复发性多软骨炎合并强直性脊柱炎一例

复发性多软骨炎是一种累及多系统、反复发作的自身免疫性疾病,临床表现多种多样,缺乏特异性诊断指标,易误诊或漏诊。治疗上以NSAID、糖皮质激素和免疫抑制剂为主,生物制剂也有一定的疗效。该文报道1例复发性多软骨炎合并强直性脊柱炎患者,使用生物制剂、糖皮质激素和免疫抑制剂联合治疗,随访2年病情稳定。该例提示,应提高对复发性多软骨炎合并强直性脊柱炎的认识,提高诊治能力,治疗中应密切监测疗效和不良反应。     

Unusual manifestations of histoplasmosis

We present 6 cases of infection with Histoplasma capsulatum that have been uncommonly or not previously reported. Our case reports include unusual manifestations of H. capsulatum presenting as meningitis, the immune reconstitution syndrome, relapsing pericarditis, and scleroconjunctivitis.     

Spectrum of pericardial disease: part II

Pericardial disease is a common disorder seen in varying clinical settings, and may be the first manifestation of an underlying systemic disease. In part I, we focused on the current knowledge and management of the more common pericardial diseases: acute pericarditis, pericardial effusion, cardiac tamponade, chronic pericarditis and relapsing pericarditis. In part II, we will focus on the knowledge and management of pericardial involvement in chylous pericardial effusion cholesterol pericarditis, radiation pericarditis, pericardial involvement in systemic inflammatory diseases, autoreactive pericarditis, pericarditis in renal failure, pericardial constriction and effusive constrictive pericarditis.     

A case of hemosuccus pancreaticus associated with hereditary pancreatitis

Relapsing Polychondritis is a rare disease which causes the repetitive inflammation of cartilage and connective tissues. Although the large airway is sometimes involved and the stenosis of them often influences the prognosis of the patients, there have been few reports concerning the manifestation of the peripheral lung. A 60-year-old man with pulmonary fibrosis was admitted to a regional hospital due to sudden deafness, and then he suffered from relapsing polychondritis. During the steroid therapy, he also suffered from bilateral pneumothoraces. His computed tomogram revealed many bilateral bullae, emphysematous changes, and fibrotic changes in bilateral lungs. The mechanism of generating peripheral pulmonary manifestations is also discussed.     

Spectrum of pericardial disease: part I

Pericardial disease is a common disorder seen in varying clinical settings and may be the first manifestation of an underlying systemic disease. It may be due to multiple causes. Epidemiologic studies are lacking, and the exact incidence and prevalence are unknown. New diagnostic techniques have improved diagnosis, allowing early diagnosis and management. There are few randomized data to guide physicians in the management of pericardial diseases. Part I of our review focuses on the current state of knowledge and management of the more common pericardial diseases: acute pericarditis, pericardial effusion, cardiac tamponade, chronic pericarditis and relapsing pericarditis.     

良性大气道狭窄的支气管镜介入治疗

目的 介绍该院支气管镜下治疗良性大气道狭窄的临床经验。方法 28例良性气道狭窄病例，根据狭窄的病变性质、部位和程度采取支气管镜下介入治疗方法，对治疗后疗效评价、肺功能改善和并发症以及综合治疗方法的采用进行分析。结果 28例病人共进行支气管镜介入治疗72次。其中行高压球囊扩张治疗16例，病因中以气管、支气管内膜结核为主；高频电治疗8例，以气管切开后肉芽组织增生和腔内息肉、良性肿瘤为主；气道内支架置入9例，病因以气管切开后气管狭窄和复发性多软骨炎气道累及为主。部分病人合用了多种治疗方法。治疗后24例病人呼吸困难迅速缓解，部分病人肺功能明显改善。高频电治疗病例中大出血1例，球囊扩张治疗以轻度胸痛和少量出血为主。支架置入后1例出现明显肉芽增生，需要介入治疗。结论 支气管镜下介入技术治疗良性气道狭窄可以迅速解除气道梗阻，呼吸困难迅速改善，肺功能改善明显，并发症较少。     

Thoracic epidural morphine in the palliation of chest wall pain secondary to relapsing polychondritis

Relapsing polychondritis is a rare disease of unknown etiology characterized pathologically by degeneration of the chondrocyte and replacement with fibrous connective tissue. The following case report presents the pain management of a 34-yr-old man suffering from intractable pain secondary to relapsing polychondritis. Systemic narcotic analgesics, adjunctive drugs, and peripheral nerve blocks with local anesthetic and steroid failed to adequately control the patient's pain. Thoracic epidural morphine was used to provide excellent relief of pain. Factors in the selection of an implantable narcotic delivery system as well as practical considerations including tolerance and potential side effects of intraspinal narcotics are discussed. Ethical issues surrounding the chronic use of intraspinal narcotics in the setting of chronic benign pain are also discussed.     

Auricular chondritis in rats. An experimental model of relapsing polychondritis induced with type II collagen

Outbred Wistar rats immunized with native type II collagen developed ear lesions resembling those of human relapsing chondritis. As in human disease, these lesions were characterized by intense chondritis, positive immunofluorescence reactions to IgG and C3, and circulating IgG reactive with native type II collagen. Furthermore, electron-dense deposits were seen near the surface of chondrocytes and corresponded with deposits of IgG and C3. These observations suggest a causal relation between humoral immunity to type II collagen and auricular chondritis in the rat and support the hypothesis than human relapsing polychondritis is an autoimmune disease mediated by immunity to type II collagen.     

Relapsing polychondritis

Relapsing polychondritis is an uncommon disease consisting of inflammation of the eyes, inner ears, cardiovascular system, and cartilaginous portions of the joints, respiratory tract, and external ear. Cutaneous manifestations are the presenting feature in more than 50% of patients. These usually consist of erythema, swelling, and pain, reflecting involvement of the underlying cartilage. Direct involvement of the skin may occur as vasculitis, lesions resembling erythema nodosum, or nonspecific eruptions. The presence of circulating antibodies to type II collagen--more against native than denatured collagen--and to human fetal cartilage, and the presence of circulating immune complexes suggest a primary role for antibody in the pathogenesis of relapsing polychondritis. There is no ideal treatment, but systemic corticosteroids and dapsone seem most effective.     

Auricular chondritis in NOD.DQ8.Abetao (Ag7-/-) transgenic mice resembles human relapsing polychondritis

Relapsing polychondritis is a multisystem autoimmune disease involving cartilage destruction but no known causative antigen. HLA-DQ8 has been associated with various autoimmune diseases in humans. To study the role of DQ8 in autoimmune diseases, we have generated transgenic mice expressing DQ8 (DQA1*0301, DQB1*0302) in a NOD background lacking endogenous class II molecules (Abetao). Upon immunization with type II collagen (CII), 85% of NOD.DQ8 mice develop severe experimental polychondritis, auricular chondritis, and polyarthritis, with clinical and histological similarities to relapsing polychondritis (RP) in humans. CII-immunized mice mount a T cell response and produce Ab's to type IX collagen (CIX) and self-CII. Transgene-negative littermates do not develop any serological and clinical manifestations following immunization. B10.DQ8 transgenic mice develop polyarthritis and Ab's to CII only. The susceptibility to auricular chondritis in NOD.DQ8 mice can be attributed to response to CIX. A higher number of activated cells, CD4+CD44(hi)CD62L(lo), and lower regulatory cells CD4+CD152+CD25+ were observed in NOD.DQ8 mice compared with B10.DQ8 mice. The NOD.DQ8 mice provide a model of RP with a high disease incidence and multiple organ involvement to investigate putative autoantigen and regulatory cells involved in disease pathogenesis. An experimental model restricted by the human class II molecule will be valuable when studying the role of various collagens in immunologic and pathologic responses in human RP.     

类风湿关节炎与复发性多软骨炎致环杓关节固定的诊断与治疗

目的 :探讨类风湿性关节炎 (RA)和复发性多软骨炎 (RP)的临床特点、诊断与鉴别诊断以及治疗与预后 ,以提高对RA和RP的认识。方法 :回顾性分析 11例RA和 15例RP患者的临床资料。结果 :1991～ 2 0 0 0年间收治 11例RA和 15例RP中 ,分别有 4例 ( 36 %)和 5例 ( 33%)累及双侧环杓关节。RP中 6 0 %有耳软骨炎 ,33%有鼻软骨炎 ,47%有呼吸系统受累 ,部分患者有心血管受累 ,用皮质激素、免疫抑制和氨苯砜控制症状 ,4例RA和 5例RP患者因呼吸道梗阻行气管切开术 ,3例RA患者行杓状软骨切除声带外展移位术。 15例RP中 1例治疗无效 ,死于呼吸道并发症。结论 :RA、RP是自身免疫性疾病 ,可累及多器官的软骨结构及结缔组织 ,在耳鼻喉科有表现 ,易误诊。早期诊断 ,采用综合治疗是提高疗效改善预后的关键。     

Pericardiectomy vs Medical Management in Patients With Relapsing Pericarditis

ObjectiveTo determine whether surgical pericardiectomy is a safe and effective alternative to medical management for chronic relapsing pericarditis.Patients and MethodsRetrospective review of 184 patients presenting to the Mayo Clinic in Rochester, Minnesota, from January 1, 1994, through December 31, 2005, with persistent relapsing pericarditis identified 58 patients who had a pericardiectomy after failed medical management and 126 patients who continued with medical treatment only. The primary outcome variables were in-hospital postoperative mortality or major morbidity, all-cause death, time to relapse, and medication use.ResultsMean ± SD follow-up was 5.5±3.5 years in the surgical group and 5.4±4.4 years in the medical treatment group. At baseline, patients in the surgical group had higher mean relapses (6.9 vs 5.5; P=.01), were more likely to be taking colchicine (43.1% [n=25] vs 18.3% [n=23]; P=.002) and corticosteroids (70.7% [n=41] vs 42.1% [n=53]; P<.001), and were more likely to have undergone a prior pericardiotomy (27.6% [n=16] vs 11.1% [n=14]; P=.003) than the medical treatment group. Perioperative mortality (0%) and major morbidity (3%; n=2) were minimal. Kaplan-Meier analysis revealed no differences in all-cause death at follow-up (P=.26); however, the surgical group had a markedly decreased relapse rate compared with the medical treatment group (P=.009). Medication use was notably reduced after pericardiectomy.ConclusionIn patients with chronic relapsing pericarditis in whom medical management has failed, surgical pericardiectomy is a safe and effective method of relieving symptoms.     

Pneumococcal pericarditis presenting as an out of hospital cardiopulmonary arrest.

Serious complications of pneumococcal pneumonia have become uncommon with effective antibiotic treatment. Purulent pericarditis is a rare though well described complication of untreated pneumococcal sepsis. A case of untreated pneumococcal pneumonia complicated by purulent pericarditis is described. This presented as an out of hospital asystolic cardiopulmonary arrest.     

Surgical treatment of the cardiac manifestations of relapsing polychondritis: overview of 33 patients identified through literature review and the Mayo Clinic records

OBJECTIVES: To analyze the cardiac findings that necessitate surgery in patients with relapsing polychondritis (RP) and to compare our results to cases in the literature. MATERIAL AND METHODS: A systematic overview of the literature was completed with the addition of cases of RP from the Mayo patient population that necessitated cardiac surgery. RESULTS: Thirty-three patients were identified (25 from the literature and 8 from the Mayo patient population). Nine patients (27%) were female, 22 (67%) were male, and sex was not stated for 2 patients (6%). The patient age ranged from 17 to 69 years (mean +/- SD, 42.5 +/- 15.7 years). At operation, 30 patients (91%) had aortic regurgitation, 21 (64%) had aortic root disease, and 13 (39%) had mitral regurgitation. The most common surgical procedure performed was aortic valve replacement in 12 patients (36%). The most common complications were death in 12 patients (36%) and prosthetic valve dehiscence in 4 patients (12%). CONCLUSIONS: Cardiac involvement is more prominent in the male population and requires more invasive procedures. Aortic valve replacement with composite graft replacement of the ascending aorta along with coronary artery ostial reimplantation should be considered in these patients. Postsurgical valvular complications include prosthetic dehiscence, paravalvular leakage, mediastinitis, and heart failure, and these complications are associated with postoperative corticosteroid therapy. Initiation of second-line immunosuppressive therapy should be substituted for corticosteroids after cardiac surgery.     

Immunopathologic Studies in Relapsing Polychondritis

Serial studies have been performed on three patients with relapsing polychondritis in an attempt to define a potential immunopathologic role for degradation constituents of cartilage in the causation and/or perpetuation of the inflammation observed. Crude proteoglycan preparations derived by disruptive and differential centrifugation techniques from human costal cartilage, intact chondrocytes grown as monolayers, their homogenates and products of synthesis provided antigenic material for investigation. Circulating antibody to such antigens could not be detected by immunodiffusion, hemagglutination, immunofluorescence or complement mediated chondrocyte cytotoxicity as assessed by (51)Cr release. Similarly, radiolabeled incorporation studies attempting to detect de novo synthesis of such antibody by circulating peripheral blood lymphocytes as assessed by radioimmunodiffusion, immune absorption to neuraminidase treated and untreated chondrocytes and immune coprecipitation were negative.Delayed hypersensitivity to cartilage constituents was studied by peripheral lymphocyte transformation employing [(3)H]thymidine incorporation and the release of macrophage aggregation factor. Positive results were obtained which correlated with periods of overt disease activity. Similar results were observed in patients with classical rheumatoid arthritis manifesting destructive articular changes. This study suggests that cartilage antigenic components may facilitate perpetuation of cartilage inflammation by cellular immune mechanisms.     

快速进展型结核性心包炎的临床治疗

目的探讨不同治疗方案对于快速进展为缩窄性心包炎的结核性心包炎（快速进展型结核性心包炎）治疗效果、并发症、死亡率、患者预后的影响,以期对结核性心包炎的治疗获得更好的效果。 方法回顾性分析2014年6月至2019年5月成都市第三人民医院心脏大血管外科对38例快速进展型结核性心包炎的治疗方式、外科手术干预时机、手术方式及疗效、并发症、死亡率和随访情况等,治疗方式包括药物治疗、心包穿刺引流术、心包开窗术、心包剥脱术等。同期观察对慢性缩窄性心包炎行外科手术治疗患者115例。 结果对于快速进展型结核性心包炎采用不同的治疗方案,随访1~5年。所有患者均采用抗结核药物治疗,其中单纯药物治疗5例,全部进展为缩窄性心包炎;早期行心包穿刺引流术12例,治愈1例,其余11例进展为慢性缩窄性心包炎;早期行心包开窗术10例,进展为慢性缩窄性心包炎1例,治愈9例;早期行心包剥脱术11例,无进展为慢性缩窄性心包炎病例。全组无围术期死亡病例。与同期行慢性缩窄性心包炎外科手术治疗患者相比,快速进展型结核性心包炎外科手术治疗患者低心排综合征发生率较低（4.8% vs 24.3%,P＜0.05）,患者心功能改善情况较好（100.0% vs 80.7%,P＜0.05）,术后5年随访生存率较高（100% vs 78%,P＜0.05）。 结论快速进展型结核性心包炎单纯采用药物治疗和心包穿刺术治疗,有很高比例的患者会进展为缩窄性心包炎,从而严重影响患者的预后。早期外科干预能显著降低快速进展型结核性心包炎进展为缩窄性心包炎的概率,从而改善患者总体预后,改善患者生存质量,减轻社会负担。     

复发性多软骨炎的临床特征及处理特点

目的：探讨复发性多软骨炎的临床特征及诊治方法,提高其诊治水平。方法：报告2例复发性多软骨炎患者的临床资料,并通过文献复习,对2例资料进行分析。结果：2例复发性多软骨炎患者经激素治疗后,均得到控制,随访1～6年,预后良好。结论：复发性多软骨炎是一种累及全身多处软骨的发作性和进行性炎症。是一种较为不见的综合症。临床表现多种多样,主要损害常见于耳、鼻、咽喉、眼球和全身软骨。激素是治疗本病最好的药物。     

Anticytoplasmic autoantibodies in the diagnosis and follow-up of Wegener's granulomatosis

Sixty-five patients with biopsy-proven Wegener's granulomatosis (WG), 54 with systemic vasculitis, 22 with relapsing polychondritis, 20 with sarcoidosis, 20 with malignant pulmonary lesions, and 15 with other conditions underwent determination of anticytoplasmic autoantibodies (ACPA) by the indirect immunofluorescence technique on neutrophil cytospin preparations to assess the specificity of ACPA for WG, their sensitivity in relationship to the extent and activity of the disease, and their value for follow-up of WG. Of these 65 patients with WG, 38 were ACPA positive. Two patients in the vasculitis group, best categorized as having microscopic polyarteritis, were ACPA positive. We obtained 125 serum samples from the 65 patients with WG and assigned them to one of two categories (limited or generalized), based on the extent of disease. Each of these categories was then subdivided into "active" or "in remission." Median ACPA titers were significantly different between active disease and remission in each category, as well as between active limited and active generalized disease. All patients whose disease changed from active to in remission had reductions in ACPA titer levels; those who experienced flares had titer increases. Patients with intercurrent illnesses or complications of treatment, mimicking WG flares, did not have titer increases. We conclude that ACPA determined by the indirect immunofluorescence technique is highly specific for WG. The sensitivity is dependent on the extent and activity of WG, and serial titer determinations are valuable in monitoring disease activity.     

Composition of urinary glycosaminoglycans in a patient with relapsing polychondritis

OBJECTIVES: Several investigators have reported an increase in urinary glycosaminoglycans (GAGs) in patients with relapsing polychondritis (RP). The aim of this investigation is to analyze the composition and structure of urinary GAGs from a Brazilian patient with RP. DESIGN AND METHODS: The identification and structural analyses of the GAGs were made by electrophoresis and degradation with specific enzymes and identification of their disaccharides products by HPLC chromatography. RESULTS: The disaccharide products formed from RP urinary chondroitin sulfate (CS) by action of chondroitin ABC lyase showed a substantial relative increase of nonsulfated disaccharides with a relative decrease of 6-sulfated disaccharides compared to control subjects. In addition, a significant change of the ratio of CS and heparan sulfate was also observed in the RP patient. CONCLUSION: The RP patient analyzed has shown a structural anomaly of the urinary CS and this may contribute to the diagnosis of this disease.     

少见原因致呼吸道狭窄三例临床分析

目的探讨引起气道狭窄的复发性多软骨炎、气管淀粉样变性、骨化性气管支气管病的临床诊治要点。方法对3例气道狭窄的临床资料进行分析并复习相关文献。结果本组复发性多软骨炎、气管淀粉样变性、骨化性气管支气管病各1例,其临床症状及体征均无明显特异性,均可引起气道狭窄,但进一步行胸部CT结合电子气管镜及病理检查可明确诊断。结论三种疾病均可引起气道狭窄,但及时进行CT、电子气管镜及病理检查可减少误诊并改善预后。