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1.
Article in Chinese | WPRIM | ID: wpr-911471

ABSTRACT

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.

2.
Article in Chinese | WPRIM | ID: wpr-911443

ABSTRACT

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.

3.
Article in Chinese | WPRIM | ID: wpr-911436

ABSTRACT

Objective:To investigate the clinical characteristics and risk factors for osteonecrosis (ON) in patients with systemic lupus erythematosus (SLE).Methods:This is a case-control study. A total of 118 patients diagnosed with SLE complicated with ON (study group) were retrospectively analyzed between 2014 and 2019. Gender, age, and course matched 118 SLE patients without ON were selected as controls. Clinical manifestations, laboratory findings, medical history, and treatments were recorded and analyzed.Results:Among 118 patients, the male to female ratio was 20 to 98 with a median age of 27 years and course of disease 1-168 months. Compared with the control group, the study group presented a longer cumulative duration of glucocorticoid therapy [36.5 (0-168) months vs. 19.0(0-168) months on average, P<0.05], a higher incidence of osteoporosis (29.7% vs. 4.2%, P<0.001), a higher frequency of immune-suppressive therapy (83.9% vs. 64.4%, P=0.035), more organs involveed [median 2 (0-5) vs. 1 (0-4)], and a higher SLE disease activity index (SLEDAI) (14.22±7.40 vs. 11.63±6.11, P<0.05) in univariate logistic regression. The control group had a higher rate of positive Coombs test (39.8% vs. 7.6%, P<0.05). No statistical difference on methylprednisolone (MP) pulse therapy ( P>0.05) was observed. Multivariate logistic regression suggested that SLEDAI ( OR= 1.070, 95% CI 1.026-1.116, P<0.005), osteoporosis ( OR=10.668, 95% CI 3.911-29.103, P<0.001) and a positive Coombs test( OR=0.492, 95% CI 0.266-0.910, P<0.05) were related to the development of ON in SLE patients. Conclusion:A higher disease activity and the presence of osteoporosis are associated with an increased risk of ON in patients with SLE, and positive Coombs test seems a protective factor of ON.

4.
Chinese Journal of Orthopaedics ; (12): 1683-1691, 2021.
Article in Chinese | WPRIM | ID: wpr-910761

ABSTRACT

Objective:To investigate the effect of anterior or posterior debridement on infections of the lumbar vertebral fusion with cage and pedical screw system.Methods:A total of 10 cases (3 males and 7 females, age 49.80±13.29 years) with infections in the lumbar fusion device were admitted from January 2013 to December 2019. The cases were diagnosed with deep infections after the preview surgery at 10.80±13.24 months, including 10 cases with 12 cages infections and 8 cases with screw system infections. 7 cases were done with debridement and removal of the cages via the anterior approach. And another 3 cases underwent posterior debridement and removal of lumbar fusion cages. The changes in laboratory examination such as WBC count, ESR, CRP, visual analogue scale (VAS) and Oswestry Disability Index (ODI) score were compared preoperatively, 2 months and 6 months postoperatively.Results:No patients were lost in the follow-up. The average follow-up time periods is 16.30±5.10 months (range 9-24 months). There was no significant difference in WBC count between 2 and 6 months after surgery compared with preoperation ( F=0.855, P=0.436). The ESR, CRP, VAS and ODI scores decreased in 2 months and 6 months after sugery, which has significant differences compared preoperation ( P<0.05). The abnormal signals in the lambar vertebral showed a gradual fading trend in the postoperative MRI. The completely fading time was 5.00±1.33 months (range 3-7 months). One-stage bone grafting was performed in 6 cases with 8 spaces via anterior approach, 5 cases with 7 spaces showed the intervertebral fusion after postoperative with 6.80±2.28 months (range 4-10 months), 1 case wirh 1 space was not fused. One-stage bone grafting was performed in 2 cases via posterior approach: 1 case was fused after postoperative and the other patient was not, which due to bone graft area infection. Postoperative bone graft displacement occurred with 1 case and 1 case with L 5 nerve root pulling injury during the operation. Conclusion:The fusion cage can be removed by debridement via posterior approach, but it is difficult to done the completely debridement, which the main reason is the obstruction of the posterior nerve and bone structure. Posterior approach also have risks of infection recurrence and nerve root injury. Cage removal via anterior approach was relatively easy and debridement was thoroughly, which has the risk of injury of important adjacent vessels.

5.
Chinese Journal of Rheumatology ; (12): 525-528, 2021.
Article in Chinese | WPRIM | ID: wpr-910202

ABSTRACT

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.

6.
Article in Chinese | WPRIM | ID: wpr-885164

ABSTRACT

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.

7.
Article in Chinese | WPRIM | ID: wpr-885160

ABSTRACT

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.

8.
Article in Chinese | WPRIM | ID: wpr-885145

ABSTRACT

IgG 4 related disease (IgG 4-RD) is an immune medicated rare disease, characterized with chronic inflammation and fibrosis in the involved organs, it is a systemic disease affected nearly every anatomic site of the body, usually involvement of multiple organs, and with diverse clinical manifestations. Due to the the relative novelty of the disease and under-recognition, the overall level of diagnosis and treatment in China is uneven. Till now, there is no relevant expert consensus or guidance of IgG 4-RD in China. In order to further improve the understanding and standardize the management of IgG 4-RD, on the basis of summarizing domestic and international experience, the China Alliance For Rare Diseases, together with the Chinese Rheumatology Association, organized an expert group and established the Chinese expert consensus on the diagnosis and treatment of IgG 4 related diseases.

9.
Article in Chinese | WPRIM | ID: wpr-799734

ABSTRACT

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.

10.
Chinese Journal of Neurology ; (12): 1021-1027, 2020.
Article in Chinese | WPRIM | ID: wpr-870918

ABSTRACT

Objective:To explore the clinical profile and prognostic features of relapsing polychondritis (RP) with nervous system involvement.Methods:The clinical data of 28 RP patients with nervous system involvement admitted to Peking Union Medical College Hospital from December 2005 to November 2019 were collected and analyzed.Results:The median age of the patients was 50 years and 57.1% (16/28) were men. Patients with central nervous system involvement accounted for 92.9% (26/28) of the total. Common manifestations included headache (69.2%, 18/26), cognitive impairment (38.5%, 10/26), and mental disorders (38.5%, 10/26); only two cases had peripheral nervous system damage. Brain magnetic resonance imaging revealed that subcortical (13/18) and cortical (8/18) tissues were most affected. Cerebrospinal fluid tests showed that the median white blood cell count was 55×10 6/L, while the protein level was also slighted elevated ((0.54±0.17) g/L). All patients were treated with glucocorticoids, and 85.7% (24/28) of patients with immunosuppressants; 27 patients showed optimistic responses to the treatment, while 24 had recurrent courses. Cognitive dysfunction was the most common residual symptom. Follow-up data of 16 patients were available with an average follow-up time of 3.3 years. Ten of the patients were in stable condition and three patients died. Conclusions:Nervous system damage, especially limbic encephalitis and meningitis may present in course of RP. However, it is a challenge to diagnose for the non-specific clinical manifestations. Treatment with corticosteroids and immunosuppressants can lead to a favorable response.

11.
Article in Chinese | WPRIM | ID: wpr-870208

ABSTRACT

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.

12.
Article in Chinese | WPRIM | ID: wpr-870202

ABSTRACT

In recent years, osteoporosis (OP) has become one of the main diseases affecting the health of middle-aged and elderly people in China, and the prevalence of OP has increased significantly. The clinical diagnosis and treatment guidelines for this disease are also constantly updated. The overall principles speciallyemphasise that doctors and patients need to work together to negotiate the details of the diagnosis and treatment guidelines, in order to improve the OP clinical diagnosis and treatment rate. Therefore, patients′ knowledge of the disease, understanding of clinical guidelines, and cooperation with doctors to implement diagnosis and treatment plans are very important. In this study, from the most concerned issues of the patients, we established the OP patient practice guideline working group. 14 recommendations, as the OP patient practice guidelines, are proposed in accordance with the relevant principles of the "World Health Organization guidelines development manual" and the international normative process.

13.
Article in Chinese | WPRIM | ID: wpr-870191

ABSTRACT

In recent years, the clinical guidelines for the diagnosis and treatment of rheumatoid arthritis (RA) have been constantly updated. Among the general principles, it is particularly emphasized that, in order to improve the ratio of treat to target(T2T) of RA, doctors and patients should work together to negotiate the details of the guidelines. Therefore, it is important for patients to further understand the disease and clinical guidelines of RA, and to better cooperate with doctors. This study was based on the most concerned issues of RA patients and international standard procedure of guideline study, we organized the working group and introduce the following 16 recommendations constituting the RA patients′ practice guidelines.

14.
Article in Chinese | WPRIM | ID: wpr-870162

ABSTRACT

Gout is a crystal associated arthritis caused by monosodium urate (MSU) accumulating in joint, and it belongs to metabolic rheumatic disease. In China, gout is common but it is insufficient for education of standardized diagnosis and treatment for gout. Based on the evidence and guidelines from China and other countries, Chinese gout Collaborative Research Group developed standardization of diagnosis and treatment of gout in China. The purpose is to standardize the methods for diagnosis of gout, treatment opportunity and strategies in order to reduce misdiagnosis, missed diagnosis and irreversible damage.

15.
Article in Chinese | WPRIM | ID: wpr-870158

ABSTRACT

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.

16.
Article in Chinese | WPRIM | ID: wpr-870152

ABSTRACT

Sj?gren′s syndrome is a chronic systemic autoimmune disease characterized by lymphocyte proliferation and progressive exocrine gland damage. In China, standardized diagnosis and treatment for Sj?gren′s syndrome lags behind other common rheumatic diseases, such as rheumatoid arthritis and systemic lupus erythematosus. Based on the evidence and guidelines from China and other countries, Chinese Sj?gren′s Syndrome Collaborative Research Group together with stomatologist and ophthalmologist developed Standardization of diagnosis and treatment of primary Sj?gren′s syndrome. The purposes are: (1) to standardize the detection and interpretation of key indicators for the diagnosis of Sj?gren's syndrome, including serum anti SSA antibody and labial gland pathology; (2) to suggest using widely accepted disease activity index in evaluation of the disease; (3) to standardize rational management for Sj?gren′s syndrome patients with topical and systemic diseases.

17.
Article in Chinese | WPRIM | ID: wpr-870150

ABSTRACT

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.

18.
Article in Chinese | WPRIM | ID: wpr-870141

ABSTRACT

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.

19.
Article in Chinese | WPRIM | ID: wpr-868177

ABSTRACT

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.

20.
Article in Chinese | WPRIM | ID: wpr-868173

ABSTRACT

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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