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PubMedDate not supplied

[Pseudoachalasia and secondary amyloidosis in a patient with rheumatoid arthritis].

Rheumatic diseases cover a wide spectrum of clinical syndromes and frequently present with gastrointestinal alterations. Systemic amyloidosis is associated with infectious diseases or chronic inflammatory processes such as rheumatoid arthritis and it can also affect the gastrointestinal tract. Although esophageal involvement is difficult to quantify because its course is frequently asymptomatic, systemic amyloidosis is recognized as a cause of motor disorders of the esophagus. Typical manometric patterns, including achalasia, are usually absent. Esophageal involvement due to amyloid deposits usually corresponds to primary amyloidosis as only a few cases of secondary esophageal deposits (type AA) have been described. We describe a new case of this exceptional association that first presented as dysphagia in a patient with rheumatoid arthritis. The initial suspicion of pseudoachalasia led to the definitive diagnosis of secondary amyloidosis.

Amyloidosis↗
PubMed2000

[Treatments for amyloidosis beyond symptomatic care].

INTRODUCTION: Amyloid syndromes are pathogenetically different, each of the various amyloid diseases requiring specific treatment. Unfortunately, those treatments are often preventive and symptomatic, some efficient therapies being limited to particular types of amyloidosis. CURRENT KNOWLEDGE AND KEY POINTS: Colchicine is effective in the prevention of amyloidosis due to familial Mediterranean fever but is less or not effective in other situations. Cytotoxic agents are useful in the treatment of AL amyloidosis with or without hemopoietic stem cell transplantation. Liver transplantation is indicated for familial polyneuropathy and kidney transplantation for dialysis-related beta 2 microglobulin amyloidosis. FUTURE PROSPECTS AND PROJECTS: In vivo binding of serum amyloid P (SAP) (component shared by all amyloid deposits) to amyloid fibril, is a new avenue in the therapeutic approach. Development of radiolabeled SAP scintigraphy allows assessment of the disease outcome and evaluation of treatment-related effects. The various treatments that were assessed until now with the objective of curing the disease are reviewed.

Amyloidosis↗
PubMed2000

[Diagnostic strategy in amyloidosis].

INTRODUCTION: The diagnosis of amyloidosis is a four-step strategy requiring: 1) a high degree of suspicion, as the clinical presentation of the disease is very polymorphous; 2) the biochemical nature of the disease; 3) the evaluation of the disease spread and 4) how it evolves. EXEGESIS: Simple and noninvasive biopsies usually make it possible to diagnose amyloidosis. Available treatments of generalized amyloidosis require an exact characterization of the nature of amyloid deposits, which is based on the clinical context, immunohistochemical analysis of amyloid deposits, and genetic testing. CONCLUSION: Management of amyloidosis should be improved by better characterization of amyloid deposits. This would result in epidemiological data which are currently lacking.

Amyloid↗
PubMed1986

[Renal and digestive amyloidosis and Behçet's disease. Apropos of a case].

The authors report the case of a 33-year old male Tunisian who, 7 years after the onset of complete Behçet's disease (diagnosed on international criteria) developed a nephrotic syndrome diagnosed as renal amyloidosis on histological evidence. Attenuation of Congo red staining by potassium permanganate pointed to AA type amyloidosis. Despite treatment with colchicine and cyclophosphamide the patient's condition deteriorated. Amyloid deposits were found in a small bowel biopsy performed for persistent diarrhoea. The patient died 3 years after amyloidosis was diagnosed. The authors have reviewed the literature concerning this exceptional association: in all cases where it was typed the amyloidosis was AA. Attention is drawn to the failure of colchicine in this patient.

Adult↗
PubMed1999

[AA amyloidosis complicating sarcoidosis].

INTRODUCTION: Amyloidosis combined with sarcoidosis has been very rarely described. EXEGESIS: We report the case of a 72-year-old man presenting with sarcoidosis and amyloidosis AA. The association of peripheral and retroperitoneal adenopathies accompanied by loss of weight and histopathological results conducted to the diagnosis of sarcoidosis, excluding other causes. Corticosteroid therapy led to a decrease in clinical manifestations and after 2 years, clinical signs of amyloidosis have not progressed. CONCLUSION: According to results previously described in the literature and the description of the present case, we conclude that sarcoidosis can be complicated by amyloidosis AA, the presence of which may justify corticosteroid therapy.

Adrenal Cortex Hormones↗
PubMed1995

Amyloidosis.

Amyloidosis is the generic term for a heterogeneous group of disorders characterised by the common finding of amyloid deposition. The various acquired and hereditary syndromes are classified according to the identity of the respective amyloid fibril sub-unit protein. Systemic amyloidosis and some local forms are progressive diseases that are frequently fatal. The diagnosis of systemic amyloidosis is only occasionally suspected on clinical grounds alone, and is more often considered when an associated disorder such as a chronic inflammatory disease or monoclonal gammopathy is present. No blood test is diagnostic of amyloidosis but routine haematological and biochemical investigations have important roles in defining the underlying metabolic disturbance and evaluating function of affected organs. The diagnosis can only be confirmed by demonstrating the presence of tissue amyloid deposits. Traditionally this required histology but the recent introduction of labelled serum amyloid P component scintigraphy is a specific alternative that provides a quantitative macroscopic whole body survey of amyloid deposits. No treatment specifically causes the resolution of amyloid but therapy which reduces the supply of amyloid fibril precursor proteins can improve survival and preserve organ function. Major regression of amyloid occurs in at least a proportion of such cases suggesting that clinical improvement reflects mobilisation of amyloid.

Amyloidosis↗
PubMed1992

Serum amyloid P component: a predictor of clinical beta 2-microglobulin amyloidosis.

Beta 2-microglobulin (beta 2M) amyloidosis is common in patients on long-term hemodialysis, but the clinical conditions associated with disease activity are poorly understood. This study was designed to determine if the serum amyloid P (AP) component concentration is predictive of beta 2M amyloid disease activity. Serum AP component concentrations were determined by rocket immunoelectrophoresis and beta 2M concentrations by a commercially available kit. Radiographic evidence of beta 2M amyloidosis was determined from bone films of the hips, shoulders, and hands. Serum AP component concentrations were not different in dialysis and control patients. However, AP component concentrations in long-term (greater than or equal to 5 years) dialysis patients were significantly lower than in short-term (less than 5 years) dialysis patients (43.0 +/- 16.9 micrograms/mL [n = 28] v 56.0 +/- 18.3 micrograms/mL [n = 31], P less than 0.05). The patients on hemodialysis for 5 or more years who had radiographic evidence of severe beta 2 M amyloidosis were significantly older (57.9 +/- 9.5 v 38.3 +/- 11.3 years, P less than 0.001) and their serum AP concentrations were significantly lower (34.3 +/- 15.0 v 50.1 +/- 15.6 micrograms/mL, P less than 0.05) than long-term dialysis patients without radiographic evidence of disease. Stepwise regression analysis showed that the patient's age and serum AP component concentration were predictors of radiographic evidence of beta 2 M amyloidosis. Thus, serum AP component concentrations are decreased in long-term dialysis patients, suggesting accelerated deposition into amyloid deposits.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1989

Resolution of nephrotic syndrome and lack of progression of heroin-associated renal amyloidosis.

The case of a patient with nephrotic syndrome and renal insufficiency due to renal amyloidosis secondary to chronic subcutaneous heroin abuse who discontinued her drug habit is reported. During the 6 years following the initial renal biopsy, the patient's nephrotic syndrome remitted: urinary protein decreased from 6.8 g/d to 170 mg/d, serum albumin increased from 2.5 g/dL to 3.9 g/dL, and she was no longer edematous. Serum creatinine remained stable at 1.8 mg/dL and creatinine clearance was also unchanged at 34 mL/min. Repeat renal biopsy showed AA amyloidosis involving the glomeruli, with minimal interstitial inflammation. This appears to be the first case of clinical improvement in heroin-associated renal amyloidosis after cessation of drug injections. As in other cases of AA amyloidosis that have improved, the clinical picture does not correlate with the findings on renal biopsy.

Amyloidosis↗
PubMed1979

Senile pulmonary amyloidosis.

Lung tissue obtained from 340 necropsies of persons aged 80 years and over was investigated for the occurrence of pulmonary amyloid deposits by means of a sensitive fluorescence technique. The incidence of pulmonary amyloidosis was about 2% below the age of 80, nearly 10% in individuals between 80 and 84 years, and more than 20% at and beyond 85 years. Vascular and concomitant alveolar-septal amyloidosis was seen slightly more often than the pure septal form of senile amyloidoisis. The two modes of deposition correspond to two different forms of cardiac amyloidosis, vascular and nodular. On the other hand, the two different modes of pulmonary amyloidosis affect different age groups. It is postulated that the disorder is related to age-linked immunological disturbances and to genetically determined factors.

Aged↗
PubMed1982

Diagnosis and classification of amyloidosis by an immuno-histological method.

Following the recent classification of amyloidosis by the amyloid proteins involved, we decided to approach its tissue diagnosis and chemical classification by an immunohistological method. Post-mortem tissue specimens from various organs of patients with primary and secondary amyloidosis were examined by the immunoperoxidase technique with specific antibodies against proteins AL and AA which respectively characterize primary and secondary amyloidosis. The major advantage of this technique is that it can be applied to formalin fixed, paraffin embedded tissues. The immunoperoxidase technique proved to be extremely sensitive for the detection of amyloid deposits. Moreover, the deposited proteins could be clearly characterized as AL or AA by the specific anti-sera. We therefore believe that the immunooperoxidase technique is extremely useful for the early tissue diagnosis and chemical classification of amyloidosis.

Amyloid↗
PubMed1986

Heart valve involvement in familial amyloidosis with polyneuropathy.

Involvement of the heart is common in the various types of amyloidosis. Little attention has, however, been paid to the presence and significance of amyloid in cardiac valves. We examined the heart valves of twelve autopsy cases with familial amyloidosis with polyneuropathy. All leaflets showed more or less abundant amyloid infiltration with significant valvular aortic stenosis due to degenerative calcification in two cases. In familial amyloidosis with polyneuropathy, amyloid deposits are thus invariably present in the valves, but the valvular function is often preserved. Amyloid may, however, produce hemodynamically significant valvular lesions, and the importance of valvular involvement in the various types of amyloidosis may still be underestimated.

Adult↗
PubMed1999

Atrial thrombosis in cardiac amyloidosis: diagnostic contribution of transesophageal echocardiography.

Few cases of atrial thrombosis detected by transesophageal echocardiography (TEE) in cardiac amyloidosis have been reported recently. We present the cases of 3 consecutive patients affected by AL-type cardiac amyloidosis, symptomatic for heart failure and in sinus rhythm. All patients had a cardiac restrictive pattern at Doppler examination. TEE showed left atrial thrombus in 2 patients and biatrial thrombi in 1 patient; conventional transthoracic echocardiography detected only 1 left atrial thrombus. Our experience confirms the association between cardiac amyloidosis and atrial thrombosis, even in sinus rhythm. TEE should be considered to assess thromboembolic risk in all cases of cardiac amyloidosis with severe diastolic dysfunction.

Aged↗
PubMed1999

Anesthetic management of a patient with laryngeal amyloidosis.

A 73-year-old woman who suffered from progressive hoarseness for 6 years and dysphagia without pain for 1 year presented with a soft tissue deposition on the posterior region of the vocal cords and narrowing in the subglottic area. Biopsy of this soft tissue and histological examination revealed laryngeal amyloidosis. A tracheostomy and partial removal of the amyloid were performed with general anesthesia. The airway was secured with a smaller diameter endotracheal tube, which was inserted atraumatically with Magill's forceps. The larynx is a rare site for amyloidosis. Laryngeal amyloidosis is fragile and hemorrhagic. Therefore, massive bleeding may occur during intubation. Anesthetists should take care in intubating the tracheas of these patients and be aware of other systemic diseases in laryngeal amyloidosis.

Aged↗
PubMed1999

[Decreased Quick percentage, acquired factor X deficiency, hemarthrosis and ecchymosis: amyloidosis].

A 68-year-old woman suffered from spontaneous hemarthrosis of the right elbow joint in april 1994. Cutaneous ekchymoses had been noted since summer 1993. Prothrombin time was prolonged (Quick percentage 40-50%) and was not corrected by prolonged administration of vitamin K. Coagulation studies showed isolated factor X (FX) deficiency without circulating FX inhibitor. This suggested the diagnosis of systemic amyloidosis which was retrospectively confirmed in small bowel biopsy specimens obtained one year before. Bilateral femoral head necrosis and femoral neck fracture due to amyloidosis, necessitated orthopedic surgery. The patient died four weeks postoperatively after several episodes of bleeding complications. Isolated FX deficiency may be hereditary but should--in the clinical context--also evoke the diagnosis of systemic amyloidosis. The possible bleeding tendency associated with amyloidosis is not attributable to acquired FX deficiency alone, but may also be caused by amyloid deposition in the microvasculature leading to acquired vascular hemorrhagic diathesis.

Aged↗
PubMed2000

Increased density of interstitial mast cells in amyloid A renal amyloidosis.

Renal interstitial fibrosis is the final common pathway leading to end-stage renal disease in various nephropathies including renal amyloidosis. However, the role of mast cells (MCs) in the fibrotic process of renal amyloidosis is not fully understood. We compared the distribution of MCs in renal biopsies from 30 patients with AA type renal amyloidosis and 20 control cases. Immunoreactivity of renal MCs to anti-tryptase and anti-chymase was studied. Interstitial myofibroblasts were stained with anti-alpha-smooth muscle actin (alpha-SMA) antibody, and inflammatory cells were identified by anti-CD45, -CD20, and -CD68 mAbs. Positively stained cells were counted, and the relative interstitial and fractional areas of anti-alpha-SMA stained cells were measured. Anti-CD29 mAb was used to detect beta1 integrin and anti-basic fibroblast growth factor (bFGF) mAb for the growth factor on MCs. MCs were rarely found in control samples. In contrast, samples showing amyloid deposition contained numerous tryptase-positive (MCT) (940.17 +/- 5.4 versus 6.74 +/- 1.1/mm2) but fewer chymase-positive (MCTC) cells (20.7 +/- 2.86 versus 1.7 +/- 0.76/mm2) in the renal interstitium. There was a significant relationship between interstitial MCT and creatinine clearance (r = -0.72), and between interstitial MCT and glomerular amyloid-index (GAI) (r = 0.723) and interstitial amyloid area (r = 0.824). Accumulation of MCs correlated significantly with the number of T lymphocytes (MCT: r = 0.694). There was also a significant relationship between mast cell (MC) number and the fractional area of alpha-SMA positive interstitium (r = 0.733) and interstitial fibrotic area (r = 0.6). Double immunostaining demonstrated intracytoplasmic presence of beta1 integrin on 87% of MCT and correlated significantly with the interstitial amyloid area (r = 0.818, P = .001) and T-cell number (r = 0.639, P = .002). bFGF was also detected on 85.5% of MCTC correlating well with the interstitial alpha-SMA-area (r = 0.789). Our results indicate that MCs constitute an integral part of the overall inflammatory process and play a crucial role in interstitial fibrosis in renal amyloidosis.

Actins↗
PubMed2006

Drug Insight: emerging therapies for amyloidosis.

Amyloidosis is a clinical disorder caused by extracellular deposition of proteins that are normally soluble as insoluble, abnormal fibrils that impair organ function. More than 20 unrelated proteins can form amyloid fibrils in vivo. All fibrils share cross-beta core structure and pathognomonic red-green birefringence when viewed under cross-polarized light after staining with Congo red. Amyloidosis can be acquired or hereditary, localized or systemic, and is classified according to the fibril precursor protein. Local amyloid deposition occurs in the brain in Alzheimer's disease and in the pancreas in maturity-onset diabetes, but a direct role in the pathogenesis of these diseases remains unproven. Systemic amyloidosis, with amyloid deposits in the viscera, blood vessel walls and connective tissues, is usually fatal and is the cause of about one death per thousand in developed countries. Recent elucidation of fundamental aspects of the pathogenesis of amyloidosis, and developments in diagnosis and monitoring of this disorder have greatly improved outcome for patients. Several exciting novel therapeutic strategies, reviewed in this article, are in development. These include interference with different stages of fibrillogenesis and enhancement of clearance of amyloid deposits.

Alzheimer Disease↗
PubMed2000

Blood stem cell transplantation as therapy for primary systemic amyloidosis (AL).

This study investigated the response rate and toxicity of blood cell transplantation as treatment for primary amyloidosis (AL). Twenty-three patients had stem cells collected between November 1995 and September 1998. Conditioning included melphalan and total body irradiation in 16 and melphalan alone in 4. Three patients did not undergo stem cell infusion because of poor performance status. Two died of progressive amyloid at 1 and 3 months. One patient is alive on hemodialysis. Fourteen males and six females (median age, 57 years) underwent transplantation. Renal, cardiac (by echocardiography), peripheral neuropathy or liver amyloidosis occurred in 14, 12, 3, and 1, respectively. Echocardiography demonstrated an interventricular septal thickness > or = 15 mm in six patients, five of whom died post transplantation. Three patients died of progressive amyloidosis at 7, 7, and 21 months. Thirteen patients are alive with a follow-up of 3 to 26 months. Twelve (60%) fulfilled the criteria of a hematologic or organ response. Severe gastrointestinal tract toxicity was seen in five (25%). We conclude that blood cell transplantation for amyloidosis had a much higher morbidity and mortality compared with transplantation for myeloma. The best results appear to occur in patients with nephrotic syndrome as the only manifestation of their disease.

Adult↗
PubMed2004

A multicenter phase 2 trial of stem cell transplantation for immunoglobulin light-chain amyloidosis (E4A97): an Eastern Cooperative Oncology Group Study.

Stem cell transplantation was introduced as a new therapeutic modality for amyloidosis. The purpose of the current study was to determine the feasibility and toxicity of stem cell transplantation for amyloidosis in a cooperative group setting in which most participating institutions would have limited experience in managing the disorder. A total of 30 patients with biopsy-proven amyloidosis shown to be immunoglobulin light-chain type were enrolled on this trial. The protocol required mobilization of a minimum of 6 x 10(8) mononuclear cells/kg or 5 x 10(6) CD34(+) cells/kg ideal body weight. These targets had to be achieved within seven collections. Patients with advanced hepatic, renal, or cardiac failure were excluded. End points included objective response rate and overall survival. The secondary end point of the protocol was nonhematologic toxicity. Accrual to the study was faster than expected. The overall response rate (hematologic and organ) was 64%, with three treatment-related deaths. Another patient died before day 30 of sudden cardiac death not treatment related. The median follow-up of surviving patients is 30.3 months. Median survival has not been reached. Stem cell transplantation for selected patients with amyloidosis is feasible in a cooperative group setting. A multicenter phase 3 trial of high-dose therapy is indicated.

Adult↗