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Pericarditis following permanent pacemaker insertion.

BACKGROUND: The appearance of pericarditis following insertion of a permanent pacemaker is not widely acknowledged in the literature. OBJECTIVES: To describe our experience with pericarditis following 395 permanent pacemaker implantations over 2 years. METHODS: We retrospectively reviewed the medical records of 395 consecutive patients in whom new pacing systems or pacemaker leads had been implanted over a 2 year period. We searched the records for pericarditis that developed within 1 month after pacemaker implantation according to the ICD-9 code. The incidence, clinical picture, response to treatment, and relationship to lead design and location were studied. RESULTS: Eight cases (2%) of pericarditis following implantation were detected. Clinical manifestations in all patients were similar to those of post-pericardiotomy syndrome and included chest pain (n = 7), friction rub (n = 1), fever (n = 2), fatigue (n = 2), pleural effusion (n = 2), new atrial fibrillation (n = 2), elevated erythrocyte sedimentation rate (n = 4), and echcardiographic evidence of pericardial effusion (n = 8). All affected patients had undergone active fixation (screw-in) lead implantation in the atrial position. The incidence of pericarditis with screw-in atrial leads was 3% compared to 0% in other cases (P < 0.05). CONCLUSIONS: Pericarditis is not uncommon following pacemaker implantation with active fixation atrial leads. Special attention should be paid to identifying pericardial complications following pacemaker implantation, especially when anticoagulant therapy is resumed or initiated. The use of passive fixation leads is likely to reduce the incidence of pericarditis but this issue should be further investigated.

Adrenal Cortex Hormones↗

Surgical treatment of constrictive pericarditis: analysis of outcome and diagnostic error.

The records of 81 patients with a diagnosis of constrictive pericarditis who underwent surgical treatment were examined to assess the effectiveness of diagnosis and therapy. A false-positive diagnosis occurred in 10 patients (12%); seven had restrictive cardiomyopathy. Of 51 variables examined, only a low right ventricular end-diastolic pressure (RVEDP) significantly and independently predicted diagnostic error. Seventy-one patients with constrictive pericarditis underwent pericardiectomy. Mean follow-up was 4.7 years (maximum 12), and only two patients were untraceable. The study population was notable: 42 patients had visceral as well as parietal pericardial involvement; 32 had idiopathic disease and 25 had pericarditis related to radiation therapy. Results were favorable in 83% of the population. There were seven in-hospital deaths (10%). Actuarial survival estimates were 74% and 64% at 5 and 10 years, respectively. Compared with a normal population, the survival rate of patients with postradiation constrictive pericarditis was significantly inferior, whereas the survival rate of the remaining patients was not significantly different. Patients with constrictive pericarditis and restrictive cardiomyopathy did no better than those with restrictive cardiomyopathy alone. Additionally, patients in NYHA functional class IV had a significantly worse prognosis. Multivariate analysis of 38 preoperative variables identified high RVEDP as a significant independent predictor of in-hospital death, and renal dysfunction and diuretic use were significant independent predictors of overall poor outcome. Differentiation between the diagnosis of constrictive pericarditis and restrictive cardiomyopathy remains a problem. Radiation therapy, pericarditis with restrictive cardiomyopathy, high RVEDP, NYHA class IV status, renal dysfunction, and diuretic use adversely influenced outcome in patients undergoing pericardiectomy.

Actuarial Analysis↗

[Post-radiotherapy pericarditis. 5 cases (author's transl)].

These cases represented 2.8% of the patients admitted for pericarditis during the same period. The cause of irradiation was a carcinoma of the breast in three cases and a carcinoma of the oesophagus in two cases. Acute forms of pericarditis occured 8 and 13 months after irradiations, and chronic forms after 1, 8 and 13 years. Three clinical forms were observed; two patients had an acute form; the first one with a slight effusion was easily cured, the second with cardiac tamponnade recovered after surgical evacuation, two others patients had a chronic latent effusion; after surgical evacuation, one recovered but the other one developped an occult constrictive pericarditis diagnosed by rapid volume expansion. The fifth case was a constrictive pericarditis which was effectively traited by pericardectomy. The difficulty of etiological diagnosis varies with the time and the amount of effusion. When the effusion is moderate the distinction must be made with an acute idiopathic pericarditis; when effusion is large the distinction must be made with a tuberculosis and specially a tumoral recurrence; in three cases pericardial biopsy was carried out and eliminated these diagnosises; lesions were similar: pericard was sclerous and little in cells, inflammatory signs were slight or absent. The postoperative prognosis in constrictive pericarditis may be agravated by associated myocardial lesions.

Acute Disease↗

[Scintigraphic diagnosis of experimentally induced pericarditis in dogs using 99mTc-labelled pyrophosphate (author's transl)].

In the present paper the possibilities of scintigraphic diagnosis of experimental pericarditis are discussed. Pericarditis was induced by either talc or formaldehyde intrapericardially. In all animals the administration of talc as well as formaldehyde produced inflammatory changes which were more serious after formaldehyde. The scan of the talc pericarditis was carried out on the 7th day after operation and of the formaldehyde pericarditis on the 3rd, 7th or 14th days. No positive scintigraphic finding was made in any of the experimental animals, apart from a massive incorporation in the operation scars. The counting of radioactive impulses after the exstirpation of the tissue samples confirmed the scintigraphic findings. Statistical evaluation did not reveal any essential difference between the accumulation of the radiopharmaceutical into the pericarditis as well as into the pericardial bag in comparison with the control animals. Although our model does not exactly reflect the situation of patients suffering from pericarditis it may be assumed that pyrophosphate scintigraphy is not suitable for the diagnosis of acute pericarditis in clinical practice.

Animals↗

The efficacy of indomethacin in the treatment of uremic pericarditis.

Uremic pericarditis is common in patients undergoing chronic hemodialysis and has been difficult to cure using conservative medical and surgical methods of treatment. Indomethacin therapy for uremic pericarditis has been reported infrequently. We present a case of uremic pericarditis in which indomethacin was particularly effective. The patient was a 45-year-old woman with thrombocytopenia due to Banti's syndrome and congestive heart failure with pericardial effusion. She had been undergoing hemodialysis for 3 years for chronic renal failure due to chronic glomerulonephritis. Uremic pericarditis was diagnosed based on serological laboratory results, aspirated pericardial fluid, and an echocardiogram. She was treated with oral doses of 25 mg t.i.d. indomethacin in addition to hemodialysis and extracorporeal ultrafiltration method. Pericardial effusion and left ventricular dysfunction disappeared after indomethacin therapy for 38 days. There are conflicting reports on the efficacy of indomethacin therapy in patients with uremic pericarditis. While indomethacin therapy was very effective in our case, there may be many causes of uremic pericarditis for which indomethacin may not be efficacious. Further investigations of indomethacin therapy for patients with uremic pericarditis are necessary to elucidate the therapeutic mechanism by which indomethacin acts.

Administration, Oral↗

Clinical response of tuberculous pericarditis to medical treatment: a retrospective survey.

BACKGROUND: The prevalence of tuberculosis has declined with advanced antituberculous chemotherapy. However, the occurrence of subsequent constrictive pericarditis in tuberculosis has not reduced. Clinical progress of tuberculous pericarditis was investigated in patients receiving antituberculous chemotherapy. METHODS: Thirteen patients with tuberculous pericarditis (11 men and 2 women aged 14 to 86 years [mean 60.0 +/- 17.0]), treated initially with antituberculous medications were analyzed. All patients underwent pericardiocentesis on admission following echocardiography. RESULTS: Dyspnea was the most common clinical pictures. Bloody effusion fluids were noted in 10 patients. Moreover, the lymphocytic fluids were present in 78% of all patients. The neutrophilic fluids tended to have a bloody color, and there was progress to constrictive pericarditis even for those on anti-tuberculous chemotherapy. Five patients with subsequent constrictive pericarditis received pericardiectomy within 3.5 months of admission. Three of them received pericardiectomy later, despite pericardial window procedure. CONCLUSIONS: Follow-up by echocardiogram is necessary within three months after commencing medical treatment because of the high incidence of progression constrictive pericarditis despite aggressive medical treatment. Pericardiectomy seems to be the only solution to the catastrophic outcome of constrictive pericarditis.

Adolescent↗

Uremic pericarditis: a prospective echocardiographic and clinical study.

During a 14 month period 59 male patients with chronic renal failure who were candidates for chronic hemodialysis (HD) were evaluated clinically and echocardiographically for pericarditis. All were evaluated prospectively prior to or at the initiation of HD. Definite pericarditis was present in 8, all of whom were severely uremic and required initiation of HD on a semiemergent basis rather than electively (i.e., preselected level of renal function). In 6 of these 8, pericardial effusion responded to dialysis alone, one required pericardiectomy because of hypotension complicating dialysis, and one expired during a right atriogram. Patients dialyzed on an elective basis were all free of pericarditis at the initiation of HD. Pericarditis arising some months after the initiation of HD was a less frequent problem. It is concluded that (a) the incidence of pericarditis in the uremic state is decreased by early initiation of HD before advanced uremic symptoms have developed; (b) pericarditis present at the initiation of HD usually but not always is resolved with the initiation of HD; (c) echocardiography is an important clinical and epidemiological toole to investigate pericarditis in uremic patient populations.

Echocardiography↗

Comparison of PCR, culture, and histopathology for diagnosis of tuberculous pericarditis.

Nucleic acid amplification techniques for the diagnosis of tuberculosis (TB) are rapidly being developed. Scant work, however, has focused on pericardial TB. Using cryopreserved specimens from a prior study of pericarditis, we compared PCR to culture and histopathology for the diagnosis of tuberculous pericarditis in 36 specimens of pericardial fluid and 19 specimens of pericardial tissue from 20 patients. Fluid and tissue were cultured on Lowenstein-Jensen and Middlebrook solid media and in BACTEC radiometric broth. Tissue specimens were stained with hematoxylin-eosin, Ziehl-Neelsen, auramine O, and Kinyoun stains and were examined for granuloma formation and acid-fast bacilli. PCR was performed with both fluid and tissue with IS6110-based primers specific for the Mycobacterium tuberculosis complex by published methods. Sixteen of the 20 patients had tuberculous pericarditis and 4 patients had other diagnoses. TB was correctly diagnosed by culture in 15 (93%) patients, by PCR in 13 (81%) patients, and by histology in 13 of 15 (87%) patients. PCR gave one false-positive result for a patient with Staphylococcus aureus pericarditis. Considering the individual specimens as the unit of analysis, M. tuberculosis was identified by culture in 30 of 43 specimens (70%) from patients with tuberculous pericarditis and by PCR in 14 of 28 specimens (50%) from patients with tuberculous pericarditis (P > 0.15). The sensitivity of PCR was higher with tissue specimens (12 of 15; 80%) than with fluid specimens (2 of 13; 15%; P = 0.002). In conclusion, the overall accuracy of PCR approached the results of conventional methods, although PCR was much faster. Therefore, PCR merits further development in this regard. The sensitivity of PCR with pericardial fluid was poor, and false-positive results with PCR remain a concern.

Adolescent↗

[Pericardectomy and acute infectious pericarditis].

Between 1980 and 1990 12 patients (5 male, 7 female) were operated on for acute infectious pericarditis at a mean age of 42 years. The infections were 6 bacterial (purulent 4, abscess 2), 4 tuberculous, 1 viral and 1 Candida. Pericarditis resulted from contiguous spread of infection from bilateral pneumonia in 3 patients, from subphrenic abscess in 2 and followed bacteremia in 1. Clinical signs were: tamponade/shock in 9, elevated jugular venous pressure in 11, edema in 6, hepatomegaly in 6, ascites in 1, and pericardial friction rub in 3. A preoperative pericardiocentesis in 9 patients allowed only 4 positive microbiological diagnoses and was an insufficient drainage in all cases. The preoperative mean NYHA class was 3.3. The pericardectomy was total in 9 patients and partial in 3. Total mortality was 1/12 patients (8%) with one late death due to recurrent tuberculous pericarditis. No patient with purulent pericarditis died. Another recurrence occurred 6 months after acute viral pericarditis. Atrial fibrillation in one patient was the only postoperative complication. After a mean follow-up period of 48.5 months no cardiac constriction had occurred in 11 surviving patients Actuarial survival after pericardectomy is 100% after 1 month and remains 91% after 5 years. The mean NYHA class has significantly improved to 1.2 (p less than 0.05) at the end of the follow-up. We conclude that pericardectomy combined with a specific antimicrobial therapy is a safe treatment for acute infectious and especially purulent pericarditis with low mortality and excellent longterm results. Early pericardectomy allows rapid decompression of the heart, removal of intrapericardial adhesions and infected tissue and prevents late constriction.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗

[Pericarditis: classification, etiology, pathogenesis].

The most frequent pericardial diseases are inflammations which in surviving patients are recognized much less frequently comparing with autopsy material. The most frequent causes of pericarditis include various infections, inflammations associated with systemic diseases, acute myocardial infarction, traumas and iatrogenic lesions, and neoplasms. Viral etiology is most often in infectious pericarditis. A considerable percentage of idiopathic pericarditis is most probably of such etiology. Bacterial (purulent) pericarditis in contrast to viral one is characterized by high mortality and frequent progression of acute to chronic type. The latter with the symptoms of pericardial constriction occurs in at least 50% of the patients with tuberculous etiology irrespective of specific treatment. In recent years the number of iatrogenic pericarditis has increased related to hypersensitivity or autoimmunity. One should mention here post-cardiotomy syndrome, pacemaker implantation, diagnostic procedures such as biopsy, cardiac catheterization, hypersensitivity to drugs and radiation pericarditis as a complication of therapy.

Acute Disease↗

Aspergillus pericarditis: clinical and pathologic features in the immunocompromised patient.

There has been a proliferation of infectious complications due to Aspergillus in patients receiving chemotherapy for cancer and transplantation; however, aspergillus pericarditis has been rarely described. Reported here are the clinical and pathologic findings of Aspergillus pericarditis in six immunocomprised patients who came to autopsy in the past 11 years. Five had leukemia, one had received a renal transplant. All had pulmonary aspergillosis. Two had clinically overt pericarditis leading to cardiac tamponade and death. Pulmonary aspergillosis preceded development of pericardial signs. Chest radiographs, serial electrocardiograms, and echocardiograms showed abnormality but were nonspecific. Pericardiocentesis was performed in one patient but proved nondiagnostic and yielded only transient hemodynamic improvement; postmortem Gram stain of the spun sediment of that pericardial fluid revealed branched hyphae. Although five patients received Amphotericin B, whether it entered the pericardial space is uncertain. Postmortem examination revealed extensive pericardial involvement by Aspergillus associated with effusions as large as 1000 ml Aspergillus penetrated the pericardium by rupture of myocardial abscesses and invasion from contiguous pulmonary foci into the pericardial space. A clinical diagnosis of Aspergillus pericarditis was never established, and at least two died of their pericardial disease. Aspergillus pericarditis is a lethal cardiac infection, which is likely to increase in frequency, and should be considered in the hemodynamically unstable immunocompromised patient, especially when signs of pericarditis or pulmonary aspergillosis are present.

Adolescent↗

The etiologic spectrum of constrictive pericarditis.

Ninety-five consecutive patients with constrictive pericarditis that was documented at the time of surgery during 1970 to 1985 were reviewed. The etiologies included idiopathic (42%), postradiotherapy (31%), post-cardiac surgery (11%), postinfective (6%), connective tissue disease-related (4%), neoplastic (3%) uremic (2%), and sarcoidosis (1%). Post-cardiac surgery etiology was seen only after 1980, but constituted 29% of cases during 1980-1985. Postradiotherapy etiology occurred with equal incidence in 1980-1985 and in 1970-1980, but the interval from radiotherapy to presentation with constrictive pericarditis was longer in the more recent period (11 vs 4.75 years). Effusive constrictive pericarditis occurred in 24% overall with similar prevalence in all of the etiologic groups except the postsurgical cases, which were caused by noneffusive fibrous constrictive pericarditis in all instances. Operative mortality was 12% overall: It was lower in the idiopathic group (8%) and higher in the postradiotherapy group (21%). Thus postradiotherapy constrictive pericarditis continues to occur despite technical changes aimed at reducing its likelihood, but recent cases have a longer latent period: and postsurgical constrictive pericarditis has emerged as an important etiology.

Adult↗

Abnormal left ventricular-left atrial posterior wall contour: a new two-dimensional echocardiographic sign in constrictive pericarditis.

We measured the angle P formed by the junction of the left ventricular and left atrial posterior walls, and also the distances from the ultrasound transducer to the left ventricular posterior wall and left atrial posterior wall (DV and DA), respectively, in the parasternal long-axis two-dimensional echocardiographic view. We studied 23 normal adults and four patient groups with conditions commonly associated with left atrial dilatation: mitral regurgitation (14), mitral stenosis (16), hypertrophic cardiomyopathy (13), and constrictive pericarditis (7). Statistically significant differences were found between the constrictive pericarditis group and each of the other groups. Angle P was less than 150 degrees in 0 of 23 normal individuals, in 0 of 14 with mitral regurgitation, in 1 of 16 with mitral stenosis, in 0 of 13 with hypertrophic cardiomyopathy, and in five of seven with constrictive pericarditis. DA minus DV exceeded 20 mm in 0 of 23 normal individuals, one of four with mitral regurgitation, in 6 of 16 with mitral stenosis, in 0 of 13 with hypertrophic cardiomyopathy, and in five of seven patients with constrictive pericarditis; We conclude that angle P greater than 150 degrees suggests constrictive pericarditis; DA minus DV greater than 20 mm suggests constrictive pericarditis if mitral stenosis can be excluded.

Echocardiography↗

Left atrial dilatation in constrictive pericarditis: a pre and post-operative echocardiographic study.

Thirty-three surgically proven cases of constrictive pericarditis were studied pre-operatively by echocardiography for left atrial dilatation and 18 of them underwent post-operative study at a mean follow-up period of 229 +/- 105 days. The degree of left atrial dilatation in these patients was compared with patients of restrictive cardiomyopathy (n = 8) and sex matched controls of similar age (n = 33). Significant left atrial dilatation was present in patients of constrictive pericarditis compared to controls and it was of a similar or greater degree compared to patients of restrictive cardiomyopathy (the left atrium to aorta ratio was 1.7 +/- 0.31, 1.53 +/- 0.18 and 1.07 +/- 0.1, in constrictive pericarditis, restrictive cardiomyopathy and controls, respectively, P = NS for constrictive pericarditis vs. restrictive cardiomyopathy and P < 0.001 for constrictive pericarditis vs. controls). There was no correlation of degree of left atrial dilatation with clinical and hemodynamic variables. Postoperatively, there was regression of left atrial size in patients with normal hemodynamics (n = 12, pre- vs. post-operative left atrium to aorta ratio 1.65 +/- 0.23 vs. 1.32 +/- 0.14, P < or = 0.001) and persisting or increasing left atrial dilatation in patients with persisting hemodynamic abnormality (n = 6, left atrium to aorta ratio 1.66 +/- 0.23 vs. 1.82 +/- 0.15, P = NS). We conclude that significant left atrial dilatation is a consistent echocardiographic feature of constrictive pericarditis. Hemodynamic normalization following successful pericardiectomy is associated with regression of atrial size.

Adolescent↗

Tuberculous pericarditis: ten year experience with a prospective protocol for diagnosis and treatment.

Thirteen patients with tuberculous pericarditis (12 men and 1 woman aged 13 to 70 years [mean 41]) were identified in a group of 294 patients consecutively admitted for primary acute pericardial disease. The diagnosis was made by the following studies: sputum culture (n = 4), culture of pericardial fluid obtained by pericardiocentesis (n = 3), histologic study and culture of pericardial biopsy (n = 3), lymph node biopsy (n = 2) and pleural biopsy (n = 1). Clinical presentation was remarkably variable: four patients had an acute, apparently self-limited course, one had relapsing tamponade, four had tamponade effectively treated with pericardiocentesis and four had toxic symptoms with persistent fever. The interval from hospital admission to diagnosis ranged from 1 to 14 weeks (mean 5.2). Constrictive pericarditis developed in six patients and effusive-constrictive pericarditis in one; all seven required pericardiectomy 2 to 3.5 months after admission. No patient died. It is concluded that 1) tuberculous pericarditis has a variable clinical presentation and therefore it should be considered in the evaluation of all instances of pericarditis without a rapidly self-limited course; 2) the diagnosis should be based only on objective data obtained with a systematic study protocol; 3) early definitive diagnosis is still difficult to achieve; and 4) development of subacute constrictive pericarditis requiring pericardiectomy is common.

Acute Disease↗

Immunosuppressive therapy prevents recurrent pericarditis.

OBJECTIVES: This study reviews the clinical outcome of a series of patients with recurrent pericarditis before and after immunosuppressive therapy. BACKGROUND: Despite anti-inflammatory treatment, some patients with acute pericarditis experience repeated relapses of the disease. The use of steroids for the treatment of recurrent pericarditis remains controversial. METHODS: Twelve patients (4 women, 8 men; mean [+/- SD] age 35.9 +/- 17.2 years, range 15 to 65) with recurrent pericarditis unrelated to any systemic disease were selected. All 12 patients previously received ineffective short-term courses of low dose steroids and had a total of 39 relapses during a mean follow-up period of 14.2 months (range 4 to 50). A 3-month course of treatment with prednisone, at an immunosuppressive dosage, was started (1 to 1.5 mg/kg body weight per day for 4 weeks, then gradually withdrawn). When prednisone reduction was undertaken, all patients started a 5-month course of treatment with aspirin (1.6 g/day until steroid suspension, then reduced to 0.8 g/day). RESULTS: During a mean follow-up period of 41.6 months (range 7 to 104), immunosuppressive treatment with high dose prednisone resulted in stable remission in all except one patient, who experienced one relapse. In this patient, the addition of azathioprine to prednisone induced a persistent remission, which remained after 1-year follow-up. During treatment, three patients had severe steroid-related adverse effects that in two patients required replacement of prednisone with azathioprine and cyclophosphamide, respectively. This variation in the immunosuppressive regimen did not modify the favorable clinical outcome. CONCLUSIONS: The dose and duration of steroid treatment are critical factors in preventing recurrent pericarditis. High dose prednisone with aspirin should be considered in the treatment of recurrent pericarditis resistant to anti-inflammatory therapy. Cyclophosphamide or azathioprine should be reserved for patients who do not respond to high dose prednisone or who experience severe complications related to steroid therapy.

Adolescent↗

Differentiation of constrictive pericarditis from restrictive cardiomyopathy using mitral annular velocity by tissue Doppler echocardiography.

This study evaluated the diagnostic role of early diastolic mitral annular velocity (E') by tissue Doppler echocardiography for differentiating constrictive pericarditis from restrictive cardiomyopathy (primary restrictive cardiomyopathy and cardiac amyloidosis). The study group consisted of 75 patients (53 men, 22 women; mean age 62 years, range 27 to 87). Of these, 23 patients had surgically confirmed constrictive pericarditis, 38 had biopsy-proved systemic amyloidosis and typical echocardiographic features of cardiac involvement, and 14 had primary restrictive cardiomyopathy. Standard mitral inflow characteristics were measured. Tissue Doppler echocardiography was used to measure E' at the septal annulus. E' was significantly higher in patients with constrictive pericarditis than in those with primary restrictive cardiomyopathy or cardiac amyloidosis (12.3 vs 5.1 cm/second, p <0.001). An E' cut-off value > or =8 cm/second resulted in 95% sensitivity and 96% specificity for the diagnosis of constrictive pericarditis. There was no overlap of E' between patients who had constrictive pericarditis and those who had cardiac amyloidosis. In a subgroup analysis of restrictive cardiomyopathy, E' of patients who had cardiac amyloidosis was significantly lower than that of patients who had primary restrictive cardiomyopathy (4.6 vs 6.3 cm/second, p <0.001). Thus, E' velocity can distinguish between constrictive pericarditis and restrictive cardiomyopathy with a specific cut-off value in patients with clinical and echocardiographic evidence of diastolic heart failure.

Aged↗

Long-term outcomes in difficult-to-treat patients with recurrent pericarditis.

Patients with many recurrences of acute pericarditis are commonly alarmed by the fear of constriction. We studied their long-term outcome and the possible presence of systemic diseases. Sixty-one Italian patients (36 men) were followed for an average of 8.3 years according to a predefined protocol, including testing for autoimmune diseases and familial Mediterranean fever. Symptomatic pericarditis lasted from 1 to 43 years (mean 5.4 years). Fifty-two patients had been referred to us after failure of previous therapies, including steroids. We observed 378 attacks with a mean of 1.6 per patient per year and 156 hospital admissions. Thirteen patients had a post-cardiac injury syndrome. In 43 (70.5%), the pericarditis remained idiopathic, whereas we made a new diagnosis of rheumatoid arthritis in 1 and of Sjogren's syndrome in 4 patients, but in these patients pericarditis represented the dominant clinical manifestation. Cardiac tamponade occurred during the initial attacks in 4 patients (6.5%) but never recurred. Pleural effusions were present during the first attack in 22 patients (36.0%) and liver involvement in 5 (8%). No patients developed constrictive pericarditis. Echocardiographic examination produced no evidence of chronic myocardial disease. Response to therapy was good. Thirty-one patients (50.8%) are in sustained remission, without any therapy; their total observation period has averaged 10.3 years. In idiopathic patients, antinuclear antibodies were present in 56.2% and anti-Ro/SSA in 8.3%. Mutations linked to familial Mediterranean fever were absent. In conclusion, in this large series of difficult patients with recurrent acute pericarditis and a very long follow-up, the long-term prognosis is good.

Acute Disease↗