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PubMed1992

Laryngeal amyloidosis: a clinicopathologic and immunohistochemical review.

The clinical nature of laryngeal amyloidosis has not been well established, and the natural history of the disease remains a controversial matter. To address these issues, we reviewed the clinicopathologic and immunohistochemical features of 22 cases (11 men and 11 women; mean age, 56 years) of laryngeal amyloidosis. Hoarseness was the most common symptom, and the most frequent site affected was the false vocal cords. Six patients had concomitant tracheal amyloidosis. Paraffin blocks were available for immunostaining in 20 cases. Nineteen cases were positive for amyloid P component. lambda-Light chains were detected in 12 cases and kappa-light chains in 5; three cases did not show definite light-chain staining. Ten patients underwent repeated operations for persistent or recurrent respiratory tract disease. One patient died of progressive tracheobronchial amyloidosis, but systemic amyloidosis did not develop in any of the patients. Laryngeal amyloidosis is a form of localized amyloidosis characterized by monoclonal light-chain deposition. Recurrent respiratory tract disease is not uncommon, but the usual clinical course is relatively benign.

Adult↗
PubMed1975

Amyloidosis induced in hamsters by a filarid parasite (Dipetalonema viteae).

Amyloidosis was induced in hmasters infected with the filarial nematode parasite, Dipetalonema viteae. The incidence of amyloidosis was 64% in a group inoculated with 150 larvae and 54% in the group receiving 150 larvae in each of two inoculations. Amyloidosis was not seen in control animals. Microfilariae probably served as the antigenic stimulus in the pathogenesis of amyloidosis, since those animals in which amyloidosis was formed had microfilaremias that were significantly greater (P less than 0.05) both in number and duration than those in infected animals that did not develop amyloidosis. Other reports of amyloidosis associated with filariasis are reviewed.

Amyloidosis↗
PubMed1997

Systemic AA amyloidosis in captive cheetahs (Acinonyx jubatus).

Ongoing disease surveillance of necropsied captive cheetahs (Acinonyx jubatus) (n = 141) revealed a high prevalence of renal amyloidosis (n = 54 [38%]; age 1 to 16 years). The prevalence increased from 20% in pre- 1990 necropsies to 70% of cheetahs necropsied in 1995. In 74% of the cheetahs with amyloidosis, renal failure was determined to be the sole or partial cause of death. Papillary necrosis was seen only in affected cheetahs and involved 25% of these animals. Amyloid was present predominantly in the medullary interstitium, with minimal glomerular involvement. The amyloid deposits were immunohistochemically identified as AA type using antisera to both human and canine protein AA. A high percentage (52%) of animals with renal amyloid also had subsinusoidal hepatic AA amyloid deposits. Inflammatory diseases were identified in 100% of affected cheetahs. The most common inflammatory disease was chronic lymphoplasmacytic gastritis. The prevalence and severity of gastritis was higher in cheetahs with amyloidosis, and the prevalence of severe gastritis increased from 16% to 43%, coinciding with the increase in prevalence of amyloidosis. These findings suggest that cheetahs have a high prevalence of systemic amyloidosis in response to inflammation and that renal amyloidosis is an increasingly significant cause of morbidity and mortality in captive cheetah populations. Factors of potential importance in the apparent high prevalence of AA amyloidosis in cheetahs are currently being investigated in our laboratories.

Acinonyx↗
PubMed1986

Amyloidosis of the lower respiratory tract. Clinical and pathologic features in a series of 21 patients.

Twenty one cases of amyloidosis of the lower respiratory tract were seen at a single center. In three patients, multifocal bronchial amyloid plaques led to stenosis and atelectasis, and in two, small pseudotumor masses were an incidental bronchoscopic finding. Two patients had nodular parenchymal amyloidosis, in one of whom the lesions were progressive and in the other static. Fifteen patients had diffuse parenchymal amyloidosis. Two of these had severe interstitial involvement and died in respiratory failure; eight had congestive cardiac failure, and parenchymal amyloidosis was a post-mortem finding; two had senile cardiorespiratory amyloidosis, also found at autopsy; and in three, the amyloidosis was associated with malignancy. The degree of respiratory embarrassment seemed to be related to the amount of amyloid in the gas diffusion zones, irrespective of the etiology of amyloidosis.

Adult↗
PubMed2006

Inflammation-responsive transcription factor SAF-1 activity is linked to the development of amyloid A amyloidosis.

Abundantly expressed serum amyloid A (SAA) protein under chronic inflammatory conditions gives rise to insoluble aggregates of SAA derivatives in multiple organs resulting in reactive amyloid A (AA) amyloidosis, a consequence of rheumatoid arthritis, Crohn's disease, ankylosing spondylitis, familial Mediterranean fever, and Castleman's disease. An inflammation-responsive transcription factor, SAF (for SAA activating factor), has been implicated in the sustained expression of amyloidogenic SAA under chronic inflammatory conditions. However, its role in the pathogenesis of AA amyloidosis has thus far remained obscure. In this paper we have shown that SAF-1, a major member of the SAF family, is abundantly present in human AA amyloidosis patients. To assess whether SAF-1 is directly linked to the pathogenesis of AA amyloidosis, we have developed a SAF-1 transgenic mouse model. SAF-1-overexpressing mice spontaneously developed AA amyloidosis at the age of 14 mo or older. Immunohistochemical analysis confirmed the nature of the amyloid deposits as an AA type derived from amyloidogenic SAA1. Furthermore, SAF-1 transgenic mice rapidly developed severe AA amyloidosis in response to azocasein injection, indicating increased susceptibility to inflammation. Also, during inflammation SAF-1 transgenic mice exhibited a prolonged acute phase response, leading to an extended period of SAA synthesis. Together, these results provide direct evidence that SAF-1 plays a key role in the development of AA amyloidosis, a consequence of chronic inflammation.

Amyloidosis↗
PubMed1996

Resolution of heart failure in patients with AL amyloidosis.

BACKGROUND: Patients with AL amyloidosis and congestive heart failure have a very poor prognosis. To date, the recovery of these patients has not been described in detail. OBJECTIVE: To determine the frequency and characteristics regression of disease in patients with congestive heart failure due to AL amyloidosis. DESIGN: Review of patients with systemic AL amyloidosis. SETTING: An international referral center for amyloidosis in the United States. PATIENTS: 140 patients with congestive heart failure due to Al amyloidosis who were seen between 1983 and 1994. MEASUREMENTS: Functional status, Doppler echocardiography, and objective measurements of disease activity. RESULTS: 3 of 140 patients (2.1%) had marked resolution of congestive heart failure and evidence for remission of disease activity. All 3 had been treated with melphalan. CONCLUSIONS: Melphalan appears to have had a favorable effect in 3 patients with AL amyloidosis and heart failure. The abolition of light chains that was seen in these 3 patients suggests that light-chain toxicity may play a role in the genesis of heart failure in patients with AL amyloidosis.

Amyloidosis↗
PubMed1999

Systemic amyloidosis presenting with angina pectoris.

BACKGROUND: Manifestations of cardiac amyloidosis may include congestive heart failure and sudden cardiac death. Although vascular involvement in patients with amyloidosis is common, systemic amyloidosis presenting with angina is rare. OBJECTIVES: To report on patients with systemic amyloidosis presenting with angina pectoris. DESIGN: Case series. SETTING: Academic medical center. PATIENTS: Five patients who presented with angina pectoris and normal coronary angiogram as the initial manifestation of systemic amyloidosis. MEASUREMENTS: Endothelial-dependent and endothelial-independent coronary flow reserve. RESULTS: All patients had coronary flow reserve abnormalities and subsequently developed congestive heart failure and systemic manifestations of amyloidosis. Histologic evaluation revealed amyloid deposition in the intramyocardial coronary vessels. CONCLUSIONS: Cardiac amyloidosis can present as angina pectoris associated with coronary flow reserve abnormalities despite normal coronary angiograms. This finding may have major therapeutic and prognostic implications in this patient population.

Aged↗
PubMed1999

[Systemic amyloidosis as a cause of so-called atherosclerotic hemorrhagic complications].

BACKGROUND: A survey of the pathogenesis, diagnostics and treatment of amyloidosis is presented, illustrated by long-term clinical experience with the treatment of 28 patients with primary amyloidosis, 4 patients with familial and 7 patients with secondary amyloidosis. METHODS AND RESULTS: To all patients combined treatment of glucocorticosteroids and cytostatics was administered. For primary amyloidosis methylprednisolone, vincristine (oncovine), cyclosphosphamide, alkeran, chlorethylnitrosourea (MOCCA), and in secondary amyloidosis cyclophosphamide, vincristine, prednisone (CVP). In all patients, this treatment was combined with dimethylsulphoxide treatment (DMSO) administered in intravenous infusion (5 g/100 ml saline) for 3-5 days before the start of cytostatic therapy. In primary amyloidosis the median of survival was 48 months, in secondary amyloidosis the median survival was 72 months. CONCLUSIONS: The authors emphasise the need of an early diagnosis and start of comprehensive therapy completed in recent years successfully with immunomodulatory therapy using hydrolytic enzymes (WOBE MUGOS).

Adult↗
PubMed2000

[Localized amyloidosis of the ureter and bladder treated effectively by occlusive dressing technique therapy using dimethyl sulfoxide: a case report].

We report here the first case of localized amyloidosis of the ureter and bladder to be treated effectively by occlusive dressing technique therapy using dimethyl sulfoxide. The patient was a 48-year-old woman whose chief complaint was macrohematuria and right back pain. Ultrasound sonography demonstrated right hydronephrosis and an intravesical mass in the region of the right ureteral orifice. Retrograde pyelography revealed severe stricture of the right lower ureter. Cystoscopy demonstrated a yellow submucosal tumor around the right ureteral orifice. We suspected urinary tract amyloidosis, and transurethral biopsy and resection of the intravesical mass were performed under right ureteral stenting. Histopathological diagnosis was amyloidosis. There was no evidence of systemic amyloidosis. To treat residual amyloidosis of the ureter and bladder, we performed occlusive dressing technique therapy using dimethyl sulfoxide every day. After 6 months of therapy, the right hydronephrosis disappeared, and there was no evidence of a recurrence of amyloidosis. We concluded that this therapy was very effective and safe for urinary tract amyloidosis.

Administration, Cutaneous↗
PubMed2001

Clinical and histopathological characteristics of primary cutaneous amyloidosis in 794 Chinese patients.

BACKGROUND: Primary cutaneous amyloidosis (PCA) is not uncommon in Chinese patients. The disease is usually persistent and quite pruritic. Patients who suffer from this disease usually respond poorly to conventional treatment. We thus reviewed our cases of PCA to discuss the clinical and pathological characteristics. METHODS: Seven hundred and ninety-four Chinese patients with PCA who visited the Department of Dermatology, Taipei Veterans General Hospital during the last 26-year period were examined and retrospectively studied. The diagnosis in these patients was confirmed by histopathological studies. RESULTS: Among the many types of PCA, lichen amyloidosis was the most common clinical variant (67%). Pure cases of macular amyloidosis accounted only 8% and were often associated with lichenoid lesions to form biphasic amyloidosis, which was composed of 25% in our series. Other rare types of PCA, such as nodular, anosacral, and vitiliginous amyloidosis, always required a careful differential diagnosis clinically from other similar skin disorders. In addition, 56 familial cases were found. Histopathologically, the most common epidermal findings of PCA were hyperkeratosis, irregular acanthosis with thinning of rete ridges, and expansion of dermal papillae by amyloid deposition. Special histochemical stains were helpful for confirming the existence of amyloid. CONCLUSIONS: Our study represents the largest number of cases of PCA collected to date. Based on the data, most cases are sporadic, except 56 familial cases which may suggest the possible genetic role. Rare types of PCA, such as anosacral and vitiliginous amyloidosis which need special attention, compose a diagnostic challenge to a dermatologist. Histochemically, H&E stain can give a primary clue for the diagnosis of amyloidosis and crystal violet stain is a very simple and sensitive method to detect the existence of amyloid.

Adult↗
PubMed1976

Inhibition of senile amyloidosis of mice by biscarboxyethyl germanium sesqui-oxide.

A mouse strain, ICR/SLC, was involved in spontaneous amyloidosis with high incidence. The amyloid deposition in this strain was seen mainly in the mucosal propria of duodenum and terminal ileum, liver, spleen, adrenal cortices, and renal glomeruli. The mice, orally administered more than 300 mg/kg of organic germanium for 22 months since 5 weeks old, did not develop amyloidosis. Half of the mice, given 30 mg/kg of organic germanium for 22 months developed amyloidosis. The mice given 5% carboxymethylcellulose, the solvent of organic germanium, were affected with systemic amyloidosis with high frequency. The results showed that the organic germanium successfully inhibited the occurrence of senile amyloidosis with dose response. The agent did not have any apparent relation to the incidence of hepatic cell carcinoma or pulmonary adenoma which is frequently combined with aged mice. Although the actual mechanism involved is not clear, the evidence of the inhibition of senile amyloidosis by organic germanium may give a light to elucidate the pathogenesis of amyloidosis.

Amyloidosis↗
PubMedDate not supplied

Hereditary systemic amyloidosis with renal involvement.

Hereditary systemic amyloidosis is caused by deposition of genetically variant proteins as amyloid fibrils. The types that present with renal disease are usually associated with mutations in the genes for either apolipoprotein AI, apolipoprotein AII, lysozyme or fibrinogen A alpha-chain. These diseases are inherited in an autosomal dominant manner with variable penetrance, and can present clinically at any time from the teen years to old age, though usually in mid-adult life. Hereditary amyloidosis is uncommon, but its precise characterization has major implications for patient management and genetic counseling, and it has been an extremely valuable model for elucidating the pathogenesis of amyloid deposition generally. The amyloidogenic variant proteins associated with hereditary amyloidosis are less stable than their normal wild type counterparts and even under physiological conditions can populate partly unfolded states, involving loss of tertiary or higher order structure, which readily aggregate with retention of beta-sheet secondary structure into protofilaments and fibrils. The clinical phenotype of hereditary renal amyloid is non-specific and is readily misdiagnosed as acquired AL amyloidosis. Indeed, we have lately demonstrated that five percent of patients with apparent sporadic amyloid have hereditary fibrinogen A alpha-chain amyloidosis associated with the valine 526 variant. Penetrance of this particular mutation is extremely low in most families obscuring the genetic etiology, but the renal histology is very characteristic showing substantial accumulation of amyloid within enlarged glomeruli, but none in blood vessels or the interstitium. DNA analysis is now performed routinely in UK National Amyloidosis Centre in patients with systemic amyloidosis in whom AA or AL fibril type cannot be definitively verified.

Amyloidosis↗
PubMed2002

[Analysis of clinical manifestations of pharyngeal and laryngeal amyloidosis].

OBJECTIVE: To study the clinical manifestations of pharyngeal and laryngeal amyloidosis. METHODS: Retrospective analysis of the clinical data of the inpatients with systemic and local amyloidosis. RESULTS: Of the 25 cases, sex ratio was 21:4, and average age was 48 years. Seventeen cases were systemic, of which 7 patients died in 2 years since diagnoses were made. One case was local bronchial amyloidosis. Seven cases were local primary pharyngeal and laryngeal amyloidosis and no affirmative death report was recorded since diagnoses were made. CONCLUSIONS: Amyloidosis occurs more likely in male and middle- and old-aged patients. Pharyngeal and laryngeal local primary amyloidosis have relatively better prognosis than that of systemic amyloidosis. Follow-up is important because local recurrence may be happened.

Adolescent↗
PubMed2003

[Amyloidosis complicating psoriatic arthritis].

BACKGROUND: Secondary AA amyloidosis is a classical complication of rheumatismal or chronic infectious diseases. Psoriasis is a rare cause of secondary amyloidosis with only around thirty cases reported in the literature. CASE REPORT: A 62 year-old man exhibited cutaneous lesions of psoriasis for six years complicated by articular involvement over the past year. The occurrence of an isolated proteinuria revealed renal and hepatic AA amyloidosis. Treatment with methotrexate (Méthrotrexate), enalapril (Renitec) and colchicine (Colchicine) was initiated and led to a stabilization of the proteinuria for two years. DISCUSSION: In psoriatic patients, secondary amyloidosis mainly complicates the arthritic diseases of prolonged progression. Our case report is original in the rapid onset of amyloidosis after the first articular signs. The clinical manifestations of secondary amyloidosis are related to renal or gastrointestinal involvement. Prognosis is usually poor. Treatment of secondary amyloidosis is difficult and relies on systemic treatment. Colchicine may be helpful.

Amyloidosis↗
PubMed1992

Chronic dialysis in patients with systemic amyloidosis: the experience in northern Italy.

The clinical outcome of 61 patients with renal amyloidosis treated with chronic dialysis was reviewed. Eighteen patients, 4 with primary or AL amyloidosis and 14 with reactive or AA amyloidosis, died within one month from starting treatment. The other 43 patients were treated with dialysis for 3 to 199 months and are the object of this study. Sixteen patients had AL amyloidosis and 27 had AA amyloidosis. Thirty-five patients were treated with hemodialysis (HD) for a mean period of 40 +/- 47 months and 8 were treated with continuous ambulatory peritoneal dialysis (CAPD) for 20 +/- 15 months. Patient survival rate at 1 and 5 years was 68% and 30% respectively. There was no difference in survival rate between patients treated with HD and those treated with CAPD, while patients younger than 45 had a better 5-year survival rate. Twenty four (60%) patients achieved a satisfactory rehabilitation with dialysis. At the last follow-up, 15 patients (14 on HD, 1 on CAPD) were alive 61 +/- 58 months after starting dialysis. Twenty-eight patients died after 30 +/- 20 months. The main causes of death were: cardiovascular accident (11), stroke (3), sepsis (5) and cachexia (5). The most important extra-renal complications of amyloidosis were related to cardiovascular involvement (heart failures, arrhythmias, hypotension) and gastrointestinal involvement (malabsorption). Intra-dialytic hypotension in patients on HD and peritonitis in patients on CAPD were the main problems related to dialytic procedure. his study confirms that life expectancy and the quality of life of dialysis patients with systemic amyloidosis are poorer than those of general dialysis population.(ABSTRACT TRUNCATED AT 250 WORDS)

Amyloidosis↗
PubMedDate not supplied

Hodgkin's lymphoma with concurrent systemic amyloidosis, presenting as acute renal failure, following lymphomatoid papulosis.

BACKGROUND: Hodgkin's lymphoma (HL) has been reported to occur infrequently in association with lymphomatoid papulosis (LP). On the other hand, amyloidosis may also occur as a complication of lymphomas including HL but has never been reported in association with LP. We herein present an unusual case of a patient with LP who 3 years later developed concomitant HL, as well as kidney and brain amyloidosis, leading to nephrotic syndrome, acute renal failure, coma and death within 3 weeks. In our patient the simultaneous development of amyloidosis and HL suggests that amyloidosis is secondary to LP, an association never reported before. METHODS: A 74-year-old man with a 3-year history of LP presented with oliguria. He was found to have acute renal failure and 37 g of proteinuria. The patient had had a right nephrectomy performed 27 years before because of a small nonfunctioning kidney. He was found to have paraaortic and mesenteric lymphadenopathy that were biopsied percutaneously. Despite supportive dialysis the patient continued to do poorly, went into coma and died. RESULTS: Biopsy of his single left kidney showed enlarged glomeruli completely replaced by amyloidosis confirmed by Congo red stain and electron microscopy. His paraaortic lymph nodes exhibited classic HL with CD30- and CD15-positive Reed-Sternberg cells in a background of mixed inflammatory cells including prominent eosinophils. A CT scan of the brain was negative; however, the MRI showed multiple periventricular parenchymatous lesions and changes of cerebral amyloid angiopathy. CONCLUSION: In this case, the concomitant presentation of systemic amyloidosis and HL post-LP suggests that amyloidosis is a late sequela of LP, an association that has never been reported before.

Acute Kidney Injury↗
PubMed2006

Lichen amyloidosis of the auricular concha: report of two cases and review of the literature.

Two cases of lichen amyloidosis of the auricular concha are presented here, supplementing the already approximately twenty cases of lichen amyloidosis of the auricular concha reported to date. The first patient is a 60-year-old woman who presented with a 4-year history of an itchy rash that started in the bowl of her left ear, and soon after appeared on the right side as well. The second patient is a 44-year-old woman who presented with a long-standing pruritic rash on her upper back. This had become noticeably darker over the past several years. In one of the two cases lichen amyloidosis of the auricular concha reported here, concomitant macular amyloidosis of the back was present. Combined cases of lichen and macular amyloidosis are termed biphasic amyloidosis, and provide support to the theory that these two variants of amyloidosis exist on the same disease spectrum.

Adult↗
PubMed1990

Splenic amyloidosis: correlations between chemical types of amyloid protein and morphological features.

Sixty-one autopsy cases of splenic amyloidosis were reviewed to assess the relationship between the morphological patterns and chemical types of amyloid protein. On the basis of immunohistochemical reactions of amyloid protein, the cases were classified into 34 cases of AA and 27 of AL amyloidosis. Amyloid deposition in the spleen was divided into three major sites: the red pulp, the white pulp, and blood vessels. Red pulp involvement by amyloid was noted in 52% of the AL cases but in none of the AA cases. White pulp amyloid deposition was found in 70% of the AL and 35% of the AA cases. This difference was statistically significant (P less than 0.001). On the other hand, vascular deposition of amyloid was invariably noted in all cases with AA or AL amyloidosis, affecting the AA cases rather severely. These results strongly suggest that the widely held concept of deposition of amyloid as predominantly vascular in AL amyloidosis and parenchymal in AA amyloidosis requires revision. Our findings indicate that parenchymal, especially the red pulp, involvement is a consistent feature of AL amyloidosis, whereas vascular involvement is a finding common to both types of systemic amyloidosis.

Adult↗