Cholesterol pericarditis--relapsing pericardial effusion in a patient with rheumatoid arthritis.

Cholesterol pericarditis is an uncommon form of pericardial disease, of unknown pathophysiology, that is characterized by chronic relapsing, usually large, pericardial effusions that are distinctive due to a high level of cholesterol. Usually it is idiopathic, but it can be associated with various systemic diseases such as hypothyroidism, rheumatoid arthritis and tuberculosis, among others. Its clinical course is usually indolent and complications such as cardiac tamponade and chronic constrictive pericarditis are relatively rare. However, the need for surgery for complete treatment has been reported in at least 10 % of cases. When rheumatoid arthritis is the underlying cause, this outcome is more frequent among those with an acute episode of pericarditis during the course of the disease. We report the case of a 61-year-old female rheumatoid arthritis patient, who presented with heart failure due to a large pericardial effusion and was successfully treated by a surgical approach.     

Polychondritis and rheumatoid arthritis. Case report and review of the literature

Summary We present the case history of a 50-year-old man with seropositive erosive rheumatoid arthritis of 30-years standing who developed polychondritis simultaneously with several extra-articular rheumatoid manifestations, such as anaemia, subcutaneous nodules, pericarditis and episcleritis. The relevant literature is reviewed. Gradually, all symptoms and signs disappeared after start of treatment with 30 mg prednisone and 100 mg azathioprine daily. We suggest that the polychronditis in this patient was also an extra-articular manifestation of rheumatoid arthritis.     

Acute pericarditis as the initial manifestation in Still's disease in an adult

Almost 90% of primary acute pericarditis are idiopathic. Between specifics forms, a very low percentage of cases are due to chronic rheumatic diseases. A case of adult Still's disease (juvenile chronic rheumatoid arthritis) with acute pericarditis being the first clinical manifestation (besides fever and general syndrome) is presented. Therapy with oral prednisone was rapidly effective, and pericardial effusion resolved after 3 weeks of treatment, as echocardiography showed.     

Management of extra-articular disease manifestations in rheumatoid arthritis

PURPOSE OF REVIEW: To discuss the rationale for various treatment strategies in rheumatoid arthritis with extra-articular manifestations, and to review advances in understanding the impact of extra-articular rheumatoid arthritis and its management. RECENT FINDINGS: Recent epidemiologic studies of extra-articular rheumatoid arthritis manifestations have emphasized their major role as predictors of premature mortality in patients with rheumatoid arthritis, and provide a rationale for aggressive ant-rheumatic treatment of extra-articular rheumatoid arthritis. Previous uncontrolled or nonrandomized studies favor the use of cyclophosphamide in patients with systemic rheumatoid vasculitis, and methotrexate in the case of other manifestations of extra-articular rheumatoid arthritis. Recent case reports indicate that patients with rheumatoid lung disease may respond to cyclosporine or tumor necrosis factor inhibitors, and that tumor necrosis factor blocking therapy also may be successful in cases of treatment-resistant vasculitis. By contrast, it has been suggested that tumor necrosis factor inhibitors may induce some manifestations of extra-articular rheumatoid arthritis. Data indicating a high risk of serious infections and cardiovascular disease in patients with extra-articular rheumatoid arthritis underline the importance of carefully monitoring such patients. SUMMARY: Extra-articular rheumatoid arthritis is a serious condition, and rheumatoid arthritis patients with extra-articular manifestations should be aggressively treated and monitored. Advances in the understanding of the pathogenesis of rheumatoid arthritis and developments of new, more specific drugs may be of particular benefit to patients with extra-articular disease.     

Cardiac tamponade, constrictive pericarditis and pericardial resection in rheumatoid arthritis.

Four patients with rheumatoid constrictive pericarditis and two patients with rheumatoid cardiac tamponade are presented, and 60 previously reported cases with these two complications are reviewed. Rheumatoid arthritis was moderate to severe in 84% of the patients with cardiac tamponade and in 74% of the patients with constrictive pericarditis. However, both these complications were also seen in patients who had only mild arthritis and in two previously reported cases constrictive pericarditis actually preceded the onset of rheumatoid arthritis. The duration of rheumatoid arthritis had no bearing on the development of these complications. In 75% of patients with cardiac tamponade, and in 66% of cases with constrictive pericarditis, subcutaneous nodules were present. In those cases where the rheumatoid factor was measured it was positive in 92% with cardiac tamponade and in 84% with constrictive pericarditis. In 63% of patients with cardiac tamponade and in 70% of cases with constrictive pericarditis a history of pericardial type of pain was obtained and/or a pericardial rub heard. The diagnosis of cardiac tamponade and constrictive pericarditis was made clinically and in doubtful cases confirmed by cardiac screening and intracardiac pressure recordings. The low sugar content in the pericardial fluid in the absence of infection or malignancy was an important clue to the rheumatoid etiology of the effusion. In the majority of the cases histological appearances of the pericardial tissue showed non-specific fibrous reaction and infiltration with plasma cells and lymphocytes. Only in five of the cases, including one from the present series, were typical rheumatoid granulomatous lesions demonstrated. Treatment with corticosteroids neither prevented the occurrence nor led to amelioration of either cardiac constriction or tamponade. Pericardial resection was life saving, producing both symptomatic and objective involvement of the cardiac function. In the present series of six cases two patients developed aortic incompetence. In one of these it was due to rheumatoid granulomatous valve disease and in the other due to non-specific aortic valvulitis. The combination of constrictive pericarditis and granulomatous aortic valve disease has not been previously recorded.     

Delayed pericardial disease after radiotherapy

Delayed chronic constrictive pericarditis developed in seven patients 51 to 268 (mean 116) months after radiotherapy. Six of the seven complained of exertional dyspnea that was initially believed to be caused by mediastinal fibrosis. All patients had raised jugular venous pressure, although in two patients this finding was not appreciated by the primary physician. There were no consistent noninvasively identifiable features to allow prediction of constrictive pericarditis other than consideration of its existence and careful examination of the cardiovascular system. All patients had constrictive pericarditis proved at cardiac catheterization. Of the five patients who underwent pericardiectomy, two had an excellent functional result, one has residual pericardial constriction and two died of unrelated causes. Recommendations for the treatment of radiation-induced pericardial disease are given.     

Extra-articular disease manifestations in rheumatoid arthritis: incidence trends and risk factors over 46 years

Objective: To investigate the trends in incidence of extra-articular rheumatoid arthritis (ExRA) in a well defined community based cohort of patients with rheumatoid arthritis (RA), and to examine possible predictors of ExRA occurrence. Methods: Using the resources of the Rochester Epidemiology Project, a retrospective medical record review was conducted of a cohort of 609 cases of RA in Olmsted County, MN, diagnosed during 1955–94. These cases had been previously classified using the ACR 1987 criteria for RA. Patients were followed up from 1955 to 2000 (median follow up 11.8 years; range 0.1–42.8), and incident ExRA manifestations were recorded according to predefined criteria. Time to first presentation of ExRA was compared in patients with RA by decade of diagnosis. Possible ExRA risk factors were identified in case record reviews. Results: ExRA occurred in 247 patients (40.6%). A subgroup of 78 patients (12.8%) had ExRA manifestations considered to be severe in a previous study from Malmö, Sweden. The incidence of severe ExRA did not change significantly over the decades (p=0.165). In a multivariate analysis the main predictors of severe ExRA were smoking at RA diagnosis (risk ratio (RR)=2.94; 95% confidence interval (95% CI) 1.68 to 5.13) and early disability (Steinbrocker class III-IV at diagnosis) (RR=2.45; 95% CI 1.51 to 4.00). The effect of smoking overwhelmed the weaker effect of rheumatoid factor seropositivity. Conclusion: There was no decrease in the incidence of extra-articular manifestations in patients with RA diagnosed up to 1995. Smoking and early disability are independent risk factors for extra-articular RA.     

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心包炎是临床常见病,其病因可分为感染性和非感染性两大类。心包炎的临床表现多种多样,在各个临床科室都可能会碰到心包炎患者。心包炎的临床类型主要包括以下几种:急性心包炎、慢性心包炎、心包积液、心包压塞、缩窄性心包炎等。近些年尽管在心包炎的诊治上取得了一些进展,但因循证医学证据的缺乏目前临床上心包炎的诊治仍是以经验治疗为主。急性心包炎的自然病程往往呈自限性,预后较好,所以治疗上以支持治疗为主。一旦心包炎的病因明确,其治疗则是以病因治疗为主。     

Value of reference tracings in diagnosis and assessment of constrictive epi- and pericarditis

Reference tracings are of great value in the diagnosis and assessment of constrictive pericarditis. The Q-h interval in the jugular venous pulse tracing is strongly correlated with the mean right atrial pressure (r=0·91). The left ventricular ejection time, the Q-A2 interval, and the Q-h interval are independent during atrial fibrillation from the preceding diastolic filling interval. This differentiates constrictive pericarditis from valvular heart disease. Cases with haemodynamically significant constrictive epicarditis are characterized by a rapid evolution, absence of pericardial calcification and absence of an early diastolic filling sound, a dominant a wave in the jugular venous pulse tracing, and a high early diastolic ventricular pressure. The haemodynamic behaviour is similar to that found in cases with myocardial fibrosis.     

Cardiac tamponade in systemic juvenile rheumatoid arthritis requiring emergency pericardiectomy

A 9-year-old boy with systemic onset juvenile rheumatoid arthritis (JRA) presented with fever and chest pain and rapidly developed pericarditis and cardiac tamponade. Despite corticosteroid treatment and pericardiocentesis, rapid deterioration necessitated the emergency placement of a pericardial window. This is the first reported instance of this type of emergency surgical intervention for JRA. Of 220 children with JRA followed for 12 years (7% systemic onset), 8 had evidence of acute pericarditis but no other had definite evidence of tamponade.     

Pericardial involvement in end-stage renal disease

Pericardial involvement in end-stage renal disease (ESRD) is manifested most commonly as acute uremic or dialysis pericarditis and infrequently as chronic constrictive pericarditis. The causes of uremic and dialysis pericarditis remain uncertain. The clinical and laboratory manifestations of acute pericarditis, pericardial effusion, cardiac tamponade, and constrictive pericarditis in patients with chronic renal failure are similar to those observed in nonuremic patients with similar pericardial involvement, except that chest pain occurs less frequently in those with ESRD. Therapeutic interventions for acute uremic or dialysis pericarditis with or without pericardial effusion include intensive hemodialysis, pericardiocentesis (infrequently used), pericardiostomy with or without instillation of intrapericardial glucocorticoids, pericardial window, and pericardiectomy. Chronic constrictive pericarditis is treated with pericardiectomy.     

The extra-articular features of rheumatoid arthritis. A systematic analysis of 127 cases

A systematic analysis was undertaken of 127 hospitalized patients with definite or classic rheumatoid arthritis. Of the group studied 76 per cent had one or more extra-articular feature including nodules, pulmonary fibrosis, digital vasculitis, skin ulceration, lymphadenopathy, noncompressive neuropathy, splenomegaly, episcleritis or pericarditis. The presence of one or more of these features correlated with signs of persistent articular inflammation, roentgenologic stages of more severe joint disease and the presence of soluble immune complexes and high titers of rheumatoid factor. They did not appear to be related to the use of corticosteroids or to the duration of rheumatoid disease. On follow-up of the total group of patients after five years a mortality of 20 per cent was found. This was twice as great in the group with extra-articular features and was particularly striking in patients 50 years of age or less.These results obtained in hospitalized patients indicate that the manifestations of rheumatoid arthritis form a spectrum in which extra-articular features should be considered as classic criteria of rheumatoid arthritis. Furthermore, these extra-articular features appear to have an important bearing on prognosis.     

Diagnosis and management of pericardial effusion

Pericardial effusion is a common finding in everyday clinical practice.The first challenge to the clinician is to try to establish an etiologic diagnosis.Sometimes,the pericardial effusion can be easily related to a known underlying disease,such as acute myocardial infarction, cardiac surgery,end-stage renal disease or widespread metastatic neoplasm.When no obvious cause is apparent,some clinical findings can be useful to establish a diagnosis of probability.The presence of acute inflammatory signs(chest pain,fever,pericardial friction rub) is predictive for acute idiopathic pericarditis irrespective of the size of the effusion or the presence or absence of tamponade.Severe effusion with absence of inflammatory signs and absence of tamponade is predictive for chronic idiopathic pericardial effusion,and tamponade without inflammatory signs for neoplastic pericardial effusion.Epidemiologic considerations are very important,as in developed countries acute idiopathic pericarditis and idiopathic pericardial effusion are the most common etiologies,but in some underdeveloped geographic areas tuberculous pericarditis is the leading cause of pericardial effusion.The second point is the evaluation of the hemodynamic compromise caused by pericardial fluid.Cardiac tamponade is not an"all or none"phenomenon,but a syndrome with a continuum of severity ranging from an asymptomatic elevationof intrapericardial pressure detectable only through hemodynamic methods to a clinical tamponade recognized by the presence of dyspnea,tachycardia,jugular venous distension,pulsus paradoxus and in the more severe cases arterial hypotension and shock.In the middle,echocardiographic tamponade is recognized by the presence of cardiac chamber collapses and characteristic alterations in respiratory variations of mitral and tricuspid flow.Medical treatment of pericardial effusion is mainly dictated by the presence of inflammatory signs and by the underlying disease if present.Pericardial drainage is mandatory when clinical tamponade is present.In the absence of clinical tamponade,examination of the pericardial fluid is indicated when there is a clinical suspicion of purulent pericarditis and in patients with underlying neoplasia.Patients with chronic massive idiopathic pericardial effusion should also be submitted to pericardial drainage because of the risk of developing unexpected tamponade.The selection of the pericardial drainage procedure depends on the etiology of the effusion.Simple pericardiocentesis is usually sufficient in patients with acute idiopathic or viral pericarditis.Purulent pericarditis should be drained surgically,usually through subxiphoid pericardiotomy. Neoplastic pericardial effusion constitutes a more difficult challenge because reaccumulation of pericardial fluid is a concern.The therapeutic possibilities include extended indwelling pericardial catheter,percutaneous pericardiostomy and intrapericardial instillation of antineoplastic and sclerosing agents.Massive chronic idiopathic pericardial effusions do not respond to medical treatment and tend to recur after pericardiocentesis, so wide anterior pericardiectomy is finally necessary in many cases.     

Successful treatment of refractory cardiac tamponade due to rheumatoid arthritis using pericardial drainage

Rheumatoid pericarditis occurs in patients with rheumatoid arthritis (RA). However, cardiac tamponade due to rheumatoid pericarditis is rare; we describe a case of a 72-year-old man with a 6-year history of rheumatoid arthritis who developed rheumatoid pericarditis with recurrent cardiac tamponade. The patient experienced relapse of the cardiac tamponade despite treatment with pericardiocentesis. Therefore, the patient underwent surgical pericardial drainage. The patient was also subsequently treated with increasing doses of corticosteroid, methotrexate and leukocytapheresis. These treatments resulted in a successful outcome without any complication. This case suggests that in addition to immunosuppressive therapy, pericardial drainage should be considered in the treatment of life-threatening refractory cardiac tamponade caused by rheumatoid arthritis.     

A clinicopathological study on pericardial heart disease in the aged

A total of 87 cases of pericardial heart disease (73 of pericarditis and 14 of hemopericardium) among 870 consecutive autopsies of aged patients was studied. Fibrinofibrouspericarditis was found in 80.8% of pericarditis, neoplastic in 13.7% and purulent in 5.5%. Representative cases of each type of pericarditis were illustrated. Among fibrinofibrous pericarditis, idiopathic was the most common and the other causes included irradiation, myocardial infarction, renal failure, rheumatoid arthritis and hypothyroidism. Frequent association of congestive heart failure or anasarca with mild to moderate fibrinofibrous pericarditis was noted. Clinical and morphologic evidences of pulmonary tuberculosis were present in nearly one third of cases with fibrinofibrious pericarditis, but actual incidence of tuberculous pericarditis could not be determined. Incidence of clinical signs and symptoms of acute pericarditis was evaluated with the stress on the relatively high incidence of supraventricular tachyarrhythmias, especially in cases with histological evidence of sinus node involvement in aged cases.     

Isolated nail fold vasculitis in rheumatoid arthritis.

OBJECTIVES--To establish the clinical significance of isolated nail fold vasculitis in patients with rheumatoid arthritis (RA). METHODS--Patients attending hospital with established RA were assessed by a single observer using unaided vision. Isolated nail fold vasculitis (NFV) was diagnosed as small nail edge or nail fold lesions without any evidence of other extra-articular or systemic vasculitis. Patients were followed prospectively. RESULTS--Thirty patients with isolated NFV were identified and followed for a median interval of 22 months. Three patients died within three months of diagnosis and six developed complications possibly indicative of extra-articular disease: xerostomia (three), pericarditis (one), pleural effusion and empyaema (one), pulmonary fibrosis (one). No patient developed systemic vasculitis. CONCLUSION--Isolated NFV has a favourable prognosis compared with systemic vasculitis. There is a low risk of developing systemic or extra-articular disease.     

Pericardial tamponade complicating rheumatoid arthritis: a case report

BACKGROUND: Cardiovascular involvement in rheumatoid arthritis (RA) is increasingly observed and may be associated with the severity of rheumatoid arthritis. It is dominated by heart ischemic diseases related to atherosclerosis. Specific rheumatoid heart disease is commonly asymptomatic and found at autopsy or by echocardiography. Pericarditis is the commonest cardiac complication of RA. It is rarely clinically apparent and pericardial tamponade is exceptional. CASE REPORT: Herein, we report an unusual case of a 53-year-old female patient with a six-year history of seropositive and erosive rheumatoid arthritis who had developed a pericarditis complicated with tamponade resolved by pericardiocenthesis and high dose systemic steroids. Histopathology showed chronic inflammation and fibrosis. Under 1mg/day of colchicine, there were no recurrences at 10 months. CONCLUSION: Pericarditis is uncommon in rheumatoid arthritis. Forms with constriction or tamponade may have a fatal outcome. Pericardectomy usually recommended in constrictive forms, is sometimes indicated for tamponade. Some observations and randomised studies of idiopathic pericarditis suggest that colchicine may be interesting for the treatment and prevention of recurrences of rheumatoid arthritis-associated pericarditis.     

Constrictive pericarditis in severe seronegative rheumatoid polyarthritis]

Constrictive pericarditis is a rare complication of rheumatoid arthritis, with 78 published cases. We report a new typical case where the pericardial disease was associated with a severe seronegative rheumatoid arthritis of 17 years duration. Constrictive pericarditis generally occurs in men (62.8% of all cases) aged 52.4 +/- 11.5 years. Its clinical features are identical with those of constrictive pericarditis due to other causes. Diagnosis rests on echocardiography and, chiefly, on right heart catheterization. The arthritis is seropositive in 85.7% of the cases, frequently nodular (75%) and advanced. There is no relation between its duration (mean: 9.6 +/- 7.4 years) and the occurrence of the pericardial pathology. The pericardial fluid has no specific abnormality. Histology shows fibrosis and a non-specific inflammatory cell infiltrate. Immunoglobulin and complement deposits in the walls of the pericardial vessels are detected by immunofluorescence. The only treatment is pericardiectomy; without it the disease is constantly lethal.     

Value of reference tracings in diagnosis and assessment of constrictive epi- and pericarditis

Reference tracings are of great value in the diagnosis and assessment of constrictive pericarditis. The Q-h interval in the jugular venous pulse tracing is strongly correlated with the mean right atrial pressure (r=0.91). The left ventricular ejection time, the Q-A2 interval, and the Q-h interval are independent during atrial fibrillation from the preceding diastolic filling interval. This differentiates constrictive pericarditis from valvular heart disease. Cases with haemodynamically significant constrictive epicarditis are characterized by a rapid evolution, absence of pericardial calcification and absence of an early diastolic filling sound, a dominant a wave in the jugular venous pulse tracing, and a high early diastolic ventricular pressure. The haemodynamic behaviour is similar to that found in cases with myocardial fibrosis.     

Cholesterol crystals pericarditis]

Cholesterol pericarditis is an uncommon, specific form of pericardial disease that is characterized by the presence of cholesterol crystals in pericardial fluid. The etiology may be either idiopathic or in association with systemic disorders such as tuberculosis, rheumatoid arthritis, myxedema or hypercholesterolemia. We report a case of cholesterol pericarditis in a 51-year-old male who was diagnosed with chronic renal failure due to polycystic kidney disease. To our knowledge no similar cases have been reported to date in the literature.