ANCA相关性肾小球肾炎合并IgG4相关性肾小管间质性肾炎一例

抗中性粒细胞胞质抗体（ANCA）相关性血管炎常累及肺肾等器官,该文报道1例ANCA相关性肾小球肾炎（ANCA-GN）合并IgG4相关性肾小管间质性肾炎（IgG4-TIN）病例,患者为51岁男性,以发热伴咳嗽起病,伴尿量减少,蛋白尿、血尿不明显,其血清学髓过氧化物酶滴度明显升高、核周型ANCA阳性,IgG4轻度升高（肾上腺皮质激素使用后）,肾脏病理学检查示有毛细血管袢断裂、皱缩伴新月体形成,肾间质可见较多淋巴细胞、浆细胞浸润伴轻度纤维化,免疫组织化学染色浆细胞显示以IgG⁺及IgG4⁺浆细胞为主,存在ANCA-GN合并IgG4-TIN可能,予抗感染、抗血管炎及血浆置换后患者病情好转出院。该例的诊治提示,对于不典型ANCA-GN患者如无明显血尿、蛋白尿、尿量减少等表现,需检测血清IgG4,病理上需注意观察有无IgG4-TIN特征性改变;对于临床上高度可疑的IgG4-TIN患者,还需同时排查IgG4所致的其他脏器改变,基线血清IgG4水平是IgG4相关性疾病活动、预测复发的标志物。     

Systemic lupus erythematosus after coronavirus disease-2019 (COVID-19) infection: Case-based review

BackgroundCoronavirus disease-2019 (COVID-19) is a novel infectious disease, which presents with various clinical manifestations. There is growing evidence of an association between COVID-19 infection and autoimmune diseases. The aim of this case report was to demonstrate the association of COVID-19 infection and the development of systemic lupus erythematosus (SLE).Case presentationA 38 year old Iranian woman presented with progressive icterus, pleuritic chest pain, palpitation, dyspnea, photosensitivity and arthralgia 18-days after COVID-19 symptoms proved by a positive polymerized chain reaction (PCR). The chest and abdomen computerized tomography (CT) scan showed pericardial and pleural effusion and enlarged liver and abdominal lymph nodes. Antinuclear antibody (ANA), anti-double stranded deoxyribonucleic acid (anti-ds DNA) antibody and perinuclear anti-neutrophil cytoplasmic antibody (P-ANCA) were positive. She was diagnosed as SLE and was successfully treated with prednisolone 30 mg daily, hydroxychloroquine 200 mg daily and azathioprine 150 mg daily and she remarkably improved. Repeated anti-ds DNA antibody was positive. Due to nausea and abdominal discomfort, azathioprine was discontinued and replaced with mycophenolate mofetil 1500 mg daily. In the article, similar cases were presented; the mean interval between COVID symptoms and SLE presentations was 24.86 days. Pulmonary and renal involvements were the most common presentations of SLE triggered by COVID-19. The most frequently reported autoantibody was ANAConclusionIt is necessary to be aware of the development of lupus disease in COVID-19 infected patients, because prompt diagnosis and treatment is very important to improve their outcome.     

Extreme gonococcal susceptibility associated with acquired complement deficiency secondary to hypocomplementemic urticarial vasculitis and systemic lupus erythematosus

Gonococcal infection is rarely associated with septic shock. We describe a recurrent case of septic shock related to disseminated gonococcemia in a patient with systemic lupus erythematosus and hypocomplementemic urticarial vasculitis, and discuss the implication of profound acquired complement deficiency secondary to these auto-immune diseases. This case raises the question of systematic antibioprophylaxis in patients with acquired complement deficiency.     

Tumor‐forming plasmacytoid dendritic cells associated with myeloid neoplasms. Report of a peculiar case with histopathologic features masquerading as lupus erythematosus

Plasmacytoid dendritic cells (PDC) belong to a subtype of dendritic cells that are normally absent in healthy skin. In some inflammatory diseases of the skin, especially lupus erythematosus (LE), these cells are occasionally recruited in great amounts, which can be used as a helpful clue for diagnosis. Rarely, PDC may also accumulate in the skin of patients with myeloid leukemia, a yet poorly known condition currently called ‘tumor‐forming PDC associated with myeloid neoplasms’. In this study, we describe a patient with unsuspected chronic myelomonocytic leukemia who developed cutaneous lesions characterized by a dermal infiltrate rich in PDC. Similarly to LE, such neoplastic PDC were accompanied by interface dermatitis‐like changes, but displayed an aberrant phenotype and shared the same chromosomal abnormality with the leukemic cells identified in the bone marrow, thus revealing the neoplastic nature of the process. This observation illustrates that tumor‐forming PDC associated with myeloid neoplasms may microscopically mimic LE in some patients. Accordingly, a hematologic workup is recommended in any skin lesion featuring excessive numbers of PDC, even if morphological alterations suggestive of interface dermatitis are found.     

Reasons for failure to receive pneumococcal and influenza vaccinations among immunosuppressed patients with systemic lupus erythematosus

ObjectiveTo better understand why immunosuppressed individuals with systemic lupus erythematosus (SLE) fail to receive influenza and pneumococcal vaccines.MethodsThese cross-sectional data were derived from the 2009 cycle of the Lupus Outcomes Study (LOS), an annual longitudinal telephone survey of individuals with confirmed SLE. Respondents were included in the analysis if they had taken immunosuppressive medications in the past year. We assessed any prior receipt of pneumococcal vaccine and influenza vaccine in the past year, and then elicited reasons for not receiving vaccination. We used bivariate statistics and multivariate logistic regression to assess frequency and predictors of reported reasons for not obtaining influenza or pneumococcal vaccines.ResultsAmong 508 respondents who received immunosuppressants, 485 reported whether they had received vaccines. Among the 175 respondents who did not receive an influenza vaccine, the most common reason was lack of doctor recommendation (55%), followed by efficacy or safety concerns (21%), and lack of time (19%). Reasons for not receiving pneumococcal vaccine (N = 159) were similar: lack of recommendation (87%), lack of time (7%), and efficacy or safety concerns (4%). Younger, less-educated, non-white patients with shorter disease duration, as well as those immunosuppressed with steroids alone, were at the greatest risk for not receiving indicated vaccine recommendations.ConclusionsThe most common reason why individuals with SLE did not receive pneumococcal and influenza vaccines was that physicians failed to recommend them. Data suggest that increasing vaccination rates in SLE will require improved process quality at the provider level, as well as addressing patient concerns and barriers.     

系统性红斑狼疮中医证型与IFN-γ、IL-2、IL-4、IL-10的相关性研究

目的观察系统性红斑狼疮(Systemic lupus erythematosus,SLE)各中医证型患者外周血清中γ-干扰素(IFN-γ)、白细胞介素(IL)-2、IL-4、IL-10的水平,以探求SLE中医辨证分型的客观化参考指标。方法根据中医辨证将SLE分为4种证型,气血热盛型、脾肾阳虚型、气阴两虚血瘀型和气滞血瘀肝郁型,采用酶联免疫双抗体夹心(ELISA)法,检测并比较不同证型SLE患者及正常对照组血清中IFN-γ、IL-2、IL-4、IL-10水平的差异。结果 SLE患者外周血清IFN-γ、IL-4、IL-10的水平高于正常对照组(P0.01),IL-2的水平低于正常对照组(P0.01);从气血热盛型、脾肾阳虚型、气阴两虚血瘀型到气滞血瘀肝郁型IFN-γ、IL-4、IL-10的水平依次递减,IL-2为依次递增,其中气血热盛型、脾肾阳虚型与气阴两虚血瘀型和气滞血瘀肝郁型相比,差异有统计学意义(P0.01)。结论血清中IFN-γ、IL-4、IL-10和IL-2水平可作为SLE中医辨证分型的客观化参考指标。     

环磷酰胺与泼尼松联合治疗狼疮性肾炎的疗效观察

目的：探讨环磷酰胺（ cytoxan，CTX）与泼尼松（ methylprednisolone，MP）联合治疗狼疮性肾炎（ lupus nephritis，LN）的临床疗效与安全性。方法：随机抽取2013年1月—2014年1月于汕头潮南民生医院就诊的LN患者90例，以随机数字表法分为观察组和对照组各45例。观察组患者给予CTX与MP联合治疗，对照组给予MP单药治疗，比较2组患者的临床疗效与安全性。结果：观察组患者的总有效率为93．33％（42／45），显著高于对照组的73．33％（33／45）（P＜0．05）；治疗后，观察组患者的尿蛋白、血沉以及血清肌酐水平均显著低于对照组（P＜0．05），血红蛋白及补体C3水平均显著高于对照组（P＜0．05）；观察组患者不良反应发生率为13．33％（6／45），与对照组的6．67％（3／45）比较，差异无统计学意义（P＞0．05）。结论：CTX与MP联合治疗LN，可改善患者临床症状及肾功能，减少蛋白尿，提高临床疗效，且不良反应较少，值得临床推广。