases analyzed in this study were rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjogren syndrome (SS), ankylosing spondylitis (AS), and gout. Patient data, including demographic characteristics [ such as age, sex, body mass index,(BMI), and smoking history], liver function test results [including alanine aminotransferase (ALT), aspartate aminotransferase, alkaline phosphatase(ALP), and total bilirubin], and use of anti-rheumatic immune drugs and liver-protective drugs, were collected and compared between groups with normal and abnormal liver functions. In addition, the proportions of abnormal liver function were compared between sex and age groups. Results: A total of 116 308 patients were included in this study, including 49 659 with RA, 17 597 with SLE, 9 039 with SS, 11 321 with AS, and 28 692 with gout. The lowest proportion of liver function abnormalities was observed in patients with RA[11.02% (5 470/49 659)], followed by those with SS[17.97% (1 624/9 039)] and AS [18.22% (2 063/11 321) ], whereas patients with SLE [21.14% (3 720/17 597) ] and gout [28.73% (8 242/28 692)] exhibited the highest proportion of these abnormalities. Elevated ALT, mostly classified as grade 1, was the most commonly noted liver function abnormality, whereas elevated ALP was the least common. Some patients who took liver-protective drugs had normal liver function, with the lowest percentage observed in patients with gout [7.45% (36/483) ] and ranging from 21.7% to 30.34% in patients with RA, SLE, SS, and AS. The proportion of liver function abnormalities was higher in males than in females for all disease types [RA: 13.8%(1 368/9 906) vs. 10.3%(4 102/39 753); SLE: 33.6% (479/1 424) vs. 20.0% (3 241/16 173); SS: 25.4%(111/437) vs. 17.6%(1 513/8 602); AS: 20.1%(1 629/8 119) vs. 13.6% (434/3 202); and gout: 29.3% (8 033/27 394) vs. 16.1% (209/1 298)]. In RA, SLE, and AS, the proportions of liver function abnormalities were similar across all age groups. In SS, the proportion of liver function abnormalities increased with age [<40 years: 14.9%(294/1 979); 40-59 years: 18.1%(858/4 741); ≥60 years: 20.4%(472/2 319)], whereas a reversal of this trend was observed in gout [<40 years: 34.9%(4 294/12 320); 40-59 years: 25.5%(2 905/11 398);≥60 years: 21.0%(1 042/4 971)]. Conclusions: The proportions of combined liver function abnormalities in patients with rheumatologic diseases were high, and the utilization rates of liver-protective drugs were low. It is necessary to pay more attention to monitoring patients' liver function, timely administer liver-protective drugs, and optimize liver-protective regimens during the treatment of rheumatic diseases.


Assuntos
Antirreumáticos , Artrite Reumatoide , Gota , Lúpus Eritematoso Sistêmico , Doenças Reumáticas , Síndrome de Sjogren , Espondilite Anquilosante , Feminino , Masculino , Humanos , Adulto , Estudos Transversais , Fígado , Fosfatase Alcalina
4.
Zhonghua Nei Ke Za Zhi ; 61(8): 969-972, 2022 Aug 01.
Artigo em Chinês | MEDLINE | ID: mdl-35922227

RESUMO

A 50-year-old man was admitted to the Department of Rheumatology at Peking Union Medical College Hospital with rash for 6 months, and fever and hematuria for 5 months. The main clinical manifestations included fever, fatigue, purpura, hematuria and thrombocytopenia. He was positive for antinuclear antibody (ANA), anti-neutrophil cytoplasmic antibodies (ANCA) and rheumatoid factor (RF), and had low complement levels. Initial blood culture, echocardiography and chest CT showed no signs of infection. Diagnosis of connective tissue disease was made initially. His disease improved under treatment with glucocorticoids and immunosuppressive agents, but relapsed when glucocorticoids were tapered. After admission, the diagnosis was reconsidered, and infective endocarditis was finally diagnosed with repeated positive blood cultures and vegetations detected by transesophageal echocardiography. Amoxicillin and clavulanate potassium were initiated, and surgery was performed. His symptoms finally recovered gradually.


Assuntos
Endocardite Bacteriana , Exantema , Anticorpos Anticitoplasma de Neutrófilos , Endocardite Bacteriana/diagnóstico , Exantema/etiologia , Febre , Hematúria/etiologia , Humanos , Masculino , Pessoa de Meia-Idade
5.
Zhonghua Nei Ke Za Zhi ; 61(7): 779-784, 2022 Jul 01.
Artigo em Chinês | MEDLINE | ID: mdl-35764561

RESUMO

Objective: To investigate the relationship between psoriasis severity and clinical features in psoriatic arthritis (PsA). Methods: Patients were recruited from the Chinese REgistry of Psoriatic ARthritis (CREPAR) between December 2018 and June 2021, and data were collected including the baseline demographic characteristics, various clinical manifestations (including arthritis, nail disease, comorbidities), laboratory tests[including erythrocyte sedimentation rate(ESR), C-reactive protein (CRP)], health assessment questionnaire (HAQ). Body surface area (BSA) and psoriasis area and severity index (PASI) were selected for the tools of assessment of cutaneous psoriasis. Patients were divided to two groups, including the severe psoriasis group (BSA>10%) and the non-severe psoriasis group (BSA≤10%). Disease assessment included ankylosing spondylitis disease activity score (ASDAS), disease activity score 28 (DAS28) and disease activity in psoriatic arthritis (DAPSA). Results: 1 074 eligible patients with PsA were recruited, and 106 (9.9%) had severe psoriasis. Compared with non-severe psoriasis group, the severe psoriasis group had more peripheral joint involvement (including patients with ever or current peripheral arthritis, 94.3% vs. 85.6%), more polyarticular joint involvement (including patients with current peripheral arthritis, 74.0% vs. 58.2%), more axial joint involvement (51.4% vs. 39.9%), more nail disease (72.6% vs. 61.4%), more frequency of smoking (20.2% vs. 18.7%), and higher proportion of hypertension (23.4% vs. 14.4%). In addition, the severe psoriasis group had higher level of ESR [33(10, 70) mm/1h vs. 20(9, 38) mm/1h] and CRP [18.6(5.0, 60.8) mg/L vs. 7.0(2.4, 18.1) mg/L], higher values of DAS28-ESR (4.5±1.7 vs. 3.7±1.5), DAS28-CRP (4.2±1.5 vs. 3.4±1.4), ASDAS-ESR (3.5±1.4 vs. 2.6±1.2), and ASDAS-CRP(3.4±1.6 vs. 2.5±1.2), higher scores of HAQ [0.6(0.1, 1.0) vs. 0.3(0.0, 0.8)]. Conclusion: Patients with PsA with severe psoriasis bore a heavier disease burden. Therefore, clinicians were supposed to pay more attention to them. In addition to skin lesions, they should also focus on examination of other clinical manifestations, such as joints and nails.


Assuntos
Artrite Psoriásica , Doenças da Unha , Psoríase , Espondilite Anquilosante , Proteína C-Reativa , Humanos , Doenças da Unha/complicações , Psoríase/diagnóstico , Índice de Gravidade de Doença
7.
Zhonghua Nei Ke Za Zhi ; 60(12): 1189-1192, 2021 Dec 01.
Artigo em Chinês | MEDLINE | ID: mdl-34856695

RESUMO

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-ß2GPⅠ 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.


Assuntos
Afasia , Lúpus Eritematoso Sistêmico , Adulto , Amenorreia/etiologia , Artralgia/etiologia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/complicações , Recidiva Local de Neoplasia , Exacerbação dos Sintomas
8.
Zhonghua Nei Ke Za Zhi ; 60(5): 453-458, 2021 May 01.
Artigo em Chinês | MEDLINE | ID: mdl-33906275

RESUMO

Objective: Longitudinally extensive transverse myelitis (LETM) could be seen in patients with connective tissue disease (CTD), especially systemic lupus erythematosus (SLE) or primary Sjögren'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.


Assuntos
Doenças do Tecido Conjuntivo , Mielite Transversa , Neuromielite Óptica , Aquaporina 4 , Autoanticorpos , Doenças do Tecido Conjuntivo/complicações , Humanos , Recidiva Local de Neoplasia , Neuromielite Óptica/diagnóstico por imagem , Estudos Retrospectivos
9.
Zhonghua Nei Ke Za Zhi ; 60(4): 388-392, 2021 Apr 01.
Artigo em Chinês | MEDLINE | ID: mdl-33765713

RESUMO

One 51 years old man was admitted to the rheumatology department with a history of prominent eyes, headache and blurred vision for half year. The main manifestations included retrobulbar inflammatory pseudotumor and retroperitoneal fibrosis. He was initially diagnosed as granulomatosis with polyangiitis. Prednisone and cyclophosphamide were administrated and effective. New mass of dura mater and osteosclerosis presented during follow up. Finally Erdheim Chester disease(ECD) was diagnosed by biopsy and pathological examination. Vemurafenib, a v-raf murine sarcoma viral oncogenes homolog B1 (BRAF) inhibitor, 480 mg was given twice a day. The patient's condition is stable and still in follow-up. Although ECD is a rare histiocytosis, clinicians should pay attention to its manifestations and differential diagnoses.


Assuntos
Doença de Erdheim-Chester , Animais , Biópsia , Diagnóstico Diferencial , Doença de Erdheim-Chester/diagnóstico , Cefaleia , Humanos , Masculino , Camundongos , Vemurafenib
11.
Zhonghua Nei Ke Za Zhi ; 59(5): 360-365, 2020 May 01.
Artigo em Chinês | MEDLINE | ID: mdl-32370464

RESUMO

Objective: To analyze the clinical and prognostic characteristics in patients with eosinophilic granulomatosis with polyangitis (EGPA). Methods: The clinical data of 146 EGPA patients hospitalized in Peking Union Medical College Hospital from 2000 to 2019 were analyzed retrospectively, including clinical manifestations, laboratory results, treatment, complications and outcome at discharge. Birmingham Vasculitis activity score-V3 (BVAS-V3) was used to evaluate disease activity. Results: The ratio of male to female was 1.8∶1 with average age (41.7±16.1) year-old. The median time from disease onset to diagnosis was 18(6, 60) months (0.5~450). The most common clinical manifestations were lung [121(82.9%)] and nose/paranasal sinuses [119(81.5%)] involvement. The positive rate of anti-neutrophil cytoplasmic antibody (ANCA) was 24.7%, mainly peripheral (P)-ANCA/myeloperoxidase (MPO)-ANCA. Compared with ANCA-negative patients, the ANCA-positive patients had a higher incidence of renal involvement and nervous system involvement (66.7% vs. 20.9%, 80.6% vs. 51.8%, P<0.001), fever and optic neuropathy (66.7% vs. 40.9%,8.3% vs. 0, P<0.05), more active disease [median BVAS-V3 25(18,30)vs. 19(14,24),P=0.001] and more elevated erythrocyte sedimentation rate [40.5(20.5,82.8)mm/1h vs. 25.0(13.3,50.8)mm/1h,P=0.006] and C-reactive protein [37.1(11.8,72.9)mg/L vs.13.5(3.4,66.1)mg/L,P=0.036]. More ANCA-negative patients had pleural effusion (20.9% vs. 5.6%, P<0.04) compared with ANCA-negative patients. Pulmonary infection was the most common complication. A total of 12 EGPA patients (8.2%)achieved remission and 6 patients (4.1%)died or discharged themselves from the hospital. Conclusion: EGPA is a rare small vessel vasculitis. The clinical manifestations and outcomes are heterogenous. The mortality rate of EGPA is high.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/sangue , Granulomatose com Poliangiite/diagnóstico , Adulto , Eosinófilos , Feminino , Granulomatose com Poliangiite/mortalidade , Humanos , Masculino , Pessoa de Meia-Idade , Peroxidase , Derrame Pleural , Prognóstico , Estudos Retrospectivos
12.
Zhonghua Nei Ke Za Zhi ; 59(4): 303-308, 2020 Apr 01.
Artigo em Chinês | MEDLINE | ID: mdl-32209197

RESUMO

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.


Assuntos
Anticorpos Monoclonais/uso terapêutico , Síndrome de Behçet/tratamento farmacológico , Imunossupressores/uso terapêutico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adalimumab , Anticorpos Monoclonais/administração & dosagem , Anticorpos Monoclonais/efeitos adversos , Síndrome de Behçet/diagnóstico , Esquema de Medicação , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/efeitos adversos , Infliximab , Estudos Retrospectivos , Resultado do Tratamento
13.
Zhonghua Nei Ke Za Zhi ; 59(3): 250-252, 2020 Mar 01.
Artigo em Chinês | MEDLINE | ID: mdl-32146758

RESUMO

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.


Assuntos
Lúpus Eritematoso Sistêmico/diagnóstico , Microangiopatias Trombóticas/diagnóstico , Proteína ADAMTS13/genética , Anemia , Edema , Exantema , Glucocorticoides/uso terapêutico , Humanos , Lúpus Eritematoso Sistêmico/complicações , Masculino , Troca Plasmática , Rituximab/uso terapêutico , Microangiopatias Trombóticas/complicações , Adulto Jovem
14.
Hum Exp Toxicol ; 39(8): 1118-1129, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32162539

RESUMO

OBJECTIVE: Methamphetamine (MA) abuse induces neurotoxicity and causes neuronal cell apoptosis. Gastrodin is a traditional Chinese herbal medicine used for the treatment of nerve injuries, spinal cord injuries, and some central nervous system diseases as well. The present study investigated the neuroprotective effects of gastrodin against MA-induced neurotoxicity in neuronal cells and its potential protective mechanism. METHODS: The primary cortex neuronal culture was divided into four groups (control group, MA group, MA + gastrodin group, and MA + gastrodin + small interfering RNA group). The neurotoxicity of MA was assessed by detecting apoptotic cells by terminal deoxynucleotidyl transferase deoxyuridine triphosphate nick-end labeling assay and cell viability by cell counting kit 8 (CCK-8) method, the Tuj1-positive cells and the average axonal length were detected by immunofluorescence, and the expressions of cyclic adenosine monophosphate (cAMP), protein kinase A (PKA), cAMP-response element-binding (CREB), and brain-derived neurotrophic factor (BDNF) proteins were detected by Western blot. RESULTS: The results of CCK-8 assay showed that 0.5 mM MA was an optimal concentration that induced neurotoxicity (p < 0.01). Pretreatment with 25 mg/L gastrodin exerted maximum protective effects on neuronal cells. The expression levels of cAMP, PKA, phosphorylated PKA, CREB, phosphorylated CREB, and BDNF proteins were decreased in the MA group, and pretreatment with gastrodin upregulated the expression levels of these proteins (p < 0.01). The expressions of PKA and CREB proteins showed no significant changes in the control group, MA group, and gastrodin group. Compared the MA + gastrodin + small interfering RNA group with MA + gastrodin group, the Tuj1-positive cells and the average axonal length were decreased significantly, while the number of apoptotic cells was increased (p < 0.05). CONCLUSION: Gastrodin has neuroprotective effects against MA-induced neurotoxicity, which exerts neuroprotective effects via regulation of cAMP/PKA/CREB signaling pathway and upregulates the expression of BDNF.


Assuntos
Álcoois Benzílicos/farmacologia , Glucosídeos/farmacologia , Metanfetamina/toxicidade , Neurônios/efeitos dos fármacos , Fármacos Neuroprotetores/farmacologia , Síndromes Neurotóxicas/metabolismo , Animais , Apoptose/efeitos dos fármacos , Fator Neurotrófico Derivado do Encéfalo/genética , Fator Neurotrófico Derivado do Encéfalo/metabolismo , Células Cultivadas , AMP Cíclico/metabolismo , Proteína de Ligação ao Elemento de Resposta ao AMP Cíclico/metabolismo , Proteínas Quinases Dependentes de AMP Cíclico/metabolismo , Feminino , Córtex Motor/citologia , Neurônios/metabolismo , Ratos Sprague-Dawley
15.
Zhonghua Nei Ke Za Zhi ; 58(10): 758-762, 2019 Oct 01.
Artigo em Chinês | MEDLINE | ID: mdl-31594174

RESUMO

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, t(2)=2.901, P=0.005], weight loss(≥4kg since PAN onset) [25(43.86%) vs. 7(18.92%), χ(2)=6.216, P=0.013], elevation of diastolic blood pressure [22(38.60%) vs. 7(18.92%), χ(2)=4.072, P=0.044], acromegaly gangrene [18(31.58%) vs. 21(56.76%), χ(2)=5.859, P=0.015], and gastrointestinal artery involvement [20(35.09%) vs. 6(1.22%), χ(2)=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.


Assuntos
Arterite/diagnóstico , Nefropatias/etiologia , Rim/fisiopatologia , Poliarterite Nodosa/diagnóstico , Adulto , Idoso , Ciclofosfamida/uso terapêutico , Gastroenteropatias , Glomerulonefrite/diagnóstico , Glucocorticoides/uso terapêutico , Humanos , Infarto , Nefropatias/fisiopatologia , Pessoa de Meia-Idade , Poliarterite Nodosa/complicações , Poliarterite Nodosa/tratamento farmacológico , Adulto Jovem
16.
Zhonghua Nei Ke Za Zhi ; 58(4): 282-287, 2019 Apr 01.
Artigo em Chinês | MEDLINE | ID: mdl-30917421

RESUMO

Objective: To explore the predictive factors for determining the therapeutic response and prognosis of severe thrombocytopenia (TP) in patients with primary Sjögren syndrome(pSS). Method: Patients with pSS and severe TP (platelet count ≤ 50×10(9)/L) admitted between 2010 to 2016 at Peking Union Medical College Hospital were classified according to their therapeutic response and analyzed retrospectively. The response parameters and clinical data including bone marrow aspiration results and laboratory findings were collected. Result: Thirty patients were finally analyzed, including twenty with appreciable bone marrow aspiration results. Fourteen and 7 patients achieved a complete response (CR) and a partial response (PR) respectively, other 9 patients with no response (NR). The megakaryocyte counts in bone marrow (BM-MK) counts per slide in each group were 13.0 (9.2,23.5) in CR patients, 7.0 (7.0,20.0) in PR patients, and 5.0 (1.0,6.0) in NR patients. BM-MK counts in patients with clinical response (CR+PR) were significantly higher than those with NR (P=0.006). A receiver-operation characteristic analysis revealed a cutoff value of BM-MK counts at 6.5 per slide stratifying patients by different responses with a sensitivity of 13/14, a specificity of 6/7, and area under the curve of 0.879. Univariate analysis indicated a better prognosis as BM-MK counts>6.5 per slide. Conclusion: BM-MK count could be a predictive factor of response in patients with pSS and severe TP. Patients with BM-MK counts≤6.5 per slide represent worse platelet improvement..


Assuntos
Megacariócitos , Síndrome de Sjogren , Medula Óssea , Humanos , Contagem de Plaquetas , Estudos Retrospectivos , Trombocitopenia
17.
Zhonghua Nei Ke Za Zhi ; 58(4): 333-336, 2019 Apr 01.
Artigo em Chinês | MEDLINE | ID: mdl-30917433

RESUMO

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.


Assuntos
Abscesso , Pele , Adulto , Sedimentação Sanguínea , Diagnóstico Diferencial , Feminino , Humanos , Imunossupressores , Pneumopatias , Dermatopatias
18.
Zhonghua Nei Ke Za Zhi ; 57(12): 942-944, 2018 Dec 01.
Artigo em Chinês | MEDLINE | ID: mdl-30486568

RESUMO

A 45-year-old woman was admitted to the Department of Rheumatology and Immunology, Peking Union Medical College Hospital, due to weakness of the upper limbs, fever, and blurred vision. She was clinically diagnosed as systemic lupus erythematosus overlapped primary biliary cirrhosis, with renal, retinal, hematological and musculoskeletal involvement, combined with severe pulmonary infection and respiratory failure. Treated with glucocorticoids, ursodeoxycholic acid, antibiotics and respiratory support, the patient got better. A couple of days later, her fever recurred and platelets count dropped to 30×10(9)/L, hemoglobin to 78 g/L, fibrinogen to<1.5 g/L, ferritin to 1 640 ng/ml, natural killer (NK) cell count to 8/µl, the activity of NK cells 2% (reference value 9.5%-23.5%), considering the occurrence of hemophagocytic lymphohistiocytosis (HLH). Cytomegalovirus pp65 antigenemia test: 13 positive cells/2×10(5) WBC. Considered the possibility of HLH caused by cytomegalovirus infection and treated by 250 mg ganciclovir intravenous drip twice a day for a full course. The temperature of the patient was gradually reduced to 36.5 ℃, the count of platelets were increased to 229 ×10(9)/L, the hemoglobin was increased to 94 g/L, and the fibrinogen was increased to 3.26 g/L. When there were unexplained critical signs of the primary disease during systemic lupus erythematosus treatment, severe complications such as infection, HLH, thrombotic thrombocytopenic purpura should be taken into account.


Assuntos
Infecções por Citomegalovirus/diagnóstico , Febre/etiologia , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/complicações , Linfo-Histiocitose Hemofagocítica/diagnóstico , Pancitopenia/diagnóstico , Antibacterianos/uso terapêutico , Infecções por Citomegalovirus/tratamento farmacológico , Feminino , Glucocorticoides/uso terapêutico , Humanos , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Pessoa de Meia-Idade , Púrpura Trombocitopênica Trombótica , Esteroides/uso terapêutico
19.
Zhonghua Nei Ke Za Zhi ; 56(6): 477-480, 2017 Jun 01.
Artigo em Chinês | MEDLINE | ID: mdl-28592052

RESUMO

A 54-year-old man was admitted to the Department of Rheumatology in Peking Union Medical College Hospital due to polyarthritis, back pain and multiple subcutaneous masses. He had symmetrical polyarthritis at onset, which was diagnosed as rheumatoid arthritis. His follow-up and treatment were irregular. Back pain and multiple subcutaneous masses developed 2 year ago. Erythrocyte sedimentation rate and C-reactive protein were elevated. Rheumatoid factor and Anti-cyclic citrullinated peptide antibody were positive. No evidence of infection or malignancy was detected. Chest CT suggested pulmonary fibrosis and osteolytic lesions of sternum. Spinal MRI showed paravertebral soft tissue. Biopsy of the soft tissue suggested the pathological finding of a rheumatoid nodule. The patient was diagnosed as rheumatoid arthritis complicated with multiple rheumatoid bursal cysts.Combination therapy of methylprednisolone(40mg/d) and cyclophosphamide(0.4 g/w) lead to a rapid improvement of clinical symptoms and laboratory parameters.The patient was still in remission at 6-month follow-up.


Assuntos
Artralgia/etiologia , Artrite Reumatoide/sangue , Artrite Reumatoide/diagnóstico , Dor nas Costas/etiologia , Proteína C-Reativa/metabolismo , Fator Reumatoide/sangue , Artrite Reumatoide/tratamento farmacológico , Autoanticorpos/sangue , Biópsia , Sedimentação Sanguínea , Ciclofosfamida/administração & dosagem , Quimioterapia Combinada , Humanos , Masculino , Metilprednisolona/administração & dosagem , Pessoa de Meia-Idade , Resultado do Tratamento
20.
Zhonghua Nei Ke Za Zhi ; 56(5): 388-392, 2017 May 01.
Artigo em Chinês | MEDLINE | ID: mdl-28460514

RESUMO

A 53-year-old woman presented with arthralgia and dyspepsia on exertion.Symmetrical joint swelling and pain of bilateral hands with pigmentation sedimentation in the articular surfaces, dry cough and short of breath were the main clinical manifestations.Elevated levels of erythrocyte sedimentation rate(89 mm/1 h), C-reactive protein (64.45 mg/L), IgG(31.22 g/L) and IgA (5.44 g/L). Rheumatoid factor was positive and anti-cyclic citrullinated peptide antibody was negative.Chest high-resolution computed tomography scan found that diffusely distributed lung nodules, round cysts with varying sizes and patchy ground glass opacities.A significant plasma cell infiltration and amyloid deposition were seen in lung tissue.The patient was finally diagnosed as rheumatoid arthritis, interstitial lung disease and pulmonary nodules.Combination therapy of prednisone 7.5 mg qodpo, thalidomide 50 mg qdpo, tocilizumab 560 mg iv once per month for 1 month.Chest computed tomography showed stable lung nodules, however, pulmonary interstitial lesions gradually aggravated.


Assuntos
Artrite Reumatoide/diagnóstico , Autoanticorpos/sangue , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Nódulos Pulmonares Múltiplos/diagnóstico por imagem , Esforço Físico , Artralgia/etiologia , Sedimentação Sanguínea , Proteína C-Reativa , Tosse , Quimioterapia Combinada , Dispepsia/etiologia , Dispneia , Feminino , Humanos , Pessoa de Meia-Idade , Prednisona/administração & dosagem , Fator Reumatoide/sangue , Talidomida/administração & dosagem , Tomografia Computadorizada por Raios X , Resultado do Tratamento
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