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1.
The aim of this retrospective study was to assess the efficacy and tolerance of intravenous rituximab in multifocal primary cutaneous follicle centre lymphomas (PCFCL). Eleven patients with a multifocal PCFCL were treated with rituximab (MabThera(?)) administrated intravenously. After four infusions, an objective response was observed in 90% of patients, and one month after all the infusions (median of 6 infusions) all the patients had an objective response and complete remission was obtained in 7 of 11 patients (64%). Follow-up ranged from 9 to 65 months (median: 30 months). Local disease recurrence was observed in five patients. The median progression-free survival time after the end of treatment was 23.6 months. This study is the largest series of patients with multifocal primary PCFCL treated with intravenous rituximab. This therapy is a safe and effective treatment and could represent an excellent alternative treatment to radiotherapy.  相似文献   

2.
This was a multicenter study of rituximab, a chimeric monoclonal immunoglobulin G antibody directed against CD20, for the treatment of refractory autoimmune bullous diseases (pemphigus and pemphigoid). Ten patients (three with pemphigus vulgaris, six with pemphigus foliaceus and one with bullous pemphigoid) were treated with a single cycle of rituximab (four weekly infusions at a dose of 375 mg/m2 of body surface area). The primary end‐points were the number of serious adverse events and rate of complete remission at 40 weeks. Five patients (50%) achieved complete remission with minimal therapy (defined as no active lesions with lower doses of systemic corticosteroids compared to that with prednisolone 10 mg/day). Improvements in clinical scores (Pemphigus Disease Area Index) and decreases in autoantibody titers in the sera were observed in the four pemphigus patients who failed to achieve complete remission. This suggests that rituximab was effective in nine of 10 cases. Two serious adverse events (Pneumocystis carinii pneumonia and septic shock due to infectious arthritis) were observed and adequately treated with hospitalization. CD19‐positive B lymphocytes in the peripheral blood decreased on day 29 following rituximab treatment, and remained at low levels throughout the observation period (280 days). Our results confirmed the efficacy of rituximab therapy for refractory autoimmune bullous diseases in Japan.  相似文献   

3.
EFFICACY OF SHORT-CONTACT THERAPY WITH DITHRANOL IN CHILDHOOD PSORIASIS   总被引:3,自引:0,他引:3  
Background. Dithranol is a potent reducing substance and is thought to achieve its effect on psoriasis by reduction of cell turnover. It was shown to be efficacious in adults, but its efficacy has not been documented in children. Methods. The efficacy of short-contact therapy, a dithranol cream preparation, was retrospectively evaluated in 58 children with psoriasis. Dithranol cream (0.1-2%) was applied daily for 30 minutes to affected skin areas. Patients were evaluated at 1-4-week intervals. Results. The median duration of therapy before the onset of remission was 2 months. Remission was achieved in 81% of the children. The median duration of remission was 4 months. Mild adverse skin reactions occurred in 20% of patients, but only one patient had to discontinue therapy. Conclusion. Short-contact therapy with dithranol cream preparation is an effective and well-tolerated treatment for childhood psoriasis. A thorough explanation of the disease and mode of therapy to patients and their parents is essential for good compliance and subsequent success.  相似文献   

4.
Background It has been previously shown in a relatively small group of patients that a combination of immunoadsorption (IA) and rituximab with daily use of high‐dose oral corticosteroids and azathioprine/mycophenolate mofetil may induce a rapid and durable remission in severe, treatment‐resistant pemphigus. Objectives To achieve a more rapid reduction of serum autoantibody levels by a more frequent use of IA in the initial phase of treatment and to reduce the number of severe adverse events of continuous oral corticosteroid therapy by switching to pulsed intravenous applications. Methods Twenty‐three consecutive patients with severe pemphigus were included. IA was performed at initially 3‐ and later 4‐week intervals until lesions healed by 90%; 1000 mg rituximab was given at weeks 1 and 3, and intravenous dexamethasone pulses were administered at first every 3 weeks and then at increasing intervals in addition to daily azathioprine/mycophenolate mofetil. Results Along with a fast and durable decline of circulating autoantibody levels, all patients showed improvement of pemphigus lesions within the first weeks of therapy and long‐term complete remission was induced in 19 (83%) patients. In the remaining four patients, one (4%) minimal disease and three (13%) partial remissions were observed. Over the long‐term follow‐up of 11–43 (mean 29) months, six (26%) patients had a recurrence and in two (9%) patients, severe adverse events occurred. Conclusions This novel protocol treatment induces a fast and long‐term remission in severe pemphigus and seems to offer an improved side‐effect profile compared with daily use of corticosteroids.  相似文献   

5.
To evaluate the long‐term outcomes of rituximab in the treatment of pemphigus and the influence of disease duration and different dose of rituximab on the clinical response, 45 patients with refractory pemphigus treated with at least one cycle of two infusions of rituximab (375 mg/m2 per infusion weekly) were retrospectively studied. All patients were followed up for more than 2 years. All patients achieved complete or partial remission within 8 months of the first cycle. Thirty‐four (76%) patients relapsed at a median of 17 months. All patients who received additional cycles after relapse achieved new remissions. Early use of rituximab within 1 year of disease duration and high‐dose therapy induced better outcomes, although the results in early use were not statistically significant. Acute respiratory distress syndrome occurred in one patient. Rituximab is effective in treating pemphigus, but relapses are frequent during long‐term follow up, and additional cycles are beneficial in relapsed cases. Early and high‐dose rituximab therapy may be more effective.  相似文献   

6.
【摘要】 目的 探讨米诺环素单药或联合小剂量糖皮质激素(简称激素)治疗红斑型天疱疮(PE)和疱疹样天疱疮(PH)的疗效和安全性及对免疫指标的影响。方法 回顾性纳入2011年6月至2021年6月于北京大学第一医院皮肤科初诊、初治方案为米诺环素单独或联合小剂量激素且随访至少6个月的PE和PH患者。收集基线及不同随访时间点患者病情、自身抗体水平变化,分析疾病严重程度、诊断、自身抗体变化趋势及与疗效间的关系。采用Kaplan-Meier方法分析完全缓解情况,用卡方检验分析不同严重度和疗法下PF患者的疗效。结果 共纳入24例汉族患者,包括15例PE和9例PH,男女比例为1.4∶1,中位年龄68.8岁,中位病程为22.1个月,平均随访时间为21.8个月。24例均获得疾病控制,疾病控制时间M(Q1,Q3)为15.9(12,20.1)周。完全缓解23例 (95.8%),完全缓解时间8.7(6.4, 10)个月。米诺环素单药治疗1年完全缓解率(11/13)与联合小剂量激素疗法(9/11)差异无统计学意义(χ2 = 0.16,P = 0.692)。随访期间复发2例(8.7%),皆处于疾病控制状态,1例调整剂量后于第38周达完全缓解,另1例换用利妥昔单抗,半年后达到完全缓解。轻中度患者间疗效差异无统计学意义(χ2 = 0.28,P = 0.599)。3例发生药物相关不良反应,1例为背部体癣,2例为全身皮肤及牙龈部位色素沉着。结论 米诺环素单药或联合小剂量激素治疗轻中度PE或PH疗效显著,且无严重药物相关不良反应,但该方案的长期疗效、不良反应及患者预后需未来进一步扩大样本量,进行多中心、前瞻性研究。  相似文献   

7.
BackgroundLow-dose rituximab is a protocol used in several autoimmune diseases, that has also shown to be effective and safe in pemphigus vulgaris.ObjectivesTo study whether low-dose rituximab is also effective for bullous pemphigoid.MethodsPatients with BP were treated with a single cycle of two infusions of rituximab 500 mg at an interval of 2 weeks. Early and late end points were monitored.ResultsSix patients, five males and a female, with a mean age of 78.6 years (range 65–89) and a mean history of BP of 6.7 months (range 2–16) were included. A rapid and marked response was observed after a single cycle of treatment, with a mean time to disease control and to end of consolidation phase of 1.9 (range 1–3), and 4 weeks (range 3–5), respectively. Four patients achieved a late end point at a mean of 15.75 weeks (range 13–20). Three of them achieved partial remission with no therapy (two patients) or with minimal therapy (one patient), and one of them achieved complete remission with no therapy. One patient has 6 weeks of clinical follow-up after rituximab administration. The remaining patient relapsed 4 weeks after the rituximab treatment, and remains in complete remission with more than minimal therapy. One patient had a herpetic gingivostomatitis related to rituximab.ConclusionsLow-dose rituximab for BP achieved acceptable remission rates and steroid-sparing activity, with a better safety profile and a lower cost, compared to standard doses. This pilot study suggests that low-dose rituximab could be a therapeutic option for BP.  相似文献   

8.
One hundred seventeen patients with mycosis fungoides were treated with topical mechlorethamine hydrochloride. The probability of achieving a clinically apparent remission within 2 years of therapy was 75.8% in patients with stage I disease, 44.6% in patients with stage II disease, and 48.6% in patients with stage III disease. Patients with stage I disease achieved complete remission sooner (median, 6.5 months) than patients with stage II (median, 41.1 months) or stage III (median, 39.1 months) disease. The median time to relapse was 44.5 months. Sixty-eight patients (58.1%) developed a delayed hypersensitivity reaction, but only one patient had to discontinue therapy as a consequence. No appreciable differences were seen in the probability to achieve complete remission or time to complete remission as stratified by gender, substage, or the development of a delayed hypersensitivity reaction. Survival analysis revealed that the probability of surviving at 5 years was 89% for all patients. These findings compare favorably with results with other treatments for early stage mycosis fungoides.  相似文献   

9.
Seventy-six patients with mycosis fungoides (MF) were given topical mechlorethamine hydrochloride therapy. Allergic contact hypersensitivity reactions to the drug developed in 51 patients (67.1%). Sixty-four patients of the original 76 continued therapy, with 43 (67.2%) achieving a complete remission and 12 (18.8%) achieving a partial remission. Stage I disease responded significantly better than did subsequent, more severe disease stages. The median times to complete remission were 5.6 months, 32.3 months, and 22.3 months for stage I, II, and III disease, respectively. The conditions of patients with contact sensitivity did not respond better than those of patients without contact sensitivity. Patients with substage A disease did not respond better patients with substage B disease. These findings are encouraging and indicate that the use of topically applied mechlorethamine for early-stage MF should be continued, despite the development of contact dermatitis to the drug.  相似文献   

10.
【摘要】 目的 探讨利妥昔单抗(RTX)治疗天疱疮患者的长期疗效和安全性及对免疫指标的影响。方法 回顾性纳入2008年2月至2017年7月于北京大学第一医院皮肤科使用RTX(375 mg/m2体表面积,每周1次,连续使用4周)治疗的天疱疮患者。收集基线及不同随访时间点患者的自身抗体、B细胞水平,分析其变化趋势及与疗效的关系。采用Kaplan-Meier方法分析疾病控制、完全缓解和复发情况,对重复测量的免疫指标(自身抗体、B细胞)计算中位数M和P25、P75,绘制随访时间-免疫指标中位水平曲线。结果 共纳入53例汉族天疱疮患者,包括40例寻常型天疱疮和13例落叶型天疱疮,男女比例为0.96∶1,中位年龄37.4岁,基线时中位病程为13.4个月,中位随访时间(P25,P75)为37.5(25.0,54.7)个月。疾病控制48例(90.6%),疾病控制时间为1.7(1.1,3.2)个月。完全缓解38例(71.7%),完全缓解时间13.1(9.6,27.5)个月。随访期间完全缓解的38例中复发12例(31.6%),复发时间为12.4(4.8,19.8)个月。随访时间-免疫指标中位水平曲线显示,皮疹缓解时抗Dsg1和Dsg3自身抗体水平下降;皮疹复发时,自身抗体水平上升。最常见的重度不良反应为肺部感染,死亡率为3.8%(2/53)。结论 使用RTX治疗天疱疮患者的长期疗效显著,最需关注的不良反应为肺部感染,自身抗体水平可作为RTX治疗天疱疮的疗效观察指标。  相似文献   

11.
BackgroundLow-dose rituximab is a protocol used in several autoimmune diseases, that has also shown to be effective and safe in pemphigus vulgaris.ObjectivesTo study whether low-dose rituximab is also effective for bullous pemphigoid.MethodsPatients with BP were treated with a single cycle of two infusions of rituximab 500 mg at an interval of 2 weeks. Early and late end points were monitored.ResultsSix patients, five males and a female, with a mean age of 78.6 years (range 65–89) and a mean history of BP of 6.7 months (range 2–16) were included. A rapid and marked response was observed after a single cycle of treatment, with a mean time to disease control and to end of consolidation phase of 1.9 (range 1–3), and 4 weeks (range 3–5), respectively. Four patients achieved a late end point at a mean of 15.75 weeks (range 13–20). Three of them achieved partial remission with no therapy (two patients) or with minimal therapy (one patient), and one of them achieved complete remission with no therapy. One patient has 6 weeks of clinical follow-up after rituximab administration. The remaining patient relapsed 4 weeks after the rituximab treatment, and remains in complete remission with more than minimal therapy. One patient had a herpetic gingivostomatitis related to rituximab.ConclusionsLow-dose rituximab for BP achieved acceptable remission rates and steroid-sparing activity, with a better safety profile and a lower cost, compared to standard doses. This pilot study suggests that low-dose rituximab could be a therapeutic option for BP.  相似文献   

12.
BACKGROUND: Rituximab is a monoclonal antibody directed against the CD20 antigen expressed on B lymphocytes. There are reports of its efficacy in the treatment of autoimmune diseases, including pemphigus. OBJECTIVES: Prospectively to evaluate the efficacy of rituximab as adjuvant treatment for pemphigus vulgaris (PV). METHODS: Patients with PV were treated with intravenous rituximab (375 mg m(-2)) weekly for 4 weeks in this prospective open-label pilot study. Other concurrent immunosuppression was continued. RESULTS: Of five patients, one achieved complete remission and was able to cease all medication, while two achieved clearance of clinical lesions but continued on systemic therapy. Two patients had progressive disease. Time to response was 2-8 months, with a 13- to 18-month response duration. Response was associated with reduction in serum antiepithelial antibodies. Two patients had significant infectious complications (one developed community-acquired pneumonia associated with delayed-onset neutropenia and the other developed cytomegalovirus infection). CONCLUSIONS: Rituximab has shown efficacy in the treatment of PV. Patients on multiple immunosuppressives should be closely monitored for infectious complications.  相似文献   

13.
Background Pemphigus is a potentially fatal autoimmune epidermal bullous disorder. Various treatment modalities have been described to treat pemphigus. In cases where the disease fails to respond to conventional therapy, rituximab has been shown to be effective. Objective To study the efficacy of rituximab in the treatment of resistant or severe pemphigus in Indian patients. Methods Patients with pemphigus were treated with intravenous rituximab 1000 mg in adults or 375 mg/m2 body surface area in children by two doses, 15 days apart in this open labelled pilot study. Anti‐desmoglein1 (anti‐Dsg1) antibodies and anti‐desmoglein3 (anti‐Dsg3) antibodies were measured at the start of therapy and at the end of the follow‐up period. The outcome was studied in terms of control of disease activity (CD), complete remission (CR), partial remission (PR) and time to disease control (TDC) as defined by the consensus statement from the International Pemphigus Committee. Results A total of 9 (90%) of 10 patients responded to the treatment. Three (30%) had CR of disease and were off all treatment. Four (40%) patients had CR and were on low dose oral prednisolone. Two (20%) patients had PR and were on low dose prednisolone. One patient died of sepsis. The mean TDC was 8 weeks. Response to treatment showed good correlation with index values of anti‐Dsg1 antibody. Infusion‐related angioedema and sepsis were seen as complications due to rituximab administration. Conclusion Rituximab is effective in treating resistant and severe pemphigus in Indian patients. Acute complications can occur during rituximab infusion and require close monitoring.  相似文献   

14.
OBJECTIVE: To compare the efficacy of oral psoralen-UV-A (PUVA) therapy with that of narrowband UV-B (NB-UVB) therapy in patients with chronic plaque psoriasis. DESIGN: Double-blind randomized study. SETTING: Phototherapy unit in a university hospital.Patients Ninety-three patients with chronic plaque psoriasis.Interventions Twice-weekly NB-UVB or PUVA therapy, starting at 70% of the minimum phototoxic or erythema dose, with 20% incremental increases. Patients were treated until clearance, up to a maximum of 30 sessions; those with clearance were followed up until relapse or for 12 months. MAIN OUTCOME MEASURES: Proportion of patients achieving clearance, number of treatments to clearance, and, among those with clearance, the proportion remaining in remission at 6 months. RESULTS: Patients with skin types V and VI had a lower rate of clearance than those with skin types I through IV (24% vs 75%; P = .001). In patients with skin types I through IV, PUVA was significantly more effective than NB-UVB at achieving clearance (84% vs 65%; P = .02). The median number of treatments to clearance was significantly lower in the PUVA group (17.0 vs 28.5; P<.001). More patients treated with PUVA vs NB-UVB were reported to have erythema at some stage during treatment (49% vs 22%; P = .004), although this difference may have been due to ascertainment bias. Six months after the cessation of therapy, 68% of PUVA-treated patients were still in remission vs 35% of NB-UVB-treated patients.Conclusion Compared with NB-UVB, PUVA achieves clearance in more patients with fewer treatment sessions and results in longer remissions.  相似文献   

15.
Background Rituximab, an anti‐CD20 antibody, was shown in open series studies to be effective in treating pemphigus at a dose of 4 × 375 mg m?2 as approved for B‐cell malignancies. Objectives We investigated whether a lower dose of rituximab is also effective for pemphigus. Methods Patients with pemphigus were treated with a single course of two infusions of rituximab (500 mg each) at an interval of 2 weeks. Clinical consensus late end points, B‐cell number, desmoglein 1 and desmoglein 3 indices were monitored. Results We enrolled 15 patients in the study: three with pemphigus foliaceus (PF) and 12 with pemphigus vulgaris (PV). The follow‐up was 32–152 weeks (median 94). All 15 patients responded to therapy. Eight patients achieved complete remission in a median period of 51 weeks (four on minimal therapy, four off therapy). Seven patients achieved partial remission in a median period of 34·5 weeks (five on minimal therapy, two off therapy). Relapses (40%) were seen between 53 and 103 weeks (median 97) after start of therapy. B‐cell numbers dropped to < 1% after first infusion, and remained undetectable in patients with sustained remission. The antidesmoglein 1 index correlated well with the clinical severity in PF, but this was less obvious in PV. Conclusions A low dose of rituximab is an effective and safe treatment for pemphigus. Relapses may occur, mostly at the end of the second year. Cost–effectiveness studies with a long follow‐up are required to determine the proper dosage of this expensive drug in pemphigus.  相似文献   

16.
Background: Cutaneous T-cell lymphoma (CTCL) may respond to many therapies, but long-term disease-free survival is uncommon. Patients with advanced disease have a median survival of approximately 3 years.Objective: Our purpose was to combine known effective agents sequentially to determine whether we could achieve remission in more patients or for longer duration.Methods: Patients with mycosis fungoides (n = 23) or Sézary syndrome (n = 5) were treated with 4 months of recombinant interferon alfa together with isotretinoin, followed by total skin electron beam therapy alone (for stage I to II disease) or preceded by chemotherapy (for stage III to IV disease). Maintenance therapy consisted of interferon for 1 year and topical nitrogen mustard for 2 years.Results: Twenty-eight patients were treated. The overall response rate (complete and partial remissions) was 82%. Although the median duration of remission was 5 months in patients with stage III to IV disease, two patients remain in complete remission at 39+ and 46+ months. In patients with stage I to II disease the median duration of remission has not been reached at a median follow-up of 18 months. Five patients, all with stage III to IV disease, have died. Overall, the regimen was well tolerated with one treatment-related death from neutropenic sepsis.Conclusion: Combined modality therapy may be effective for the treatment of CTCL with similar response rates to other current therapies.  相似文献   

17.
We conducted a retrospective investigation of the efficacy and safety of dabrafenib and trametinib combination therapy in Japanese patients with BRAF V600 mutation-positive advanced melanoma in real-world clinical practise. The study analyzed 50 patients who received dabrafenib and trametinib combination therapy for BRAF V600 mutation-positive advanced melanoma in our hospital (26 men and 24 women, aged 21–86 years, inclusive; median age, 53 years). The response rate was 72.3%, with complete response (CR) achieved in eight cases (17.0%), partial response in 26 (55.3%), stable disease in nine (19.1%) and progressive disease in four (8.5%). Median progression-free survival (PFS) was 12 months, and median overall survival (OS) was 23 months. Disease progression occurred in 29 of the 50 patients during the study period, and 25 patients died. Baseline lactate dehydrogenase and the number of organs with metastasis were important predictive factors for PFS and OS, and CR to combination therapy was a predictive factor for long-term remission. Adverse events occurred in 88% of cases; 16% were grade 3 or worse. The adverse events observed in 50% of more of patients were rash (56%) and pyrexia (52%). The efficacy of dabrafenib and trametinib combination therapy in Japanese patients was similar to that reported in global studies, and the same adverse events were generally reported; however, rash tended to occur more frequently in the patients in our study.  相似文献   

18.
Atopic dermatitis (AD) is a common disease. Severe AD has a significant effect on quality of life and is difficult to treat. We have previously reported 10 patients treated with azathioprine over a 7-year period, to good effect. We have now treated 37 patients over 18 years, and report our findings. The aim of the study was to review the efficacy and safety of azathioprine as a long-term treatment for severe atopic eczema. Patients who began treatment between 1987 and 2005 were identified. The dose and number of courses of azathioprine, duration of treatment, treatment response, and adverse events were recorded up to June 2006. In total, 37 patients were treated with azathioprine. Of these, 15 (40.5%) achieved remission in a median period of 5 months (range 4-29). Nine patients had an initial good response but either did not have sufficient clearance to be able to stop azathioprine, or deteriorated while on treatment over a period of 4-27 months; these were considered late failures. One patient showed no response, and five experienced reactions leading to withdrawal of the drug. Our experience with azathioprine for the treatment of severe atopic eczema, is very encouraging.  相似文献   

19.
BACKGROUND: Dermatomyositis is an autoimmune disease that is associated with muscle and skin inflammation. Using quantitative scales, we sought to evaluate the effects of rituximab therapy on muscle strength and skin disease in patients with dermatomyositis. OBSERVATIONS: An open-label trial of rituximab therapy was conducted in 8 adult patients with dermatomyositis. Patients received 2 infusions of rituximab (1 g each) 2 weeks apart without peri-infusional steroids. The primary outcome was partial remission at week 24 (prespecified reduction in elevated creatine phosphokinase levels, muscle strength deficit (Manual Muscle Test), or skin disease (Dermatomyositis Skin Severity Index). After the first infusion of rituximab, all patients achieved sustained depletion of peripheral B cells. One patient withdrew at week 16 owing to a lack of treatment efficacy. Three patients (38%) achieved partial remission at week 24, in each case by improvement in muscle strength. Muscle enzyme levels and skin scores at week 24 were not significantly changed from those at baseline. Rituximab infusions were well tolerated, with no serious infectious complications. One patient died of metastatic cancer 9 months after his last infusion. CONCLUSION: Depletion of peripheral B cells had modest effects on muscle disease and limited effects on skin disease in our cohort of patients with dermatomyositis.  相似文献   

20.
BACKGROUND: Mycophenolate mofetil is increasingly being used as a corticosteroid-sparing agent in immunosuppressive regimens. OBJECTIVE: To elucidate the effectiveness of mycophenolate as adjuvant therapy in the treatment of both pemphigus vulgaris and pemphigus foliaceus. DESIGN: Historical prospective study. SETTING: University hospital. PATIENTS: The study included 42 consecutive patients with pemphigus (31 with pemphigus vulgaris and 11 with pemphigus foliaceus) who had relapses during prednisone taper or had clinically significant adverse effects from previous drug therapy. RESULTS: Remission was achieved in 22 (71%) and 5 (45%) of patients with pemphigus vulgaris and pemphigus foliaceus, respectively. Partial remission was achieved in 1 (3%) and 4 (36%), respectively. The median time to achieve complete remission was 9 months (range, 1-13 months). The treatment was administered for a median of 22 months, and the median follow-up period was 22 months. Seventy-seven percent of patients had no adverse effect. Two patients had side effects severe enough to necessitate discontinuation of treatment, one because of symptomatic but reversible neutropenia and the other because of nausea. CONCLUSION: Mycophenolate is an effective and safe adjuvant in the treatment of both pemphigus vulgaris and pemphigus foliaceus.  相似文献   

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