An unusual case of fatal pericarditis due to Listeria monocytogenes

Pericarditis due to Listeria monocytogenes is a very uncommon and serious disease. We describe a case of fatal subacute pericarditis that was caused by L. monocytogenes in a 61-year-old woman with Hodgkin's disease who was diagnosed in 1975 and considered cured. In addition, we review the literature on this condition.     

Chylous ascites due to constrictive pericarditis

Chylous ascites due to constrictive pericarditis is an extremely rare clinical entity, possibly caused by the augmented lymph production and high impedance to lymph drainage due to central venous hypertension. The authors describe a patient with chylous ascites caused by constrictive pericarditis in the absence of lymphatic obstruction. Cardiac catheterization is essential for the confirmation of accurate diagnosis of constrictive pericarditis. Magnetic resonance imaging of the heart is also very helpful in the diagnosis. The patient was symptom free and his ascites and edema completely resolved after pericardiectomy.     

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.     

Amyloid goitre in familial Mediterranean fever: report on three patients and review of the literature

Familial Mediterranean fever (FMF) is a hereditary disease, the most threatening complication of which is systemic amyloidosis. The thyroid gland may be asymptomatically involved in most patients with systemic amyloidosis secondary to FMF. However, clinically detectable thyroid goitre is quite rare, and until now only nine cases of thyroid goitre secondary to amyloid deposition in FMF have been reported. Of 1,100 FMF patients regularly followed up at our centre, thyroid goitre due to the accumulation of amyloid substance could be detected in only three (0.27%). In this report, we summarise the clinical and laboratory features of these patients. All three patients were euthyroid. Total thyroidectomy was performed for compressive symptoms in one patient and for aesthetic purposes in the other two. In countries with a high prevalence of FMF, such as Turkey, secondary amyloidosis of the thyroid gland should be borne in mind in long-standing FMF patients.     

Fine-needle aspiration biopsy from subcutaneous fat. An easy way to diagnose secondary amyloidosis

Secondary amyloidosis is usually diagnosed by demonstrating amyloid deposits on histological sections by Congo red staining. An alternative method is a fine needle aspiration biopsy (FNAB) from subcutaneous fat which, in this study, was carried out on 301 patients. In order to test the efficiency of FNAB we analysed 146 patients from whom in addition to FNAB one or two histological samples including 125 oral and 65 rectal biopsies were available. FNAB proved very reliable for demonstrating secondary amyloidosis as estimated by the index of sensitivity (0.82). The corresponding figures for rectal and oral biopsy were 0.97 and 0.64 respectively. Although the rectal biopsy proved to be the best method, we strongly recommend FNAB from the subcutaneous fat as the preferred method for screening and diagnosing secondary amyloidosis.     

Diagnosis of constrictive pericarditis by two-dimensional echocardiography: studies in a new experimental model and in patients

The purpose of this study was to determine the value of two-dimensional echocardiography in detecting constrictive pericarditis. Serial two-dimensional echocardiography was performed in eight closed chest conscious dogs with experimental constrictive pericarditis, using a new model that creates constrictive pericarditis by the introduction of a pericardial irritant mixture. Constrictive pericarditis was confirmed in these dogs by cardiac catheterization and pathologic examination. Four patients with constrictive pericarditis and three patients with restrictive cardiomyopathy (amyloidosis) were also studied. Analysis of short-axis two-dimensional echocardiograms was performed to determine the frame by frame change in left ventricular cavity areas throughout diastole. Curves of diastolic left ventricular cavity area change versus percent duration of diastole were constructed for each animal and human subject. Pericardial thickness was measured at various gain settings on two-dimensional and M-mode echocardiograms and at post-mortem examination. In dogs with constrictive pericarditis, the echocardiograms seriously overestimated and correlated poorly with pathologic measurements of pericardial thickness. In dogs after constrictive pericarditis developed, 69 +/- 11% (mean +/- SD) (range 50 to 84) of cavity area change occurred in the initial 30% of diastole compared with 35 +/- 7% (range 20 to 45) in control two-dimensional echocardiograms (p less than 0.001). Four patients with constrictive pericarditis showed similar accelerated cavity expansion in early diastole, but three patients with cardiac amyloidosis showed more variable left ventricular diastolic expansion rates. It is concluded that two-dimensional echocardiograms can demonstrate characteristic diastolic filling abnormalities in constrictive pericarditis, but cannot accurately measure pericardial thickness.     

An Unusual Presentation of Colon Cancer: Purulent Pericarditis and Cardiac Tamponade due to Bacteroides fragilis

Extra-abdominal infections caused by colonic anaerobic organisms have been shown to be associated with colorectal cancers. We report a very unusual case of a patient who suffered from cardiac tamponade secondary to anaerobic bacterial pericarditis caused by Bactericides fragilis as an initial presentation of colon cancer. The medical literature is reviewed.     

Primary cardiac amyloidosis: an angiocardiographic clue to early diagnosis

Restrictive cardiomyopathy produced by primary cardiac amyloidosis may have a clinical syndrome identical to noncalcific constrictive pericarditis. We report two patients with amyloidosis and restrictive physiology who had enlarged papillary muscles shown on left ventricular angiocardiogram. Rapid volume expansion with normal saline failed to produce ventricular filling pressure equilibration as in occult constrictive pericarditis. The absence of diastolic pressure equilibration and enlarged papillary muscles may permit early diagnosis of cardiac amyloidosis.     

Secondary amyloidosis in progressive systemic sclerosis

Progressive systemic sclerosis (PSS) is a connective tissue disease that may affect many organs, including the kidneys. It is quite rare to see secondary amyloidosis due to PSS. We present a patient with a 9-year history of PSS who developed nephrotic syndrome, and whose renal biopsy was compatible with secondary amyloidosis. He died from massive upper gastrointestinal bleeding caused by oesophageal telangiectasia. Received: 23 August 2000 / Accepted: 26 February 2001     

Tako-tsubo-like syndrome, a case report

In this report we briefly describe a 54-year-old woman who was referred to our institution for evaluation and management of newly diagnosed congestive heart failure associated with a skin rash. Detailed investigations revealed the presence of restrictive cardiomyopathy due to isolated primary cardiac amyloidosis as well as the presence of a skin disease named ‘porokeratosis of Mibellli’. Interestingly, porokeratotic lesions rarely have been associated with localized cutaneous amyloidosis. Presumably, porokeratosis induces secondary dermal amyloid deposition by a yet unknown mechanism. This is the first case of primary amyloidosis associated with porokeratosis reported in the literature.     

Renal transplantation in patients with familial Mediterranean fever

Amyloidosis is the most common and devastating complication of familial Mediterranean fever (FMF). Renal transplantation is the choice of treatment of in most end-stage renal disease (ESRD). We report our experience on the outcomes in eight patients who underwent renal transplantation for ESRD due to FMF secondary to amyloidosis, and we provide a discussion on the current evidence on this topic of study. The clinical charts of eight renal transplant patients (seven male, one female) who underwent ESRD due to FMF-related amyloidosis were investigated. Five patients underwent living-donor renal transplantation and three patients underwent deceased-donor renal transplant. The mean follow-up period was 35 months (range 3-72). All patients were on triple immunosuppressive treatment and received colchicine. All allografts are currently functioning well with a mean serum creatinine level of 1.4 (range 0.7-2.6) mg/dL. Posttransplantation complications included acute rejection (n = 4), chronic rejection (n = 1), severe gastroenteritis (n = 2), and erythrocytosis (n = 5). None of the patients had proteinuria. During follow-up, we did not observe clinically severe FMF attack, septicemia, rhabdomylosis, symptoms related to vasculitis, and clinical neuropathy. The clinical outcome of the patients in this cohort was similar to that of other renal transplant patients with ESRD due to other causes. This study shows favorable prognosis of eight ESRD patients due to amyloidosis caused by FMF after renal transplantation. Renal transplantation is a safe procedure for ESRD patients having amyloidosis due to FMF. Regular use of colchicine after transplantation should be mentioned.     

Campylobacter jejuni arthritis in secondary amyloidosis

Summary We describe a case of infectious arthritis caused by C. jejuni which developed after C. jejuni enteritis in a patient with rheumatoid arthritis and hypogammaglobulinaemia. Extraintestinal infections of this enteropathogen are rare, but in patients with hypogammaglobulinaemia C. jejuni bacteriaemia is relatively common. The present patient suffered from hypogrammaglobulinaemia due to a nephrotic syndrome in secondary amyloidosis. C. jejuni infection must be considered in patients with hypogammaglobulinaemia and arthritis.     

Amyloid goiter as the first clinical sign of systemic amyloidosis in a patient with adult-onset Still’s disease

We present a patient with amyloid goiter due to secondary amyloidosis which resulted from adult-onset Still’s disease. Amyloid goiter occurs as one of the rare manifestations of systemic amyloidosis. In our case the initial manifestation of amyloidosis was a fast growing goiter without other clinical abnormalities, which is unusual in the context of previously reported cases.     

Fulminant hepatic failure due to secondary amyloidosis

Systemic amyloidosis frequently involves liver, however, clinically apparent liver disease is rare and, if observed, is usually mild. Rarely, primary amyloidosis may present with evidence of portal hypertension or hepatic failure. Although secondary amyloidosis may involve the liver and rarely causes liver pathologies such as cholestatic hepatitis and portal hypertension, to our knowledge, hepatic failure due to secondary amyloidosis has been reported once before. Herein, we presented a woman with subacute fulminant hepatic failure who had no known history of a chronic disease other than episodes of urinary tract infection, and secondary amyloidosis was diagnosed with post-mortem liver biopsy.     

Primary meningococcal pericarditis caused by meningococcus serogroup C

We report the case of a 15-year-old female, with no previous medical history, who presented cardiac tamponade secondary to purulent pericarditis caused by Neisseria meningitidis serogroup C. This microorganism is the etiologic agent in 6-16% of the cases of purulent pericarditis, most in association with previous or concomitant central nervous system involvement (meningitis). Exceptionally, as in this case, the pericarditis is not accompanied by meningitis (Primary Meningococcal Pericarditis). The patient was treated with antibiotics, pericardiocentesis and steroids with excellent response. It is important to point out that meningococcal disease may present in unusual forms which may lead to diagnostic and therapeutics difficulties.     

Cardiac amyloidosis masquerading as constrictive pericarditis

Cardiac amyloidosis cannot always be differentiated from constrictive pericarditis even after careful review of the clinical, laboratory, and hemodynamic data prior to pericardial exploration. This case report underscores the necessity of precise histologic examination for cardiac amyloidosis in all suspected cases of constrictive pericarditis at the time of pericardiectomy.     

Lysozyme amyloidosis: report of 4 cases and a review of the literature

Autosomal dominant hereditary amyloidosis represents not 1 disease but a group of diseases, each the result of mutations in a specific protein. The most common form is transthyretin amyloidosis, which has been recognized clinically for over 50 years as a familial polyneuropathy. Nonneuropathic amyloidoses (Ostertag type amyloidosis) include those due to abnormalities in lysozyme, fibrinogen Aalpha-chain, and apolipoprotein A-I and A-II. The role of lysozyme in amyloid-related human disorders was first described in 1993; to date, there have been only 9 publications describing this disorder, which is a nonneuropathic form of hereditary amyloidosis. Reported cases have involved 7 unrelated families. We describe here our own experience with 4 families suffering from lysozyme amyloidosis: the first had prominent renal manifestations with sicca syndrome, the second and third had prominent gastrointestinal symptoms, and the fourth had a dramatic bleeding event due to rupture of abdominal lymph nodes. To our knowledge, this last symptom has not been reported previously, but is reminiscent of the hepatic hemorrhage seen in a previously reported case of a patient with lysozyme amyloidosis. To characterize the manifestations of this disorder, we performed an exhaustive literature review.Although hereditary amyloidosis is thought to be a rare disease, it is probably not as rare as we think and may well be underdiagnosed. Moreover, some cases of lysozyme amyloidosis are probably confused with acquired monoclonal immunoglobulin light-chain (AL) amyloidosis, formerly known as primary amyloidosis, which is the most frequent type of amyloidosis. Because treatment for each type of amyloidosis is different, and because therapy directed at 1 type may worsen symptoms of the other types, it is important to determine precisely the nature of the amyloid protein. Thus, hereditary lysozyme amyloidosis should be considered in all patients with systemic amyloidosis, particularly in patients who present with renal, gastrointestinal, or bleeding complications without evidence of AL or AA (secondary) amyloidoses.     

Amyloid goiter as the first clinical sign of systemic amyloidosis in a patient with adult-onset Still’s disease

Abstract

We present a patient with amyloid goiter due to secondary amyloidosis which resulted from adult-onset Still’s disease. Amyloid goiter occurs as one of the rare manifestations of systemic amyloidosis. In our case the initial manifestation of amyloidosis was a fast growing goiter without other clinical abnormalities, which is unusual in the context of previously reported cases.     

Rheumatic heart disease associated with secondary renal amyloidosis

Introduction: Amyloidosis is a disorder of protein folding in which normally soluble proteins undergo conformational changes and are deposited in the extracellular space in an abnormal fibrillar form. Accumulation of these fibrils causes progressive disruption of the structure and function of tissues and organs, and the systemic forms of amyloidosis are frequently fatal. The conditions that underlie amyloid deposition may be either acquired or hereditary. Amyloid-A (AA) amyloidosis is the most common form of systemic amyloidosis worldwide, AA amyloidosis occurs in the course of chronic inflammatory diseases, hereditary periodic fevers, and with certain neoplasms such as Hodgkin disease and renal cell carcinoma. Amyloidosis due to rheumatic heart disease (RHD) is not common but can be seen. We report here a patient with RHD and AA renal amyloidosis. Case Report: we present a 30 year-old Egyptian male with a history of RHD, accidently discovered to have nephrotic range proteinuria and rising serum creatinine. Serology studies were negative or normal, including antinuclear antibody (ANA) and antineutrophil cytoplasmic antibody (ANCA). C3 and C4 complement levels were normal. Kidney biopsy revealed AA renal amyloidosis. CT chest and abdomen revealed bilateral hilar and mediastinal lymphadenopathy and para-aortic lymph nodes. Endobronchial biopsy and bronchoalveolar lavage revealed non-specific chronic inflammatory changes. The patient's secondary amyloidosis was presumed to be related to the long standing RHD after exclusion of other causes of secondary amyloidosis. The patient finally died due to heart failure and acute pulmonary edema. Conclusion: Long standing RHD can lead to secondary AA amyloidosis. Keywords: AA Amyloidosis; Kidney Biopsy; Rheumatic Heart Disease; Renal Amyloidosis.     

Primary systemic amyloidosis presenting as constrictive pericarditis

The most frequent presentation of cardiac amyloidosis is with endomyocardial deposition, and resultant restrictive cardiomyopathy. We present a case of primary systemic amyloidosis causing constrictive pericarditis (CP) and congestive heart failure without clinical evidence of endomyocardial deposition. A comprehensive evaluation by noninvasive and invasive studies facilitated the differentiation of CP from restrictive cardiomyopathy and the patient was effectively treated with pericardectomy. To our knowledge, this is the first documented case of primary systemic amyloidosis causing selective CP with successful antemortem diagnosis and treatment in a young man.