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PubMed1978

Study of light-, electron- and immunofluorescence microscopy of urinary sediment in amyloidosis.

Urinary sediment of 11 patients with amyloidosis and 12 without (with proteinuria or in good health) have been sutdied by different morphological techniques. By light microscopy, an amyloid-related substance was occasionally demonstrated both in patients with amyloidosis and in control subjects. Immunofluorescence (IF) showed substance A (amyloid component) to be present in some cases of amyloidosis and in controls. On electron-microscopy, fibrils with characteristic appearance of amyloid substance were found in some cases of amyloidosis (4 out of 11), but were also found in controls. It therefore seems difficult to establish the diagnosis of amyloidosis by microscopic studies of the urinary sediment.

Amyloid↗

PubMedDate not supplied

Vitreous amyloidosis without systemic or familial involvement.

Vitreous amyloidosis is a rare condition that occurs in some forms of Transthyretin hereditary Amyloidosis, mainly in Familial Amyloidotic Polyneuropathy type I. Vitreous opacities may be the earliest occurring or, in some cases, only symptom of this disorder. In such cases a family history of amyloidosis is usually present. We report on a case of vitreous amyloidosis in an 80-year-old man where there is no evidence of systemic involvement and no family history of amyloidosis.

Aged↗

PubMed1982

[New aspects of amyloidosis (author's transl)].

Amyloid is a substance that has the same composition of the basic qualities even in the different patterns of amyloidosis. Electron microscopic investigations have revealed that all forms of amyloidosis do not only exhibit homogeneous basic qualities but also common principle of accumulation. This may be commented as follows: Fibrils of amyloid are always found in close connection with basement membranes or basement membrane-like substances respectively produced by cells with the property of contracting (myocytic or "myopotent' cells). Collagen fibres of different types do not display a regular relation to the substance of amyloid. The origin and development of the various forms of amyloidosis depends on the three following factors: 1. On the extent of the production of amyloidogenic proteins which may belong to different types of proteins according to the basic disease; 2. On the way of protein silting (hematogenic silting-generalized amyloidosis; local enrichment--local amyloidosis); 3. On the site of predilection of all deposits of amyloid in the areas of basement membranes or basement membrane-like substances resp. produced by cells with the properties of contracting. A new classification should be made on the basis of these principles.

Amyloid↗

PubMed1987

Behçet's disease associated with amyloidosis.

A case of Behçet's disease associated with amyloidosis is reported. A 34 year-old woman was referred to our Department after suffering diarrhea for one month. She had the complete type of Behçet's disease which began development when she was 12 years old. Diarrhea disappeared after she was admitted to the hospital but stool occult blood was strongly positive. A barium enema revealed the disappearance of haustration in the left colon. Urinalysis was normal. She suffered from cholecystolithiasis, and a cholecystectomy was performed. A histopathological examination revealed amyloid deposits in the stomach, duodenum, large bowel, gallbladder, and kidney. Amyloid was indicated as AA type after treating kidney and colon sections with potassium permanganate. Upon discharge, she was free of symptoms. Up to the present time, 19 cases, including our case of Behçet's disease with amyloidosis, have been reported. Four cases in Japan have been reported in Japanese. Reviewing the 19 cases, however, all of them developed initially as Behçet's disease, followed by amyloidosis and there have only been three cases including ours where the type of amyloid was studied. Our finding was identical to the previous two reports; namely identification of the AA type which is common in secondary amyloidosis. These findings lead to a conclusion that amyloidosis associated with Behçet's disease is secondary.

Adult↗

PubMedDate not supplied

AA amyloidosis in psoriatic arthritis.

BACKGROUND: Amyloidosis is an extremely rare complication of psoriatic arthritis (PsA) and is associated with a poor prognosis. We report a case of amyloidosis secondary to severe PsA in a young patient and the course of his disease over a 13-year period of aggressive immunosuppression. METHODS: Diagnosis of renal amyloidosis was made on biopsy: multi-agent immunosuppressive therapy was continued with stabilisation of renal function. RESULTS: Marked deterioration in renal function subsequently occurred following a reduction in cyclosporin A (CyA) dose and repeat biopsy confirmed worsening amyloidosis. CONCLUSION: This case report emphasises the need for aggressive control of the inflammatory response in secondary amyloidosis.

Adult↗

PubMed2000

Amyloid goiter in a case of systemic amyloidosis secondary to ankylosing spondylitis.

Infiltrative diseases of the thyroid include systemic sclerosis, hemochromatosis, sarcoidosis, chondrocalcinosis and amyloidosis. Only rarely does thyroid amyloidosis result in clinically palpable goiter. Classically, amyloidosis is associated with tuberculosis, rheumatoid arthritis, multiple myeloma or inflammatory bowel disease. Only rarely does clinical amyloidosis develop in the setting of ankylosing spondylitis. We describe a case of amyloid goiter in a patient with ankylosing spondylitis-associated amyloidosis.

Adult↗

PubMed2000

New therapeutic approaches in primary systemic AL amyloidosis.

Primary systemic AL (amyloid light-chain) amyloidosis is a plasma cell disorder in which depositions of amyloid light-chain protein cause progressive organ failure. The prognosis of primary amyloidosis is generally poor, with a median survival of 1-2 years. There is no available treatment which improves impaired organ function by induction of amyloid mobilization. Since amyloidosis is a dynamic process, measures that reduce the supply of the amyloid fibril precursor protein can result in a major regression of the deposits. Conventional-dose melphalan can prolong the median duration of survival from 8.5 to 18 months, but the clinical response rates with improvement of impaired organ function are low and the response is slow. Preliminary data suggest that VAD is effective in AL amyloidosis. Up-front high-dose chemotherapy with autologous peripheral blood stem cell transplantation can result in an improvement of the patient's clinical condition, but the treatment-related toxicity can be high, owing to impaired organ function. The use of VAD followed by high-dose chemotherapy is the concept of a German trial. The improvement of the patient's condition prior to high-dose chemotherapy by induction of a remission with VAD might reduce the transplantation-related morbidity and mortality in amyloidosis.

Amyloid↗

PubMed2006

The value of the levels of acute phase reactants for the prediction of familial Mediterranean fever associated amyloidosis: a case control study.

In order to determine the role of levels of acute phase proteins (APPs) for the development of amyloidosis in familial Mediterranean fever (FMF) patients, the levels of serum amyloid A (SAA), C reactive protein (CRP), fibrinogen and erythrocyte sedimentation rate were measured in paired sera of 36 FMF patients during and in between acute attacks, 39 of their healthy parents (obligate heterozgotes), and 15 patients with FMF associated amyloidosis. To compare the levels of APPs, 39 patients with chronic infections or inflammatory diseases who may develop secondary amyloidosis, 20 patients with acute infections who are known to have elevated acute phase response but will never develop amyloidosis and 19 healthy controls were included. The median levels of all APPs are increased in the patients with FMF during attacks and a significant decrease was observed after the attack was over. The level of SAA was above reference range in all FMF patients during the attack free period and the level of at least one other APP was also above normal in 64% of the patients. Both CRP and SAA levels were found to be higher in obligate heterozygotes compared to controls. The levels of SAA in patients with FMF during the attack-free period, obligate heterozygotes and patients with FMF-amyloidosis were found to be similar. The levels in each group were found to be higher than SAA levels found in healthy controls yet lower than the levels measured in the patients with acute infections and patients with chronic inflammation or chronic infections. In conclusion, our results show that SAA level reflects subclinical inflammation with high sensitivity but its value for the prediction of amyloid formation process seems to be low.

Acute-Phase Proteins↗

PubMed2002

Primary systemic amyloidosis presenting as angina pectoris due to intramyocardial coronary artery involvement: a case report.

We describe a 76-year-old Japanese woman with primary systemic amyloidosis who presented with angina pectoris associated with ST-segment and T-wave abnormalities resulting from intramyocardial coronary artery amyloidosis. The patient was admitted to our hospital because of dyspnea and pretibial edema 7 years after the diagnosis of variant angina. A diagnosis of primary systemic amyloidosis (AL amyloid protein) was made after examination of gastric and endomyocardial biopsy specimens. The patient died of progressive, uncontrolled heart failure 3 months later. An autopsy study demonstrated only mild-to-moderate atherosclerosis in the epicardial coronary arteries. However, histological examination of the heart revealed diffuse stenoses and obstructions in the intramural coronary arteries by amyloid deposits. This patient had small-vessel coronary disease with ST-segment changes and angina caused by cardiac amyloidosis. A correct diagnosis of ischemic heart disease due to primary amyloidosis is important for estimation of the prognosis and for appropriate management.

Aged↗

PubMed2003

Endoscopic management of laryngo-tracheobronchial amyloidosis: a series of 32 patients.

Between June 1983 and December 2002, 32 patients were treated for primary localized laryngo-tracheobronchial amyloidosis (LTBA) at our institution. For enrollment in this retrospective study, at least one positive biopsy with Congo red stain and a diagnostic test battery excluding systemic or secondary amyloidosis were mandatory. The most common presenting symptom was dyspnea, which was observed in 75% of cases. Endoscopic appearance was described as submucosal plaques and nodules with a cobblestone appearance in 44% of patients, tumor-like in 28% and circumferential wall thickening in 28% of cases. Tracheobronchopatia osteochondroplastica was associated with LTBA in 22% of patients, both at the first treatment (four cases) or during follow-up examinations (three). Synchronous or metachronous disease in the larynx and tracheobronchial tree was observed in 47% of cases, while only one patient showed lung involvement with extensive amyloidosis of the trachea and bronchi. All but two patients were endoscopically treated. One of these was affected by a tracheal amyloidoma cured by endoscopic debulking and further tracheal resection-anastomosis. The other patient presented massive laryngo-pharyngeal involvement and was therefore treated by total laryngectomy. A total of 58 endoscopic procedures (range: 1-7; mean: two per patient) were carried out, 11 with CO2 laser (for supraglottic, glottic and selected subglottic lesions) and 47 with Nd:YAG laser (for the most part of subglottic lesions and amyloid deposits located in trachea and bronchi). Four patients required a tracheotomy and three necessitated a T-tube stent during management, which was subsequently removed in all cases. One patient was lost to follow-up, nine were endoscopically controlled elsewhere (Group A) and 22 were followed at our institution (Group B). In Group A, the patient with tracheal amyloidoma is asymptomatic and free of additional LTBA localizations 8 years after surgery. The remaining eight are asymptomatic, but with persistent endoscopic signs of amyloidosis. In Group B, five patients died: one from heart failure and another one from pneumonia 3 and 4 days after surgery, respectively. In one case death was from unrelated causes, and in the two remaining patients it was from respiratory failure due to uncontrolled bilateral bronchial amyloidosis in the subsequent 5 to 6 years. One patient is completely cured 5 years after surgery, and 16 are asymptomatic with persistence of LTBA.

Adult↗

PubMed2003

Efficacy of cyclophosphamide combined with prednisolone in patients with AA amyloidosis secondary to rheumatoid arthritis.

Secondary amyloid A (AA) amyloidosis is an uncommon yet important complication of rheumatoid arthritis (RA). It is one of the most relentless of the extra-articular features of RA, and suitable treatments have not yet been found. We studied the efficacy of cyclophosphamide (CYC) combined with prednisolone (PSL) in amyloidotic patients who had serum amyloid A (SAA) 1.3 genotype, which is a risk factor for secondary amyloidosis in Japanese RA patients. Fifteen RA patients who were SAA1.3 homo- and heterozygotes with biopsy-confirmed AA amyloidosis were treated with a combination of CYC and PSL. Laboratory variables of C-reactive protein (CRP), rheumatoid factor (RF), erythrocyte sedimentation rate (ESR), serum albumin (Alb), serum creatinine (Cre) and Lansbury's index were carried out by statistical analysis of changes between before and during the medication. According to the Mann-Whitney rank test, CRP, RF, ESR, Alb and Cre levels improved significantly with the combination treatment (p<0.05). Also, paired t-tests showed significance in Lansbury's index between before and during the medication (p=0.007). CYC combined with PSL ameliorated not only laboratory markers but also clinical rheumatoid activity in patients with amyloidosis secondary to RA, whose genotypes were SAA1.3 homo- and heterozygous. CRP, ESR, RF, Alb and Cre will be surrogate markers of therapeutic efficacy. The combination of CYC and PSL appears to be beneficial for Japanese RA patients who are SAA1.3 homo- and heterozygous carriers, associated with secondary AA amyloidosis.

Aged↗

PubMed2005

From scleredema to AL amyloidosis: disease progression or coincidence? Review of the literature.

Scleredema (also called scleredema of Buschke) is a fibromucinous connective tissue disorder of unknown cause that belongs to a group of scleroderma-like disorders. We report the case of a 64-year-old lady with long-standing scleredema, associated with a paraprotein, and progressing to multiple myeloma and AL amyloidosis. The relationship of scleredema with paraprotein and multiple myeloma is well established, but only two cases of scleredema associated with amyloidosis have been reported to date. We suggest that amyloidosis may be underdiagnosed in patients with scleredema and paraproteinaemia. Features attributed to extracutaneous manifestations of scleredema could represent systemic amyloidosis. We review published reports of scleredema associated with paraprotein and discuss the difficulties in the differential diagnosis of scleroderma-like disorders. We discuss the diagnosis of plasma cell dyscrasias and amyloidosis and their relevance in rheumatology practice.

Amyloidosis↗

PubMed2004

Renal function at the time of renal biopsy as a predictor of prognosis in patients with primary AL-type amyloidosis.

BACKGROUND: Amyloid light-chain (AL)-type amyloidosis is a plasma cell disorder with a poor prognosis for survival. Although prognostic factors, such as the number of organs involved and heart function or failure in respond to therapy have been clarified based on studies including a large series of patients, there are large interindividual differences in the prognosis of patients with primary AL-type renal amyloidosis. METHODS: To clarify the prognostic factors of AL-type renal amyloidosis, we retrospectively investigated the clinical manifestations, histopathological data, and prognosis of 21 patients with amyloidosis, who had been diagnosed by renal biopsy. RESULTS: Eleven patients died, at a mean observational time of 21.7 months after renal biopsy, whereas the mean observational time was 51.0 months for the 10 patients who survived. The creatinine clearance rate was significantly higher, and the serum creatinine concentration and the grade of interstitial damage were significantly lower in surviving patients (P < 0.05). The presence of amyloid fibrils in organs other than the kidney did not influence prognosis for survival. However, the intraventricular septum was thinner in surviving patients (P < 0.1). Thirteen patients had undergone melphalan-prednisolone therapy, but it did not affect prognosis for survival. Cox proportional hazard regression analysis revealed that the renal function at the time of diagnosis was a significant and independent prognostic factor for survival. CONCLUSIONS: Our study demonstrated that renal function at the time of biopsy and renal interstitial damage are the best predictors of survival in AL-type renal amyloidosis.

Amyloid↗

PubMed2002

Primary localized cutaneous amyloidosis in association with papillary thyroid carcinoma.

BACKGROUND: Lichen amyloidosis, a type of primary localized cutaneous amyloidosis, is characterized by pruritic brown or grayish pigmented papules. OBJECTIVE: We report a case of lichen amyloidosis in which the skin lesion consisted of asymptomatic salmon-pink-colored papules on the back of the neck to the upper back. This was different from typical clinical pictures of lichen amyloidosis. Our patient proved to have thyroid papillary carcinoma. CONCLUSION: Our case may suggest that lichen amyloidosis could be a cutaneous manifestation of thyroid cancers.

Adult↗

PubMed2005

Hereditary periodic fever and reactive amyloidosis.

Hereditary periodic fever syndromes (HPF) are a group of diseases characterised by recurrences of fever and inflammation separated by symptom-free intervals. Familial Mediterranean fever (FMF) is the most frequent entity within this group of disorders which further consists of hyperimmunoglobulinaemia D and periodic fever syndrome (HIDS), tumour necrosis factor receptor-associated periodic syndrome (TRAPS) and cryopyrin-associated periodic syndrome (CAPS). In recent years the causative genes have been identified. Reactive amyloidosis is a severe complication of HPFs. This is caused by deposition of fibrils that consist of the proteolytically cleaved acutephase protein serum amyloid A (SAA). Several factors have been identified that modulate the risk for developing amyloidosis, including SAA concentrations, polymorphisms in the SAA gene and ethnic origin. Furthermore, the risk of developing amyloidosis varies widely between the different HPFs. Colchicine is the cornerstone in the management of FMF, as it reduces the severity and frequency of attacks and is also effective in preventing amyloidosis. In the other HPFs, the introduction of anticytokine-based therapies is a promising new option in treating these inflammatory conditions and they potentially can prevent amyloidosis.

Amyloidosis, Familial↗

PubMed2026

Daratumumab monotherapy for relapsed AL amyloidosis: a single center retrospective analysis.

PURPOSE: Treatment of relapsed AL amyloidosis remains challenging as patients frequently suffer from severe organ dysfunctions. Daratumumab, a cornerstone drug in multiple myeloma and newly diagnosed AL amyloidosis, has also shown promise in relapsed AL amyloidosis. MATERIALS AND METHODS: To assess the efficacy of daratumumab, we conducted a retrospective study of 40 patients who received daratumumab monotherapy in our prospective amyloidosis cohort. RESULTS: The median age of the cohort was 67 years, and 31 patients were classified as stage III/IV by the Mayo 2012 guidelines and 32 patients as stage IIIa/IIIb by the European staging system. The overall hematologic and cardiac response rates were 72.5% and 42.4%, respectively. With median follow-up duration of 26.9 months, the estimated median progression-free survival was 21.9 months. Deep hematologic (p < 0.001) and cardiac response (p = 0.001) were associated with prolonged progression free survival. The duration of hematologic/cardiac responses were 26.2 months and 27.8 months, respectively. The median overall survival was 30.9 months. Treatment was well tolerated, as with 3 patients experiencing grade 3-4 hematologic adverse events and 8 experiencing grade 3-4 non-hematologic adverse events. DISCUSSION: Our findings suggest that daratumumab monotherapy provides rapid and durable responses and is generally well tolerated in patients with relapsed AL amyloidosis.

AL amyloidosis↗

PubMed1995

Cardiac immunocyte-derived (AL) amyloidosis: an endomyocardial biopsy study in 11 patients.

The objective of this study was to investigate the spectrum of morphologic features in myocardial biopsy specimens from patients with cardiac immunocyte-derived (AL) amyloidosis. Cardiac involvement is the most important predictor of survival in AL amyloidosis. Myocardial biopsy remains the method of choice for diagnosing cardiac amyloidosis when noninvasive studies give equivocal results. Histologic, immunohistochemical, ultrastructural, and morphometric studies were made on myocardial biopsy specimens from 11 patients in whom the diagnosis of AL amyloidosis was based on the demonstration of a monoclonal immunoglobulinopathy and of amyloid deposits in tissues. Histopathologic study showed amyloid in 10 of the 11 biopsies. In one biopsy (Congo red negative), the diagnosis was made by ultrastructural identification of amyloid fibrils. In all patients, the deposits formed perimyocytic layers that measured up to 18 microns in thickness. These layers formed along the basement membranes, which were partially preserved in 5 patients and unrecognizable in 6. Interstitial nodular deposits were also present in 5 patients. Immunohistochemical studies for the characterization of the proteins in the amyloid deposits were diagnostic in 1 patient and confirmatory in 10. Nodular deposits, thick perimyocytic layers of amyloid and small myocyte diameters were associated with shorter survival of the patients. Small-vessel involvement and myofilament loss occurred in all patients. In conclusion, myocardial biopsy serves to (1) establish the diagnosis of cardiac amyloidosis; (2) characterize immunohistochemically the proteins in the amyloid fibrils and (3) assess the degree of myocyte damage and atrophy.

Adult↗

PubMed1981

Amyloidosis in systemic lupus erythematosus.

Amyloidosis occurs in a significant proportion of patients with rheumatologic diseases. The fibrillar amyloid proteins in such patients are composed predominantly of amyloid A protein, which is characteristic of the amyloid deposits associated with chronic inflammatory diseases. Only four patients with amyloidosis associated with systemic lupus erythematosus (SLE) have been described previously; analyses of their fibrillar amyloid proteins were not reported. We present herein, a patient with SLE and amyloidosis. Histochemical staining of our patient's renal tissue with Congo red demonstrated that the amyloid deposits contained amyloid A protein, as defined by permanganate sensitivity. In addition, the patient's serum contained increased concentrations of serum amyloid A proteins. In review, each of the previously described patients with amyloidosis associated with SLE had renal amyloid deposits, with diagnosis in three during evaluation of proteinuria. Thus, although rare, amyloidosis should be considered in the differential diagnosis of proteinuria in patients with SLE.

Adult↗