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PubMed1998

Development of gastrointestinal beta2-microglobulin amyloidosis correlates with time on dialysis.

Dialysis-associated beta2-microglobulin (beta2m) amyloidosis affects predominantly musculoskeletal tissue, but visceral involvement also occurs. To evaluate the clinical significance and prevalence of gastrointestinal beta2m amyloidosis, we studied hemodialysis patients admitted for gastrointestinal-related complaints. Hemodialysis patients (excluding those with non-beta2m amyloidosis) who were admitted with gastrointestinal complaints from 1984 to 1994 were identified. Gastrointestinal tissues from patients with available autopsy or surgical specimens were examined using hematoxylin and eosin stain, Congo red stain, and beta2m immunostain. Each case was evaluated independently by two pathologists and scored for quantity and location of beta2m amyloid and associated pathology. Of 24 patients, eight (four men and 4 women) had beta2m amyloid deposits within the gastrointestinal tract. Acute clinical presentation ranged from abdominal pain to gastrointestinal bleeding and was not significantly different for patients with or without gastrointestinal beta2m amyloid deposits. However, the mean time on dialysis of 15.3 +/- 5.7 years (range 6-24 years) for patients with gastrointestinal beta2m amyloidosis was significantly greater than that of patients without gastrointestinal beta2m amyloidosis (10.5 +/- 7.0 years, range <1 to 22 years, p < 0.05). Vascular histopathology ranged from mild focal thickening of vessel walls to massive vascular beta2m amyloid deposition with thrombosis. Extravascular beta2m amyloid ranged from mild to severe with marked expansion of the submucosa. Mucosal pathology ranged from none to severe ulceration. The degree of beta2m amyloid and the associated pathology tended to increase in severity with time on dialysis. Gastrointestinal beta2m amyloid deposition is an underappreciated complication of chronic hemodialysis that is significantly associated with increased time on dialysis. Gastrointestinal beta2m amyloidosis should be considered in any patient on hemodialysis 10 years or more who has gastrointestinal symptoms and can be identified in resection specimens as well as some biopsy specimens. Congo red stain and beta2m immunostains may be necessary for sensitive histopathologic evaluation of gastrointestinal beta2m amyloidosis.

Adult↗
PubMed1985

Crohn's ileitis complicated by amyloidosis: observations and therapeutic considerations.

We present a patient with clinically asymptomatic amyloidosis associated with Crohn's ileitis. A distinction should be made between immunocytic dyscrasia associated with amyloidosis (formerly primary or myeloma-associated amyloidosis) and acquired systemic amyloidosis (formerly secondary amyloidosis). We compare the natural course of amyloidosis complicating Crohn's disease with these complicating familial Mediterranean fever (FMF), and discuss the role of resection and the rationale behind colchicine therapy. Our patient is the first reported case in which colchicine therapy alone has been successful in the prophylactic treatment of amyloidosis complicating Crohn's ileitis.

Adult↗
PubMed2003

Amyloidosis of the upper aerodigestive tract.

OBJECTIVES/HYPOTHESIS: To delineate the clinical and pathologic characteristics of upper aerodigestive tract amyloidosis with particular attention to laryngeal amyloidosis. STUDY DESIGN: Retrospective chart review of patients with amyloidosis of Thomas Jefferson University and its affiliated hospitals. MATERIAL AND METHODS: The charts of 16 patients with upper aerodigestive tract amyloidosis identified from the databases of the Thomas Jefferson University pathology department were reviewed and included in the study. RESULTS: Sixteen patients (9 male and 7 female, with an average age of 49.8 years) with upper aerodigestive tract amyloidosis were identified. The most common site of amyloid involvement was the larynx. Consequently, patients most commonly presented with hoarseness (14 of 16). All patients underwent surgical removal of the amyloid deposits. Fourteen patients had primary localized amyloidosis. Two experienced systemic involvement. Seven of the 16 patients developed recurrences requiring further treatment. CONCLUSIONS: Amyloidosis of the upper aerodigestive tract generally behaves as a benign, localized condition treatable by surgical resection. Regular follow-up with laryngoscopy is indicated for early diagnosis of recurrence, and multiple surgical procedures may be required to control symptoms.

Adult↗
PubMed1986

Cranial nerve deficits due to amyloidosis associated with plasma cell dyscrasia.

Cranial nerve dysfunction is exceedingly rare in amyloidosis. Cranial nerve deficits have been described in the very rare form of familial amyloidosis in which there appears to be an autosomal dominant pattern of inheritance. By contrast, they are virtually unreported in the two common forms of amyloidosis, that is, secondary amyloidosis (AA), usually associated with a chronic inflammatory lesion, and primary amyloidosis (AL), resulting from light-chain immunoglobulins of plasma cell dyscrasias. Although neurologic involvement in these forms of amyloidosis has been reported in the peripheral nerves, spinal nerve roots, and autonomic ganglia, the absence of reports of cranial nerve lesions is remarkable. We report the case of a woman with multiple bilateral cranial nerve deficits involving the left trigeminal, left facial, right abducens, and hypoglossal nerves. She had nonfamilial amyloidosis associated with a plasma cell dyscrasia of the IgG kappa type with increased production of light-chain immunoglobulins. The rarity of cranial nerve involvement in this clinical setting is highlighted by three previous studies, each of which reported involvement of only a single nerve (either the facial or olfactory nerve). Since aging has been correlated with congophilic changes in the brain, the explanation for the sparing of cranial nerves can be only speculative.

Amyloidosis↗
PubMed2007

Primary solitary cervical amyloidosis: case report and review of the literature.

STUDY DESIGN: A case report and literature review are presented. OBJECTIVE: To describe and review the clinical presentations, characteristic findings from imaging studies, types, differential diagnosis, prognosis, and treatment of amyloidoma arising within the vertebrae. SUMMARY OF BACKGROUND DATA: Amyloidoma can occur in the bone, skin, larynx, lymph nodes, urinary bladder, eye, tongue, and gastrointestinal system. However, amyloidomas affecting the vertebral bones are very rare. To our knowledge, only 3 cases of amyloidoma involving cervical spine have been reported previously. METHODS: In this report, we present a case of solitary amyloidosis of the cervical spine. RESULTS: The differential diagnosis of primary solitary spinal amyloidoma includes metastasis, infection, primary bone tumors, plasmocytoma, and Potts abscess. The correct diagnosis can be achieved only after the specific staining of tissue. The prognosis of amyloidosis is related to the specific form of amyloidosis. However, primary solitary amyloidosis has the best prognosis, although a limited number of patients without long-term follow-up studies have been reported. CONCLUSIONS: Primary solitary amyloidosis is a rare form of the amyloidosis, which is different from the other forms of amyloidosis because of excellent prognosis with surgical excision. Combined surgical excision and spinal stabilization is the best treatment.

Aged↗
PubMed1991

Gene rearrangement studies in the diagnosis of primary systemic and nodular primary localized cutaneous amyloidosis.

Difficulties may arise in the diagnosis of patients with clinical features suggestive of plasma cell dyscrasia-related amyloidosis (amyloidosis L), but without evidence of a paraprotein. We have employed gene rearrangement methodology to demonstrate the clonality of bone marrow cells not only in a patient with myeloma-associated systemic amyloidosis, but also in a patient with "primary" systemic amyloidosis without overt myeloma or a detectable paraprotein. Furthermore, we have shown the clonality of the amyloid-producing plasma cells within a skin nodule of a patient with primary localized cutaneous amyloidosis; by contrast, clonal rearrangement was not detected in bone marrow cells from this patient. This finding provides definitive proof that organ-limited nodular primary localized cutaneous amyloid deposits arise in relation to cutaneous plasmacytomas. Gene rearrangement studies may enable early diagnosis and initiation of treatment in patients with systemic amyloidosis L, as well as their differentiation from patients with organ-limited nodular cutaneous amyloidosis, who do not require aggressive therapy.

Aged↗
PubMed1983

Amyloidogenesis in organ-limited cutaneous amyloidosis: an antigenic identity between epidermal keratin and skin amyloid.

Epidermal keratin was extracted and antibody against this protein was produced in rabbits. Various forms of organ-limited cutaneous amyloidosis (lichenoid, macular, and nodular amyloidosis, and basal cell epithelioma) and primary systemic amyloidosis were immunohistochemically examined to test the identity between epidermal keratin and skin amyloid. Amyloids in lichenoid and macular amyloidoses, and in basal cell epithelioma had an identical antigenicity with epidermal keratin, whereas amyloids in nodular amyloidosis and systemic amyloidosis did not have this identity. In addition, amyloid in lichen amyloidosis contained disulfide bonds as in keratin. Connective tissue components including filaments of fibroblasts and vascular endothelial cells did not react with this antikeratin antibody. It was concluded that at least some of the amyloid substance in organ-limited cutaneous amyloidosis is derived from degenerated epidermal keratinocytes through filamentous degeneration or apoptosis.

Amyloid↗
PubMed2004

Generation of B220low B cells and production of autoantibodies in mice with experimental amyloidosis: association of primordial T cells with this phenomenon.

To investigate the immunological state in amyloidosis, mice were twice intraperitoneally injected (2-week interval) with casein emulsified in complete Freund's adjuvant. Two weeks after the treatment, amyloid deposits were detected in the spleen and other organs of these mice. The number of lymphocytes yielded by the liver and spleen increased significantly. The most affected lymphocyte subset was found to be B cells, namely, the total number of B cells increased and unusual B220low B cells were newly generated in the liver and spleen. In other words, not only normal B220high B cells but also unusual B220low B cells were detected in these organs of mice with amyloidosis. In parallel with this phenomenon, autoantibodies against denatured DNA were detected in sera. Since such autoantibodies are known to accompany the functional activation of NKT cells, NKT cell-deficient mice were used for the induction of amyloidosis. Such mice showed less formation of amyloidosis and lower levels of autoantibodies in sera. Athymic nude mice were NKT cell-deficient but NK1.1- TCRint cells were present. These athymic mice showed an intermediate induction of amyloidosis. The cytokine profile seen in mice with amyloidosis was the Th0 type, showing simultaneous production of IL-4 and IFNgamma. These results suggest that the generation of B220low B cells and the production of autoantibodies in aid of primordial T cells may be major immunological mechanisms in amyloidosis mice.

Amyloidosis↗
PubMed1980

Experimental murine amyloidosis. Evaluation of induction methods and strain difference.

Several experimental methods inducing murine amyloidosis were tested using six strains of mice including congential asplenic mice and athymic nude mice. Traditional induction method of amyloidosis by casein injections failed to cause amyloidosis in C3H/He mice. Single injection with complete Freund's adjuvant reinforced with Mycobacterium butyricum was also unable to induce amyloidosis in C3H/He, CBA, and BALB/c mice. Six or four injections with complete Freund's adjuvant at an interval of once a week successfully induced amyloidosis in CBA mice with high incidence but not in C3H/He, ICR/SLC, and BALB/c mice. Athymic nude mice with genetic background of BALB/c and congenitial asplenic mice, cross-bred with C57BL/6 X C3H, were free from amyloidosis after six or fourteen injections with the adjuvant. Experimental amyloidosis in mice, therefore, might mainly depend on the strain of mice used and the induction method chosen.

Amyloidosis↗
PubMed2006

Immunohistochemical study of immunoglobulin light chain amyloidosis with antibodies to the immunoglobulin light chain variable region.

To detect immunoglobulin (Ig) light chain amyloidosis (AL amyloidosis) in formalin-fixed, paraffin-embedded tissue sections by immunohistochemistry, polyclonal antibodies were generated against synthetic peptides corresponding to amino acids 1-19 of the Ig lambda light chain V lambda VI subgroup (anti-V lambda VI (1-19)) and the Ig kappa light chain Vkappa I subgroup (anti-Vkappa I (1-19)). Anti-V lambda VI (1-19) antibody reacted with amyloid deposits in 21 of 22 Alambda amyloidosis cases, and anti-Vkappa I (1-19) antibody reacted with amyloid deposits in 10 of 11 Akappa amyloidosis cases. Immunoreactivity varied in intensity by case and within specimens. Surprisingly, amyloid deposits were positive for anti-V kappa I (1-19) staining in one case of Alambda amyloidosis. Analysis of anti-V lambda VI (1-19) and anti-Vkappa I (1-19) antibody reactivity by ELISA showed some cross-reactivity with peptides other than antigen peptides. The antibodies were not reactive in all cases of AL amyloidosis examined but may be useful, together with anti-Ig constant region antibodies, for immunohistochemical diagnosis of AL amyloidosis.

Amyloid↗
PubMed1991

Doppler assessment of diastolic function in cardiac amyloidosis.

The hallmark of cardiac amyloidosis is abnormal diastolic function secondary to amyloid infiltration of the ventricular walls, which accounts for the term "stiff heart syndrome." The abnormal diastolic function has not yet been well characterized, however. Thus, we assessed left and right ventricular diastolic function in 53 patients with cardiac amyloidosis. We measured the left and right ventricular inflow and venous flow velocities with Doppler echocardiography and found a range of Doppler filling abnormalities. These abnormalities were dependent on the degree of amyloid infiltration of the heart as measured by mean left ventricular wall thickness. Patients with advanced cardiac amyloidosis (a wall thickness greater than or equal to 15 mm) showed restriction, while patients with early cardiac amyloidosis (a wall thickness less than 15 mm) showed abnormal relaxation or normal filling. In another study, during a 13-month follow-up, patients with early cardiac amyloidosis showed Doppler patterns that evolved from abnormal relaxation through a normal stage, to an advanced stage of restrictive disease. We also have demonstrated the importance of left ventricular inflow variables in predicting the outcome of patients with cardiac amyloidosis. We concluded that Doppler echocardiography is useful in characterizing abnormal diastolic function in patients with cardiac amyloidosis.

Amyloidosis↗
PubMed1993

Conjunctival amyloidosis: is it related to trachoma?

Due to presence of trachoma in a large percentage of cases of conjunctival amyloidosis, trachoma has been blamed for causing secondary localized amyloidosis. However, there is no study to demonstrate evidence of amyloidosis in tarsoconjunctival specimens of trachomatous lids. 50 eyes of 35 patients having trachomatous eyelids with thickened tarsal plate, trichiasis and entropion had a tarsoconjunctival biopsy (3 mm x 20 mm) from the upper lid. Histopathological examination using Haematoxylin--eosin and congo red stains failed to reveal evidence of amyloid deposition in any of the biopsies. This is the first histopathological study of amyloidosis in trachomatous patients. Our study rules out the long-standing concept of trachoma having causal relationship with conjunctival amyloidosis. It is concluded that conjunctival amyloidosis occurring in trachomatous lids should be considered as primary localized amyloidosis.

Aged↗
PubMedDate not supplied

The pathogenesis of reactive systemic amyloidosis.

The diagnosis of amyloidosis is based on the presence of extracellular tissue deposits of proteinaceous material that demonstrate a characteristic green color when stained with Congo red and viewed under polarized light. Several different proteins are amyloidogenic but, in domestic animals, spontaneously occurring systemic amyloidosis is reactive in nature and characterized by the presence of amyloid protein AA. This type of systemic amyloidosis may occur secondary to chronic inflammatory or neoplastic disease, but in many instances no predisposing disease is found. A sustained increase in the serum concentration of serum amyloid A protein (SAA) is necessary but not sufficient for the development of reactive amyloidosis. Other inherited and acquired host-related factors are likely to be important in the development of reactive amyloidosis because this condition develops in few patients with chronic inflammatory disease. The tissue tropism of amyloid deposits varies with the amyloid protein itself and species affected. The consequences of amyloidosis for the host depend upon the tissues involved and the response of these tissues to the presence of the amyloid deposits. In domestic animals, reactive systemic amyloidosis is nephropathic, leading to end-stage renal disease, and the clinical presentation is that of uremia.

Amyloidosis↗
PubMed2004

Primary amyloidosis presenting as small bowel encapsulation.

Amyloidosis is a pathological process which encompasses a spectrum of diseases that result from extracellular deposition of pathological fibrillar proteins. Clinical presentations vary depending on the organs involved. There is no documented case of amyloidosis presenting as small bowel encapsulation. A previously healthy 62-year-old man developed a small bowel obstruction in 1997. At surgery, a peculiar membrane encasing his entire small bowel was discovered. This appeared to have no vascularity and was removed without difficulty, exposing a grossly normal bowel. Histopathology revealed thick bands of collagen overlying the peritoneal surface, which was congo red positive and showed apple green birefringence. The findings were consistent with encapsulating peritonitis due to amyloidosis. There was no history or symptoms of any chronic inflammatory condition and he became symptom-free postoperatively. An abdominal fat pad biopsy failed to demonstrate amyloidosis. Endoscopic duodenal biopsies revealed classical primary amyloidosis. Quantitative immunoglobulins, lactate dehydrogenase, C3, C4 and beta-2 microglobulin were normal. Protein electrophoresis identified monoclonal paraprotein, immunoglobulin G lambda 3.7 g/L. Bone marrow biopsy and aspirate revealed only a mild plasmacytosis (5% to 10%). Echocardiogram and skeletal survey were normal. He had mild proteinuria. Complete blood count, C-reactive protein, calcium, albumin and total protein were normal. No specific therapy was instituted. In January of 1998 the patient remained asymptomatic with no gastrointestinal, cardiovascular or constitutional symptoms. He had developed nephrotic range proteinuria (3.95 g/24 h), microalbuminuria, hypoalbuminemia and a renal biopsy consistent with renal amyloidosis. In 1999 there was an increase in the monoclonal paraprotein (6.2 g/L). The remaining investigations were normal except for an echocardiogram which showed left ventricular hypertrophy but a normal ejection fraction and no diastolic dysfunction. He went on to have high-dose chemotherapy and an autologous stem cell transplant in September, 2000. He has subsequently developed renal insufficiency. To our knowledge this is the first reported case of primary amyloidosis presenting as small bowel obstruction from encapsulating peritonitis.

Amyloidosis↗
PubMedDate not supplied

Behcet's disease and AA-type amyloidosis.

Behcet's disease (BD) is a multisystem disorder characterized by vasculitis. To our knowledge, 52 patients with BD and amyloidosis have previously been described in the literature. Nephrotic syndrome was the most common type of presentation of amyloidosis in these patients. The prognosis of patients with BD and amyloidosis has not been reported before. In this report, we present a patient with BD and AA-type amyloidosis and analyze the prognosis in these patients. Follow-up and prognosis have been reported in 23 patients. Ten of these 23 patients died and most of these deaths occurred within 3 months after the diagnosis of amyloidosis. End-stage renal disease developed shortly in 4 patients. BD should be considered in the differential diagnosis of AA amyloidosis. In conclusion, the cases with BD and amyloidosis carry poor prognosis.

Adult↗
PubMed1999

Reversal of nephrotic syndrome due to AA amyloidosis in psoriatic patients on long-term colchicine treatment. Case report and review of the literature.

A case of nephrotic syndrome due to AA amyloidosis in a young woman suffering from erythrodermic psoriasis and psoriatic arthropathy is reported. The nephrotic syndrome regressed completely during long-term (57 months) colchicine treatment. There are 39 case reports in the literature of psoriasis associated with amyloidosis. More than 85% of these patients had concomitant arthropathy. This suggests that arthritis may be an important factor in the appearance of amyloidosis. 59% of psoriatics with amyloidosis had renal failure and 56% of them died shortly after diagnosis of amyloidosis. These observations support the view that amyloidosis associated with psoriasis is an aggressive disease that may be fatal. However, the clinical course of our patient suggests that renal amyloidosis associated with psoriasis may be successfully treated by colchicine.

Adult↗
PubMed2001

Prognosis of renal amyloidosis: a clinicopathological study using cluster analysis.

Progression of renal amyloidosis is associated with severe proteinuria or nephrotic syndrome, and various mechanisms have been postulated to explain these complications. We studied the acceleration of proteinuria and reduced renal function by cluster analysis using clinical parameters, renal histological findings, type of renal amyloidosis and follow-up data. We divided 97 cases into three groups of renal amyloidosis. Accelerated progression correlated with serum creatinine (s-Cr) levels at renal biopsy and histological grade of renal damage by amyloid deposition (p < 0.0001). The most influential prognostic factors (s-Cr level > or =2.0 mg/dl) were tubulointerstitial and vascular damage induced by amyloid deposition at biopsy (odds ratio 96.9 and 69.2, respectively). In addition, we found amyloidosis type amyloid associated (AA) correlated with more amyloid-mediated vascular and tubulointerstitial damage than amyloidosis type amyloid light chain (AL) (p < 0.001, p < 0.01, respectively). Proteinuria and nephrotic syndrome were more severe in cases of amyloidosis AL than in amyloidosis AA (p = 0.076). In conclusion, less tubulointerstitial and vascular damage was caused by amyloid deposition; this was slowly progressive. Amyloid AA was detected in tubulointerstitial tissue and vessels more frequently than amyloid AL. Heavy proteinuria and/or nephrosis were not indicators of rapid progression.

Adult↗
PubMed2004

Continuous ambulatory peritoneal dialysis in familial Mediterranean fever amyloidosis patients with end-stage renal failure: a single-centre experience from Turkey.

BACKGROUND/AIMS: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterised by recurrent fever attacks and polyserositis which may lead to the development of AA amyloidosis and end-stage renal disease (ESRD). In this study, we aimed to evaluate the efficacy of continuous ambulatory peritoneal dialysis (CAPD) in FMF-amyloidosis patients with ESRD. METHODS: Forty age- and sex-matched patients undergoing CAPD at our centre between 1996 and 2002 were included in the study. Of these, 10 had FMF-amyloidosis, 10 had diabetes mellitus (DM), 10 had chronic glomerulonephritis (CGN) and 10 had chronic interstitial nephritis (CIN). Efficiency of CAPD, development of complications, presence of other diseases and survival were compared. RESULTS: With the onset of ESRD, the frequency of FMF peritonitis attacks decreased, with less attacks occurring during CAPD in FMF-amyloidosis patients (p < 0.05). There was no significant difference between the FMF-amyloidosis group and other groups in terms of efficiency of CAPD, peritoneal function, complications and survival. DM patients had a shorter survival period compared with CGN and CIN patients (p < 0.05), but there was no survival difference between FMF-amyloidosis patients and other groups (p > 0.05). CONCLUSIONS: We conclude that CAPD is an effective and safe renal replacement therapy for FMF-amyloidosis patients with ESRD.

Adult↗