结节性脂膜炎30例临床分析

目的　总结结节性脂膜炎的临床特点。方法　总结 30例经病理证实的脂膜炎患者的临床资料。结果　本病多发于中青年 ,主要表现有皮下浸润性伴自觉痛及触痛的结节或斑块 (10 0 % )、发热 (90 % )。临床常分为皮肤型和系统型 ,后者可伴有呼吸、循环、消化、血液系统脏器受累及实验室检查异常。治疗应祛除诱因 ,系统型需加用皮质激素、免疫抑制剂。预后一般良好 ,死亡常因感染、严重脏器受累和出血。结论　脂膜炎病因不清 ,临床表现多样 ,皮肤型较系统型患者预后良好。     

中药治愈脂膜炎1例

脂膜炎又称回归型发热性结节性非化脓性脂膜炎,为一反复发作的皮下脂肪组织局限性炎性包块或结节性少见疾病,病原不明,激素治疗易复发,我们采用中药治愈一例。     

45例原发性膜性肾病治疗的探讨

目的通过45例原发性膜性肾病(IMN)患者的治疗随访,探讨IMN的治疗.方法Ⅰ组24h尿蛋白≤5g的肾病综合征(NS)患者12例,采用血管紧张素转换酶抑制剂(ACEI)+抗血小板粘附剂和(或)抗凝治疗.Ⅱ组24h尿蛋白＞5g的患者33例,采用糖皮质激素(激素)+免疫抑制剂治疗,其中ⅡA组(26/33)糖皮质激素+环磷酰胺冲击治疗.ⅡB组(7/33)Ponticelli方案激素与瘤可宁交替治疗,共6个月.结果Ⅰ组平均随访32.6(7～60)个月,67%患者缓解(完全+部分缓解),33%未缓解,无一例出现肾功能不全.ⅡA组平均随访28.1(6～74)个月,58%出现缓解(部分或完全),42%未缓解,其中慢性肾功能不全2例(一例死于心衰与肺部感染).ⅡB组随访36(24～48)个月,67%缓解(完全+部分缓解),33%未缓解.结论24h尿蛋白≤5g的NS患者或有NS倾向者,可不用激素+免疫抑制剂治疗,仅用ACEI+抗血小板粘附剂或(和)抗凝治疗,有67%的缓解.24h尿蛋白＞5g的NS患者,需用激素+免疫抑制剂治疗,兼以抗血小板粘附或(和)抗凝治疗,免疫抑制剂CTX和瘤可宁均有效,二者无明显差异.     

白塞病眼病111例临床分析

 目的 分析白塞病眼部病变的临床特点,提高对该病的认识。方法 回顾性分析北京协和医院2002—2012年收治的白塞病眼病患者的临床资料。结果 白塞病眼病111例,男性患者多见,男∶女为2.08∶1。白塞病发病年龄(27.6±10.8)岁,发生眼病时年龄(31.5±10.7)岁;发生眼病时白塞病的中位病程24(0,372)个月。眼部受累:85.6%的患者双眼受累,66.7%的患者视力下降,且病程中视力0.1以下占60.4%;葡萄膜炎多见,占90.10%,其中全葡萄膜炎占62.16%。男性患者出现视力下降(57例)、增殖性视网膜病变(8例)多于女性(17例;0例),白内障发生率(58.67%)、HLA-B₅₁阳性率(14/21)高于女性(36.11%;1/9)(P＜0.05),而皮肤黏膜、内脏器官受累等眼外表现男女间差异无统计学意义。患葡萄膜炎者均予糖皮质激素、免疫抑制剂治疗, 77例患者视力改善,49例患者反复发作,28例(25.5%)致盲。糖皮质激素、免疫抑制剂治疗无效或反复者(19例)给予生物制剂治疗,其中12例症状好转,2例无效,5例仍反复发作。结论 白塞病眼病以男性多见,多为葡萄膜炎,且临床表现较重,并发症多,HLA-B₅₁阳性率高。葡萄膜炎对糖皮质激素、免疫抑制剂治疗有效,但易复发,致盲率高。TNFα 拮抗剂对复发性葡萄膜炎部分有效。     

重组人干扰素α2a治疗难治性白塞综合征的疗效及安全性

目的探讨重组人干扰素α2a(interferonα2a,IFN-α2a)治疗难治性白塞综合征(Beh et syndrome,BS)的疗效和安全性。方法回顾性分析北京协和医院2014年11月至2019年7月收治的9例BS患者因顽固性黏膜溃疡、并血管或神经系统受累,接受IFN-α2a治疗的临床资料。结果9例患者(包括7例男性和2例女性)平均年龄(36.55±5.03)岁,病程(122±84.45)个月。其中顽固性黏膜溃疡3例,反复多发静脉血栓1例,多发下肢动脉狭窄、闭塞,下肢深静脉血栓伴发血栓后综合征1例,神经系统受累3例(1例并发顽固黏膜溃疡),另1例同时并神经、血管、眼受累,经激素联合免疫抑制剂积极治疗后临床无明显改善,且4例并发乙肝病毒感染,1例并发肺结核。接受IFN-α2a治疗后,平均随访(9.78±2.72)个月,所有患者临床症状均有改善,皮质类固醇激素使用剂量减少,随访6个月时中位泼尼松剂量从10(0,60)mg逐渐减少到5(0,15)mg,末次随访中位泼尼松剂量为4.375(0,12.5)mg(P<0.05);中位免疫抑制剂种类从2(1,3)种逐渐减少至1(0,2)种,末次随访中位免疫抑制剂种类为1(0,1)种(P<0.05)。1例(12.5%)患者停用激素,3例(33.3%)患者停用免疫抑制剂。结论IFN-α2a与皮质类固醇激素和免疫抑制剂联合使用,对治疗难治性BS有效且耐受性良好,有利于皮质类固醇激素和免疫抑制剂减量。     

免疫抑制剂治疗顽固性葡萄膜炎6例报告

顽固性葡萄膜炎为累及全身多系统的自身免疫性疾病,不易治愈,可导致患者视功能严重受损。近5年来,我院对6例顽固性葡萄膜炎反复发作者应用免疫抑制剂联合糖皮质激素进行治疗,效果满意。现报告如下。     

ANCA相关性血管炎患者的远期疗效分析

目的 了解抗中性粒细胞胞浆抗体相关性血管炎(antineutrophil cytoplasmic antibody-associated vasculitis,ANCA)患者免疫抑制治疗的远期疗效.方法 回顾分析在我院随访5年以上的所有ANCA相关性血管炎(AAV)患者的随访资料,计算其确诊时及每次随访时的伯明翰血管炎活动评分(BVAS),并就疾病活动性与时间变化及免疫抑制治疗等因素进行相关性评估.结果 共9例患者,这些患者均接受了糖皮质激素和免疫抑制剂的联合治疗,初治时的激素剂量相当于泼尼松(73.9±37.6)mg/d.治疗的第一个半年内缓解率达100％,BVAS在治疗后4年内稳定地降低,但第5年起又开始上升,以后出现反复波动.仅1例长期维持缓解,其余均有复发,其中5例表现为反复发作.平均首次复发时间在治疗后(54.6±23.3)m.复发的危险因素依次为无免疫抑制治疗、生存时间超过5年以及糖皮质激素相当于泼尼松≤10 mg/d.药物副作用包括激素相关糖尿病、骨质疏松、严重感染等,主要发生在前14月内.结论 ANCA相关性血管炎复发率高,多数患者需激素和/或免疫抑制剂不间断的长期维持治疗.     

复发性多软骨炎20例临床分析

目的：提高对复发性多软骨炎的认识。方法：收集并分析20例复发性多软骨炎患者的临床和实验室检查资料。结果：发病年龄35－86岁，平均53．4岁，平均病程1．1年。90％的患者均有耳软骨炎；80％有呼吸系统受累，其中75％有鼻软骨炎，4例出现严重的气管、支气管软骨炎；部分患者有关节、皮肤、心脏、血管和神经系统受累；20％合并有其他风湿性疾病或自身免疫病。结论：气管、支气管软骨受累是病情严重的信号。早期诊断，给予激素和免疫抑制剂治疗是改善预后的关键。     

组织细胞吞噬性脂膜炎一例

组织细胞吞噬性脂膜炎(cytophagic histocytic panniculitis．CHP)是一种病因不明的疾病．病理学特征为良性组织细胞浸润脂肪组织，产生㈩血、坏死性脂膜炎。本病可有多器官受累．表现为反复发热、浆膜炎、肝脾肿大等，为复发性结节性非化脓性脂膜炎(Weber-Christian综合征)的一个亚型。本文报告1例CHP的诊治经过．并进行相关文献复习．以期望进一步提高临床医师对CHP的认识。     

干燥综合征伴弥漫性肺泡出血1例

弥漫性肺泡出血是SS的一种罕见而危险的肺部表现, 具有病情凶险、进展迅速的特点, 国内外鲜有报道。本文报道1例SS伴弥漫性肺泡出血的青年女性患者, 既往有视神经脊髓炎病史, 表现为反复咯血、气促、晕厥, 予甲泼尼龙40 mg治疗后肺泡出血控制欠佳, 后经甲泼尼龙0.5 g/d冲击等治疗后病情缓解, 随访3年无明显疾病进展。CTD伴弥漫性肺泡出血需注意早期识别、尽早足量激素及免疫抑制剂治疗。     

Haemophagocytic syndrome and cutaneous T-cell lymphoma

Haemophagocytic syndrome is a rare disorder of immune regulation. Clinical features are fever, haemophagocytosis, hepatosplenomegaly, pancytopenia, lymph nodes enlargement, hypertriglyceridaemia and coagulopathy. Systemic findings may also include pulmonary infiltrates, renal failure and an inappropriate antidiuretic state. It has been described associated to many diseases, like lymphomas, most of them T-cell lymphomas, infections and systemic diseases, among other entities. Haemopoietic cells are actively ingested by monocytes and macrophages in lymph nodes, bone marrow, liver and spleen. This is a diagnostic criteria for haemophagocytic syndrome. The treatment of haemophagocytic syndrome is difficult and requires intensive supportive therapy, treatment of coagulopathy disorders, high-dose corticosteroids and immunosuppressive treatment, although in most cases there is no response to treatment and the evolution is fatal. The T-cell lymphomas that involve subcutaneous tissue and simulate panniculitis are classified recently as primary cutaneous T-cell lymphomas, and their presentation as inflammatory nodules of the legs are uncommon.     

Weber-Christian disease with ileocolonic involvement successfully treated with infliximab

Weber-Christian disease(WCD) is an inflammatory disease whose main histological feature is lobular panniculitis of adipose tissue. The location of panniculitis determines the clinical presentation,being the subcutaneous adipose tissue the most frequent one,followed by liver,spleen,bone marrow and mesenteric adipose tissue.Systemic corticosteroids are first line treatment,but other options should be considered if systemic symptoms are observed or in case of refractory clinical situation.We report herein a case with WCD showing orbital,mesenteric and ileocolonic involvement,which required surgical treatment and also developed postoperative recurrence.Symptoms were resolved by administration of thalidomide and,afterwards,infliximab.To our knowledge,this is the first report of Weber-Christian disease with luminal ileocolonic involvement,treated with infliximab.     

Primary Cutaneous Plasmacytoma after Rejection of a Transplanted Kidney: Case Report and Review of the Literature

Immunosuppressed organ allograft recipients are at risk of developing lymphomas and lymphoproliferative disorders as a consequence of immunosuppressive therapy and long-term antigenic stimulation from both the graft and possible viral infections. No more than 4% of the malignant tumors detected in organ recipients are plasmacytomas. Primary cutaneous plasmacytoma is a rare type of cutaneous B-cell lymphoma arising primarily in the skin. It is derived from clonally expanded plasma cells with various degrees of maturation and atypia. We report the occurrence of a solitary cutaneous plasmacytoma in a 56-year-old male patient undergoing hemodialysis after rejection of a grafted kidney. The diagnosis was made a few months after the kidney had been surgically removed. A thorough examination showed no evidence of systemic disease. Skin lesions were successfully treated with local radiotherapy. After 2 years of follow-up there were no local or systemic recurrences.     

Corticosteroid therapy in an additional 13 cases of Stevens-Johnson syndrome: a total series of 67 cases.

Stevens-Johnson syndrome (SJS) is a severe cutaneous eruption that can be a life-threatening emergency. Previously, we have reported our favorable experience in treating 54 patients with SJS with systemic corticosteroids. We continued our prospective analysis of consecutive patients with SJS treated with corticosteroids. Possible etiologic factors and clinical outcomes of the patients are described. All 13 patients improved with initiation of systemic corticosteroid therapy. There was no mortality or permanent sequelae attributable to SJS. Drugs were the offending agents in all 13 cases. There was one death unrelated to SJS. In conclusion, prompt treatment with systemic corticosteroids reduces morbidity and improves outcome of SJS patients. This analysis extends our series to 67 consecutive patients with SJS who were treated with corticosteroids and had a favorable outcome.     

Resistance index in differential diagnosis of liver lesions by color doppler ultrasonography

AIM: To investigate the specific value of resistance index (RI) in color Doppler ultrasonography in the diagnosis of focal hepatic lesions. METHODS: Eight hundred patients with 893 hepatic solid lesions were studied with color Doppler flow imagimg (CDFI) and pulsed Doppler, induding 644 malignant cases (596 primary malignant liver tumors, and 48 metastatic liver tumors), 156 benign cases. All were confirmed by operation and pathology. RESULTS: The detection rate of arterial flow in malignant tumors was 92%, and 52% in benign lesions. Doppler spectrum analysis showed that the resistance index in primary malignant tumors was 0.75&#177;0.12, 0.73&#177;0.09 in metastatic tumors, and was below 0.6 in benign lesions. The difference was significant (P&lt;0.001). This difference was related with its histopathologic structure. CONCLUSION: The arterial flow with RI≥0.6 identified by CDFI within the liver lesion can be regarded as a criterion of malignant tumors, RI&lt;0.6 can be regarded as benign disorders. RI is useful in differential diagnosis of liver neoplasms.     

Non-respiratory tuberculosis with Mycobacterium tuberculosis after penetrating lesions of the skin: five case histories.

Tuberculosis is primarily transmitted from person to person via the respiratory route. We describe five cases of patients who developed tuberculosis at the site of a skin injury: three after being treated repeatedly with local corticosteroids via intramuscular injections, and two who cut themselves accidentally with a knife. All cultures yielded normal-sensitive Mycobacterium tuberculosis, and all patients responded well to anti-tuberculosis treatment. These unusual manifestations of non-respiratory tuberculosis may support the assumption that persistent, painful, reddish and/or fistulous areas of the skin might also indicate an infection caused by M. tuberculosis, via either reactivation of pulmonary tuberculosis or primary infection with M. tuberculosis by cutaneous transmission.     

27例血行播散型结核病临床分析

目的 通过对27例血行播散型结核病临床资料的分析,提高对该病的认识。方法 回顾性分析1961～2000年我院收治的尸检证实的27例血行播散型结核病的临床资料。结果 本组患者临床表现多不典型,呼吸道症状隐匿,以发热为主要表现,部分患者可有肝、脾肿大,皮疹,血三系减低。胸部影像学以浸润性改变为主,空洞少见,粟粒性结节影出现较晚。结核菌素试验多阴性。病原学检测阳性率低。尸检证实,急性血行播散型结核病22例(包括无反应性结核3例),慢性血行播散型结核病5例。均合并活动性肺结核,肺外病变主要累及肝、脾、肾、淋巴结等部位。生前误诊12例,误诊率为44．4％。12例中有11例(92％)因伴发或疑为结缔组织病、血液系统疾病或肿瘤而误诊。27例患者中17例(63％)长期应用激素或反复化疗导致结核病播散。结论 临床医师应加强对结核病的警惕性,特别是有结核病史、免疫缺陷(包括长期使用激素或化疗)病史或合并营养不良的患者。出现长期发热和(或)有多系统损害、原发病经正规治疗无缓解时,应警惕耐多药结核的可能。应严格掌握糖皮质激素使用的适应证,未确诊前不能盲目应用糖皮质激素,以免诱发结核复发或血行播散。     

The prevalence of tuberculosis and positive tuberculin skin tests in a steroid-treated asthmatic population.

The prevalence of active tuberculosis and positive tuberculin skin tests was assessed in 132 corticosteroid-treated asthmatics for an evaluation period that represented 620 corticosteroid-years of therapy. There was no evidence of active tuberculosis in any of these patients. Positive intermediate-strength tuberculin tests were present in 28% of patients and correlated with increased age. In patients treated with daily corticosteroids, tuberculin negativity was associated with a higher dose of corticosteroids. Positive second-strength tuberculin tests were found in 42.3% of the patients with negative intermediate-strength tests and also correlated with increased age. Based on these data as well as the prevalence of significant liver disease in older patients receiving isoniazid (INH), routine INH chemoprophylaxis for corticosteroid-treated asthmatic patients, regardless of their tuberculin cutaneous reactivity, is not indicated.     

Mycobacterium tuberculosis infection in patients with systemic rheumatic diseases. A case-series.

OBJECTIVE: To describe the clinical characteristics of patients with systemic rheumatic diseases and tuberculosis. A retrospective case series from 1987 to 1994, drawn from a tertiary-care hospital in Mexico City, was studied. RESULTS: Thirty patients were included (20 women, 10 men), with mean age of 39.8 years (range 14-66), and a mean duration of the systemic rheumatic disease of 44 months (1-372). The rheumatic diseases included systemic lupus erythematosus (SLE) (n = 13), rheumatoid arthritis (7), polymyositis or dermatomyositis (5), and other diseases (5). During the six months previous to the diagnosis of tuberculosis, 22 patients had received corticosteroids, and 13 others immunosuppressants. Mycobacterium tuberculosis was isolated from 18 patients. Pulmonary tuberculosis was found in 10 patients, and extrapulmonary tuberculosis in 20, seven of these with miliary disease. SLE was seen in 6 of the patients with miliary tuberculosis. The clinical manifestations were: fever (67%), weight loss (67%), diaphoresis (60%), cough and sputum (53%), lymph node enlargement (43%), and dyspnea (33%). Sixteen patients had an abnormal chest film. Of 18 patients tested by PPD RT-2, 8 had an induration > 10 mm. Patients were initially treated with 3 or 4 anti-tuberculosis drugs for 15 days to 6 months, followed by 6 to 10 months of isoniazid plus rifampicin. Three relapsed, and 2 died of respiratory failure. CONCLUSIONS: This case series showed a particular pattern of tuberculosis in patients with systemic rheumatic diseases.     

Serum thrombopoietin in chronic liver disease: relation to severity of the disease and spleen size

BACKGROUND/AIMS: Thrombopoietin is a thrombopoietic factor mainly synthesized in the liver. Its production is regulated by the mass of megakaryocytes and platelets. Impaired production of thrombopoietin may be responsible for thrombocytopenia in chronic liver disease. METHODOLOGY: We studied thrombopoietin serum concentration in 68 patients with chronic liver disease of various degrees (39 with thrombocytopenia), in 5 patients with thrombocytopenia due to hematological disease, and in 27 healthy controls. RESULTS: Thrombopoietin concentration was higher in patients with liver disease than in controls. Patients with hematological disease had much higher thrombopoietin concentration than patients with liver disease. Among patients with liver disease and thrombocytopenia, thrombopoietin concentration was higher in cirrhosis than in chronic hepatitis. A negative correlation was found between platelet counts and spleen size and between thrombopoietin concentration and spleen size. No correlation was found between thrombopoietin concentration and liver disease severity. CONCLUSIONS: Patients with liver disease and thrombocytopenia have serum thrombopoietin concentration higher than normal controls. It seems therefore that the liver, even seriously diseased, maintains the ability to produce thrombopoietin. In the liver patients the number of circulating platelets and the serum levels of thrombopoietin are inversely correlated with the size of the spleen suggesting that thrombopoietin, although normally produced, might be turned over in platelets sequestrated in the spleen.