Images in clinical medicine. Rotator-cuff tear.
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Biomedical subjects
Publications and source records attributed to Clement J Michet.
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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.
PURPOSE: Studies on mortality associated with systemic sclerosis have been limited by small sample sizes. We aimed to obtain large-scale evidence on survival outcomes and predictors for this disease. METHODS: We performed a meta-analysis of individual patient data from cohorts recruited from seven medical centers in the United States, Europe, and Japan, using standardized definitions for disease subtype and organ system involvement. The primary outcome was all-cause mortality. Standardized mortality ratios and predictors of mortality were estimated. The main analysis was based only on patients enrolled at each center within 6 months of diagnosis (incident cases). RESULTS: Among 1645 incident cases, 578 deaths occurred over 11,521 person-years of follow-up. Standardized mortality ratios varied by cohort (1.5 to 7.2). In multivariate analyses that adjusted for age and sex, renal (hazard ratio [HR] = 1.9; 95% confidence interval [CI]: 1.4 to 2.5), cardiac (HR = 2.8; 95% CI: 2.1 to 3.8), and pulmonary (HR = 1.6; 95% CI: 1.3 to 2.2) involvement, and anti-topoisomerase I antibodies (HR = 1.3; 95% CI: 1.0 to 1.6), increased mortality risk. Renal, cardiac, and pulmonary involvement tended to occur together (P <0.001). For patients without adverse predictors for 3 years after enrollment, the subsequent risk of death was not significantly different from that for the general population in three cohorts, but was significantly increased in three cohorts that comprised mostly referred patients. Analyses that included all cases in each center (n = 3311; total follow-up: 19,990 person-years) yielded largely similar results. CONCLUSION: Systemic sclerosis confers a high mortality risk, but there is considerable heterogeneity across settings. Internal organ involvement and anti-topoisomerase I antibodies are important determinants of mortality.
Vasculitic neuropathy usually presents with painful mononeuropathies or an asymmetric polyneuropathy of acute or subacute onset. The disorder should be classified as being systemic or non-systemic. Systemic vasculitis should be further classified into one of the primary and secondary forms. Although specific treatment regimens vary among neurologists, basic principles can be applied. Corticosteroids and cytotoxic drugs have been the mainstay of treatment for most forms of vasculitic neuropathy. Here we discuss dosing, potential side-effects, and management recommendations of conventional treatments. New treatments showing promise include intravenous immunoglobulin and biological agents and trials of the newest treatments are being reviewed. Future trials should compare commonly used treatment regimens and better establish the efficacy of newer, potentially safer, treatments.
SUMMARY: Relapsing polychondritis is a unique, rare autoimmune disorder in which the cartilaginous tissues are the primary targets of destruction but the immune damage can spread to involve noncartilaginous tissues like the kidney, blood vessels, and so forth. The manifestations of the disease can take many different forms and the pathogenesis is still unclear. It may occur in a primary form or it may be associated with other disease states. This article summarizes important aspects of the disease with a focus on recent information regarding clinical manifestations, disease associations, pathogenesis, and advances in therapeutics.
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The clinical presentation of new onset headache, temporal artery tenderness, and elevated inflammatory markers is classical for giant cell arteritis (GCA). We describe a patient who presented with clinical features of GCA, but was found to have isolated necrotizing arteritis of the temporal artery in the setting of acute myeloid leukemia. This report emphasizes that if the temporal artery histology is not classical for GCA, or if the response to treatment is incomplete, further evaluation for systemic or paraneoplastic vasculitis should be considered.
Relapsing polychondritis (RP) is a rare disease causing inflammation and destruction of cartilage and other connective tissues. Specific laboratory aberrations are lacking. Predominant clinical manifestations include auricular chondritis, polyarthritis, nasal chondritis, ocular inflammation, audiovestibular damage, and respiratory tract chondritis. A relapsing course is characteristic. Airways are involved in 50% of patients and may cause dyspnea, stridor, wheezing, hoarseness, aphonia, and laryneal or tracheal tenderness. Airflow obstruction may result from RP involving the tracheobronchial tree; there is no interstitial or pulmonary vascular component. Collapse or failure of the trachea to dilate during inspiration is a key feature. Fast computed tomographic (CT) scanners can visualize dynamic airway collapse. Randomized, controlled trials of therapy have not been done. Corticosteroids and nonsteroidal anti-inflammatory drugs (NSAIDs) are used most commonly, but optimal regimens and duration of therapy have not been elucidated. Endobronchial stents or tracheostomy may be required for severe stenoses refractory to medical therapy.
Relapsing polychondritis is an uncommon disease of unknown etiology, usually manifested by inflammatory changes of cartilaginous tissues. Cardiovascular complications are rare but have been associated with adverse prognosis. Aortitis, vasculitis of large- and medium-sized arteries with aneurysm formation, valvulitis, pericarditis, and atrioventricular conduction disturbances have been reported as late complications of relapsing polychondritis. We describe a 42-year-old man who developed all the known cardiovascular complications of relapsing polychondritis except for clinically evident pericarditis. This case illustrates the multiple, varied, and potentially fatal cardiovascular complications that can occur with this disorder. Patients with relapsing polychondritis should be monitored closely for development of such complications.
OBJECTIVE: To identify factors influencing the development of psoriatic arthritis (PsA) in a population-based, inception cohort of psoriasis (PS) patients. METHODS: Using the population-based data resources of the Rochester Epidemiology Project. which ensures virtually complete ascertainment of all clinically defined conditions, we previously identified all incident cases of PsA and prevalent cases with PS from 1/1/1982 to 12/21/1991. In this nested case-control study, we assessed potential factors influencing the development of PsA in this cohort using medical record and patient survey information. Each case of PsA was matched with 2 PS controls on age, gender and PS duration/date of onset. Factors influencing the development of PsA were identified, adjusting for the influence of other variables using conditional logistic regression for medical record data and logistic regression for survey data. RESULTS: Sixty incident PsA cases were matched with 120 controls with PS. The median age at onset of PS was 31.7 (3.0-78.3) years, and 49% of subjects were male. There were 67% (n = 40) survey responders among cases and 48% (n = 58) among controls. Corticosteroids were used by 10 cases and 6 controls in the 2 years prior to onset of PS through to the development of PsA, and increased the risk of developing PsA (odds ratio 4.33, 95% CI = 1.34-14.02). Pregnancy occurred in 2 cases and 12 controls in the same period, and decreased the risk of developing PsA (odds ratio 0.19, 95% CI = 0.04-0.95). These associations remained significant after adjusting for the influence of gender, age, and duration of psoriasis. CONCLUSION: Corticosteroid use and pregnancy, both of which modulate the immune response, may influence the development of PsA in patients with PS.
OBJECTIVE: To determine whether the incidence of gout is higher in 1995-1996 compared to 1977-1978. METHODS: Using the Rochester Epidemiology Project computerized medical record system, all potential cases of acute gout in the city of Rochester, Minnesota during the time intervals of 1977-1978 and 1995-1996 were identified. The complete medical records of all potential cases were screened and all who fulfilled the 1977 American College of Rheumatology proposed criteria for gout were included as incidence cases. Demographic data, body mass index, clinical presentation, and associated comorbid conditions were abstracted. The overall and age-gender adjusted incidence rates from the 2 cohorts were calculated and compared. RESULTS: A total of 39 new cases of acute gout were identified during the 2 year interval 1977-1978 representing an age and sex-adjusted annual incidence rate of 45.0/100,000 (95% CI: 30.7, 59.3). For the interval 1995-1996, 81 cases were diagnosed, representing an annual incidence rate of 62.3/100,000 (95% CI: 48.4, 76.2). There was a greater than 2-fold increase in the rate of primary gout (i.e., no history of diuretic exposure) in the recent compared to the older time periods (p = 0.002). The incidence of secondary, diuretic related gout did not increase over time (p = 0.140). CONCLUSION: Our results indicate that the incidence of primary gout has increased significantly over the past 20 years. While this increase might be a result of improved ascertainment of atypical gout, it may also be related to other, as yet unidentified, risk factors.