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PubMed1995

[The detection of secondary amyloidosis in rheumatoid arthritis by aspiration biopsy of the subcutaneous fatty tissue].

81 rheumatoid arthritis patients were regularly and periodically examined in rheumatological department of Tartu University Clinic. Depending on the presence of amyloid in subcutaneous fat biopsies the patients were divided into two comparable groups: amyloid-positive (group 1 of 22 patients) and amyloid-negative (group 2 of 59 patients). Between the groups some differences were found: group 1 patients had more active and severe course of rheumatoid arthritis and higher level of rheumatoid factor and circulating immune complexes in the sera. Before biopsy secondary amyloidosis was diagnosed in 17.4% of the examines, biopsy of abdominal subcutaneous fat increased this rate to 27.2%. We could not ascertain any effect of previous treatment on secondary amyloidosis development except the connection with long-term use of corticosteroids. The analysis of 10379 autopsies showed that cases of RA with amyloidosis account for 30.9% of all amyloidosis cases. Multiple stepwise regression analysis determined predisposition factors to secondary amyloidosis as severity of locomotor system involvement, long-term use of corticosteroids, high level of rheumatoid factor and activity of rheumatoid process.

Abdomen↗
PubMed1996

Dose-intensive melphalan with blood stem cell support for the treatment of AL amyloidosis: one-year follow-up in five patients.

The morbidity and lethality of AL amyloidosis is caused by the deposition of lg light chains as fibrillar amyloid protein in vital organs, disrupting their function, and not by the generally low burden of clonal plasma cells that produce the paraproteins. Survival of patients with AL amyloidosis is no more than 1 to 2 years from the time of diagnosis with current management approaches. Clearly, more effective therapies are needed for this rapidly lethal disease. Five patients were treated with dose-intensive melphalan and blood stem cell support and followed for a period of 1 year. Patients were diagnosed with AL amyloidosis by tissue biopsy and categorized by performance status and organ involvement. Their plasma cell dyscrasias were evaluated with immunofixation electrophoresis of serum and urine specimens, quantitative serum lgs, and immunohistochemical staining of bone marrow biopsy specimens. After treatment with dose-intensive intravenous melphalan followed by infusion of autologous growth-factor-mobilized blood stem cells, clinical evaluations and plasma cell studies were repeated at 3 and 12 months. Three men and 2 women aged 38 to 53 years were treated. Median performance status (SWOG) was 2 (1 to 3), and clinical presentations included nephrotic syndrome (n = 1), symptomatic cardiomyopathy (n = 1), gastrointestinal involvement with polyneuropathy (n = 2), and hepatomegaly (n = 1). With a median follow-up of 13 months (12 to 17 months), all five patients are well and have shown stable or improved performance status and clinical remission of organ-related dysfunction, including a 50% reduction in daily proteinuria with no change in creatinine, reversal of symptoms of cardiomyopathy and reductions of posterior wall and septal thickening, reversal of polyneuropathy and gastric atony, and resolution of hepatomegaly by computed tomographic scan. In 3 of the 5 patients (60%) at 12 months after treatment, plasma cell dyscrasias could not be detected. Dose-intensive chemotherapy with intravenous melphalan and growth-factor-mobilized blood stem cell support is feasible therapy for patients with AL amyloidosis, even when there is clinical evidence of cardiac involvement. At least some patients with AL amyloidosis achieve complete remission of their plasma cell dyscrasia, improvement in performance status, and clinical remission of organ-specific disease after this form of treatment.

Adult↗
PubMed1996

[Pleural amyloidosis].

Pleural involvement in primary (AL) amyloidosis was reported. Pulmonary involvement in patients with primary amyloidosis is not so uncommon, but the pleura is regarded as an unusual site for amyloid deposition. A woman, born in 1942, was referred for evaluation of a four-month history of edema of eye lids and lower legs in 1987. Nephrotic syndrome with Bence Jones (lambda-type) proteinuria was demonstrated. She was diagnosed of having renal amyloidosis. In 1990, X-ray films incidentally demonstrated a small amount of right pleural effusion. A needle biopsy of the pleura was performed. Congo red stain demonstrated amyloid deposition in the pleura. The following clinical features indicating systemic amyloidosis occurred in succession: orthostatic hypotension, diarrhea or constipation, carpal tunnel syndrome, and weight loss. In 1993, sudden death due to cardiac arrest occurred. The patient survived 6 years after the diagnosis of renal amyloidosis. A combined use of melphalan, prednisolone, colchicine, and dimethyl sulfoxide (DMSO) might have contributed to the long survival of the patient. At postmortem examination, systemic deposition of type AL amyloid was confirmed. In patients with pleural effusion and multiorgan involvement or monoclonal gammopathy, a closed pleural biopsy should be performed, especially if the effusion is transudative.

Amyloidosis↗
PubMed1997

[Gastrointestinal amyloidosis. Differential diagnosis or a complication of inflammatory bowel disease?].

A 77 year-old man developed intermittent diarrhoea and malabsorption. Endoscopic findings and preliminary histological examination indicated ulcerative colitis. Special staining of biopsies from the duodenum and colon revealed amyloid deposits. Classification of the amyloid fibril protein verified AL-amyloidosis, and the diagnosis primary idiopathic amyloidosis was made. Amyloid deposit in the gastrointestinal tract are a common feature of primary and secondary amyloidosis. The symptoms and findings are nonspecific and resemble those of chronic inflammatory bowel disease and ischemic colitis. Secondary amyloidosis can be seen as a rare complication of Crohn's disease and ulcerative colitis. Special staining is necessary to show amyloid deposit, and the distinction between primary and secondary amyloidosis requires immunohistochemistry.

Aged↗
PubMed1998

Scintigraphic functional hyposplenism in amyloidosis.

UNLABELLED: Functional asplenia or hyposplenism may predispose patients to spontaneous splenic rupture and potentially increase the risk of serious infection. In addition, hyposplenism may be a marker of more extensive systemic amyloidosis and has been correlated to a reduction in survival. Decreased splenic function is generally diagnosed by the presence of abnormal red blood cell morphology and decreased splenic uptake on 99mTc-sulfur colloid or microlite scans. We compared liver spleen scans with red cell morphology and anatomic imaging results in all patients with biopsy-proven amyloidosis who presented to the nuclear medicine department over a 12-yr period. Patients were referred from a center for amyloid disease for work-up of suspected hepatic involvement. METHODS: Between 1983 and 1995, 23 liver spleen scans from 21 patients (one patient had three scans) with known amyloidosis were referred for the assessment of degree of hepatic involvement with amyloid. All scans were retrospectively reviewed, and the degree of splenic uptake was graded. Medical records were reviewed for evidence of splenomegaly on physical exam. Extent of splenic involvement also was assessed by anatomical imaging (CT or MRI) in 45% of cases. Peripheral smear reports were reviewed for the presence of abnormal red cell morphology consistent with hyposplenism. RESULTS: Splenic activity was moderately or markedly reduced in 22 of 23 liver spleen scans (21 patients). Eight of these scans had correlative anatomic splenic imaging: four were abnormal and four were normal. Forty-one percent of available peripheral smears contained abnormal red cell morphology. Nine patients had palpable splenomegaly at the time of the liver spleen scan. Splenic pathologic studies were available for three patients (two autopsy, one surgical) and demonstrated diffuse splenic infiltration with amyloidosis. One patient had spontaneous splenic rupture. Fourteen patients died, four of overwhelming infection. CONCLUSION: Reduced splenic uptake on liver spleen scans for patients with suspected hepatic infiltration with amyloid is a common finding. Liver spleen scanning appears to be a more sensitive marker of splenic amyloidosis than clinical parameters or anatomical imaging.

Adult↗
PubMed1998

Transplantation in renal amyloidosis.

In this report, the results of renal transplantation in patients with renal amyloidosis were retrospectively analysed and compared with the control group. Fifteen (3.04%) of the 493 renal transplant recipients whom were followed up in Istanbul School of Medicine transplant outpatient clinic, between 1983 and 1997, were included in the study. The etiology of amyloidosis was familial Mediterranean fever in all patients. The mean follow-up period was 38.3 +/- 31.8 (range 7-65) months. Twelve of the patients were male and 3 female with the mean age 34.13 +/- 10.87 (range 21-60) years. Seven patients had living related, 4 living-unrelated and 4 cadaveric donors. Five patients were lost because of different complications: Three patients died from cardiac amyloidosis all with well functioning grafts, 2, 3 and 36 months after the operation. Sepsis and cardiovascular failure was the probable cause of death in 1 patient who also had chronic rejection. Another one patient with chronic rejection died from hepatic failure. Acute rejection developed in 2 patients. Renal functions of these patients improved by anti-rejection therapies. Chronic rejection developed in 3 patients. In the control group, acute rejection and chronic rejection were diagnosed in 5 and 1 patients, retrospectively. While 1 patients returned to hemodialysis in control group, the others are alive with satisfactory graft function. There was no death in control group. The 5-yr graft and patient survival rates in amyloidosis and the control groups were 75, 77, 95 and 100%, respectively. It was concluded that although transplantation is not a contraindication for the treatment of end stage renal failure in patients with renal amyloidosis, it carries high risk of cardiac complications in the postoperative period. Detailed preoperative cardiovascular evaluations are mandatory in these patients and this intervention should improve the prognosis by excluding the patients who have already been complicated with this problem.

Adult↗
PubMed1998

[Beneficial effect of salazosulfapyridine (SASP) in a patient with secondary renal amyloidosis].

A 55-year old female with rheumatoid arthritis (RA) had presented with proteinuria since July 1996. She was referred to us for persistent edema on her face and legs in November 1996. On admission, her 24-hour urinary protein excretion was 4.4 g/day, total serum protein level was 5.3 g/dl, and serum level of amyloid A protein (SAA) was elevated to 45.8 mg/ml. A percutaneous renal biopsy was performed, and light microscopy revealed varying degrees of amyloid deposits in the mesangial areas and arteriolar walls. The diagnosis of secondary amyloidosis (AA amyloidosis) was based on immunohistochemical staining for amyloid A protein using monoclonal antibody against SAA. Four weeks after treatment with salazosulfapyridine (SASP) and dipyridamole, proteinuria began to decrease and the edema had disappeared. Finally she recovered from nephrotic syndrome. AA amyloidosis has been thought to have a poor prognosis, with progression to renal failure. Since there is no specific effective therapy for the disease, it is very important to reduce the activity of the underlying cause. In our patient with renal amyloidosis following RA, SASP was evidently effective for arthritis and improvement of renal function. SASP might have a beneficial effect on AA amyloidosis by suppressing inflammatory cytokines.

Amyloidosis↗
PubMed1998

Fibrilization in mouse senile amyloidosis is fibril conformation-dependent.

Amyloidosis refers to a group of diseases characterized by tissue deposition of amyloid fibrils. A single intravenous injection of a very small amount of the native mouse senile amyloid fibrils (AApoAII) induced severe systemic amyloid deposition in young mice having the amyloidogenic apoA-II gene (Apoa2c). After AApoAII injection, amyloid deposition occurred rapidly and advanced in an accelerated manner, as observed in spontaneous senile amyloidosis in mice. However, the injection of denatured AApoAII, native apoA-II in high-density lipoprotein (HDL), and denatured apoA-II monomer, which have the same primary structure but without a fibril conformation, did not induce amyloidosis. No amyloid deposition was observed in mice having an amyloid-resistant apoA-II gene (Apoa2b) even 3 months after AApoAII injection. Significantly less amyloid deposition was observed in mice having both types of apoA-II genes heterozygously (Apoa2b/c). These findings suggest that the nucleation-dependent polymerization found in vitro also occurs in vivo, and that the fibril conformation is required for the injected amyloid fibrils to act as seeds in vivo. Fibril conformation-dependent fibrillization is proposed as a general model of the pathogenesis of various kinds of amyloidosis occurring in vivo; it may be useful in both elucidating the pathogenesis of amyloidosis and developing effective therapeutic modalities to treat this disease.

Aging↗
PubMed2002

Iodine-123 metaiodobenzylguanidine scintigraphic analysis of myocardial sympathetic innervation in patients with AL (primary) amyloidosis.

BACKGROUND: Although a high incidence of myocardial adrenergic denervation has been reported in patients with familial amyloid polyneuropathy, assessment of cardiac sympathetic nerve function has not been available in patients with AL (primary) amyloidosis. METHODS: To test the hypothesis that myocardial sympathetic nerve innervation might be impaired and variable according to the presence or absence of clinical autonomic abnormalities and congestive heart failure in AL amyloidosis, we examined 25 patients by use of iodine-123 metaiodobenzylguanidine (MIBG) scintigraphy. RESULTS: Ten of the 16 patients without autonomic symptoms and 5 of the 9 patients with autonomic neuropathy showed congestive heart failure. The heart/mediastinal activity (H/M) ratio (1.53 +/- 0.06 vs 1.29 +/- 0.05 at 3 hours, P <.001) and myocardial washout ratio (41.5% +/- 4.8% vs 30.8% +/- 4.0%, P <.001) of MIBG were significantly increased in patients without autonomic symptoms compared with patients showing autonomic neuropathy. In patient groups with and without autonomic dysfunction, patients demonstrating congestive heart failure exhibited a significantly decreased H/M ratio and increased washout compared with patients with no heart failure, and left ventricular fractional shortening was positively correlated with the H/M ratio and inversely correlated with the washout ratio. There were significant correlations between the low-frequency component of the heart rate variability and the H/M ratio and washout ratio in the entire patient population. CONCLUSIONS: Patients with AL amyloidosis and no autonomic dysfunction showed variable degrees of enhanced cardiac adrenergic neuronal activity with presynaptic sympathetic dysfunction. In contrast, patients with AL amyloidosis and autonomic neuropathy exhibited prominent myocardial adrenergic denervation with normal or impaired sympathetic neural function of the heart. This study demonstrates that myocardial uptake and turnover of MIBG in patients with AL amyloidosis are heterogeneous and dependent on the presence or absence of congestive heart failure and cardiac autonomic dysfunction.

3-Iodobenzylguanidine↗
PubMed2001

Spontaneous abdominal hemorrhage with AA-amyloidosis and vasculitis in a patient with rheumatoid arthritis.

Both rheumatoid vasculitis and amyloidosis in rheumatoid arthritis (RA) are uncommon. We describe a patient in whom they occurred together and were associated with fatal intra-abdominal hemorrhage. A 56-year-old Caucasian woman was referred because of increasing lethargy, edema, and proteinuria. She had suffered from seropositive, erosive, nodular RA for 14 years. Previously, she had undergone numerous joint replacements, a thyroidectomy for amyloid-associated (AA) amyloidosis of the thyroid that caused a large goiter and a renal biopsy that showed renal AA-amyloidosis in the context of nephrotic syndrome. As her condition deteriorated, this patient became increasingly reluctant to go to the hospital and to take drugs beyond analgesics. Thus, her RA was chronically under treated. While in the hospital for evaluation, this patient suddenly developed hypotension, tachycardia, and a severe colicky left-sided abdominal pain radiating from the left upper quadrant/epigastric region to the left iliac fossa. Computed tomography (CT) showed a large amount of echogenic free fluid within the abdomen and marked thickening of the omentum. At laparotomy, 2 liters of free blood was found adjacent to a hematoma of the greater omentum, and it was evacuated without identification of a discrete bleeding point. All solid and hollow organs were normal. The omentum was noted to be very friable. She developed a more disseminated bleeding diathesis and persistent peritoneal hemorrhage via her abdominal drains. She succumbed shortly afterward. Histology revealed extensive omental hemorrhage and one large vessel within the area of hemorrhage showed a severe necrotizing vasculitis. Extensive amyloid deposition was also found within the walls of the smaller omental arterioles. Vasculitis in the context of RA is relatively rare and is associated with under treated, seropositive disease. Skin and nerve involvement are most common, but bowel involvement has been reported, with a highly significant morbidity (partly due to late presentation/recognition). Similarly, AA-amyloidosis is a rare but feared long-term concomitant of under treated RA. Early recognition can permit successful anti-inflammatory therapy to affect a clinical and pathological remission; continued inflammatory stimulation is associated with rapid progression and demise. Chronically under treated patients with RA are more prone to rare but potentially devastating complications. Gastrointestinal catastrophes are a feature of both rheumatoid vasculitis and of amyloidosis, here uniquely co-localized.

Journal Article↗
PubMed2005

Incidence and risk factors of venous thromboembolism (VTD) in patients with amyloidosis.

BACKGROUND: Coagulation problems in amyloidosis are historically associated with bleeding tendencies (mostly Factor X abnormalities). Increased clotting was observed in isolated cases diagnosed with low-grade disseminated intravascular coagulation (DIC). Problem of venous thromboembolic disaease (VTD) in amyloidosis was not systematically investigated. METHODS: We evaluated frequency of VTD and risk factors for VTD in 56 consecutive amyloidosis patients with a documented disease evaluated and followed up at our Center from 1991-2001. Data was collected in 5 categories: (a) demographics, (b) disease and treatment, (c) thrombosis case information, (d) major risk factors for thrombosis and (e) baseline laboratory data. Univariable correlates of VTD were assessed using Kaplan-Meier analysis and Cox proportional hazards analysis. RESULTS: Mean age of the patients was 67 (years range 21-83). Male/female percentage ratio was 70/30. 29% of the patients had high creatinine level (> 1.4 mg/dl). Personal or family history of VTD was recorded in 2 and 0% of patients, respectively. Known hypercoagulable state was present in 1 patient (2%). 8% of patients were smokers. Of 56 patients, 6 developed VTD (11%). Median time from diagnosis of amyloidosis to VTD was 12.5 month (range 1-107). Treatment was given within a median of 1 month (range 0-4) from the development of thrombosis. Only sites of VTD were lower extremities. No cases were associated with I.V. line. 1 case (17%) was identified postoperatively. We identified several univariable correlates of VTD in amyloid patients, including greater age at diagnosis (HR-2.99, P = .041), personal history of DVT (HR-47.7, P = .006) and immobility (HR-11.78, P = .006). Presence of circulating serum M-protein had protective role in our analysis (HR-.08, P = .031). There was no correlation with the type of treatment patients were receiving. CONCLUSION: Risk for thromboembolic diseases in patients with amyloidosis is similar to one previously described for multiple myeloma. Additional studies with higher number of thromboembolic events could help to further elucidate risk factors for VTD in this population of patients.

Journal Article↗
PubMed2006

Diagnostic performance of 123I-labeled serum amyloid P component scintigraphy in patients with amyloidosis.

PURPOSE: To assess the diagnostic accuracy and additional information provided by 123I-labeled serum amyloid P component (SAP) scintigraphy in patients with systemic and localized amyloidosis. SUBJECTS AND METHODS: 123I-labeled human SAP was injected intravenously into 20 controls and 189 consecutive patients with histologically proven amyloidosis: of AA type in 60 cases, AL type in 80, hereditary ATTR type in 27, and localized amyloidosis in 22 cases. SAP scintigrams were obtained 24 hours after tracer injection and were analyzed for abnormal patterns of uptake. Sensitivity and specificity were determined, and scintigraphic findings were compared with clinical data. RESULTS: Diagnostic sensitivity of SAP scintigraphy for systemic AA, AL, and ATTR amyloidosis was 90%, 90%, and 48% respectively, and specificity was 93%. The distribution of amyloid was less diverse in AA than in AL type. Myocardial uptake was not visualized in any patient. Splenic amyloid was very frequent (80%) in AA and AL type but rarely detected clinically (14%). Abnormal tracer uptake in the liver and kidneys correlated with disturbed liver function and proteinuria, respectively. Bone marrow uptake was specific for AL (21%) and was more frequent in AL kappa than AL lambda. Localized amyloid deposits were not imaged. CONCLUSION: SAP scintigraphy is diagnostic of amyloid in most patients with AA and AL type but fewer with hereditary ATTR type, relating to differing distributions and burdens of amyloid in these disorders. It usually reveals more widespread organ involvement than is identified clinically, and certain distributions of amyloid are characteristic of particular fibril types.

Adult↗
PubMed1984

Hepatic amyloidosis. An unusual cause of ascites and portal hypertension.

I treated a patient who had amyloidosis with predominantly hepatic involvement and portal hypertension. The main clinical features were hepatomegaly, gross ascites, proteinuria, and elevated alkaline phosphatase levels. Despite permanganate-sensitive AA protein being present in the biopsy specimen, none of the recognized disease entities associated with secondary amyloidosis were found. A review of the literature and the mechanism of portal hypertension in amyloidosis is given. It is suggested that elevated portal pressures may be of greater importance in the pathogenesis of ascites in amyloidosis than has been appreciated.

Aged↗
PubMed2006

Amyloidosis and the heart: a comprehensive review.

Infiltration of the heart from insoluble protein deposits in amyloidosis often results in restrictive cardiomyopathy that manifests late in its course with heart failure and conduction abnormalities. While the rare primary amyloidosis-related heart disease has been well characterized, senile amyloidosis occurring in the seventh decade of life most frequently affects the heart. Early diagnosis of cardiac amyloidosis may improve outcomes but requires heightened suspicion and a systematic clinical approach to evaluation. Demonstration of tissue infiltration of biopsy specimens using special stains, followed by immunohistochemical studies and genetic testing, is essential in defining the specific protein involved. The therapeutic strategy depends on the characterization of the type of amyloid protein and extent of disease and may include chemotherapy, stem cell transplantation, and liver transplantation. Heart transplantation is controversial and is generally performed only at isolated centers.

Amyloidosis↗
PubMed1995

Multifocal amyloidosis of the pediatric airway.

Amyloidosis of the upper aerodigestive tract is rare in the pediatric age group. We present the first reported case (to our knowledge) of multifocal primary upper airway amyloidosis in a child. An otherwise-healthy 15-year-old girl presented with hoarseness, nasal congestion, and odynophagia. Diagnostic evaluation included flexible nasopharyngoscopy, rigid nasal endoscopy, direct laryngoscopy, bronchoscopy, and biopsy. The results of Congo red staining of the specimen were characteristic of amyloid. The findings of an extensive immunologic and systemic evaluation were normal. We discuss the treatment and postoperative course of this patient, as well as the clinical and pathologic characteristics of amyloidosis, with particular reference to the otolaryngologic manifestations of amyloidosis.

Adolescent↗
PubMed1975

Selective massive amyloidosis of small intestine.

A patient with intestinal amyloidosis with perforation was successfully treated by resection of almost the whole involved small intestine and is recuperating on hyperalimentation therapy. Because there were no signs of amyloiditic deposition in other parts of the body and no other cause of the amyloidosis, the case was considered to be selective localized intestinal amyloidosis. This is the third reported case of perforated amyloiditic intestine and apparently the first case of selective small intestinal amyloidosis involving such a complication.

Amyloidosis↗
PubMed1997

Normalization of plasma factor X levels in amyloidosis after plasma exchange.

Some patients with systemic light chain amyloidosis develop bleeding complications that can be caused by vascular infiltration with amyloid or by alterations of the coagulation or fibrinolytic systems. Factor X deficiency is the most common cause of bleeding manifestations, although deficiencies of other clotting factors, a disruption in the conversion of fibrinogen to fibrin, and circulating heparin-like anticoagulants have also been reported. Deficiency of factor X is a well-recognized cause of bleeding manifestations in patients with light chain amyloidosis. This acquired disorder appears to be secondary to adsorption of factor X to the amyloid fibrils. Previous studies have shown that infusion of plasma into patients with acquired factor X deficiency and amyloidosis induces a transitory improvement of the coagulation tests. However, there is a rapid return to pretransfusion levels. In this manuscript we report the clinical application of plasma exchange in the management of a patient with systemic light chain amyloidosis with acquired factor X deficiency.

Amyloidosis↗
PubMed2003

Spontaneous rupture of the spleen in AL amyloidosis.

The frequency of splenic involvement in AL amyloidosis is not precisely known. However, splenomegaly has been reported in 4-13% of patients. We report four cases of spontaneous splenic rupture in patients with AL amyloidosis. Splenic rupture was the initial manifestation of the disease in one of these patients. The other three experienced splenic rupture during or after high-dose intravenous melphalan with autologous peripheral blood stem cell transplantation (HDM/SCT): one during stem cell mobilization with G-CSF prior to HDM/SCT and two after hematopoietic recovery following treatment. Two of the four patients had Factor X deficiency, the most common coagulation abnormality associated with AL amyloidosis. All four patients underwent splenectomy without significant postoperative complications. Splenic rupture in AL amyloidosis as a complication of aggressive treatment with HDM/SCT has not been reported previously.

Amyloidosis↗