Showing papers on "Primary systemic amyloidosis published in 1994"
TL;DR: A retrospective review of the relevant literature on treatment and prognosis in localized and systemic amyloidosis dating back to 1975 found the presence of congestive heart failure, beta 2-microglobulin, and whether peripheral neuropathy dominates the presentation are the most important prognostic factors.
97 citations
TL;DR: The clinical characteristics, temporal artery biopsy findings, and the response of vascular and musculoskeletal symptoms to corticosteroid therapy in 4 patients with primary systemic amyloidosis who presented with manifestations of GCA or PMR are described.
Abstract: Primary systemic amyloidosis may present with features suggesting a vasculitis, including giant cell arteritis (GCA) and polymyalgia rheumatica (PMR). In this report, we describe the clinical characteristics, temporal artery biopsy findings, and the response of vascular and musculoskeletal symptoms to corticosteroid therapy in 4 patients with primary systemic amyloidosis who presented with manifestations of GCA or PMR.
66 citations
TL;DR: A vigorous attempt to control disease activity of the RA should be made in order to eliminate the production of SAA, an acute phase protein, and attention should be given to adequate supportive treatment.
Abstract: Approach to the management of AA amyloidosis complicating RA. (A) In case of proteinuria or loss of renal function a rectal biopsy or a subcutaneous fat biopsy is a suitable screening method for the detection of amyloidosis. If in any doubt, try to ascertain the diagnosis by renal biopsy. Adequate staining with alkaline Congo red and preferably immunohistochemical staining with anti-AA antibodies should be performed. Beware of renal pathology other than amyloidosis even in the presence of a positive rectal biopsy. (B) A vigorous attempt to control disease activity of the RA should be made in order to eliminate the production of SAA, an acute phase protein. The response to treatment should be monitored by serial measurements of CRP and preferably SAA. (C) The function of some vital organs should be evaluated: (a) endogenous creatinine clearance and the extent of proteinuria; (b) electrocardiogram and optional echocardiography; (c) thyroid function and adrenocortical function; (d) intestinal absorption tests; (e) optional--SAP scintigraphy and turnover studies. (D) Attention should be given to adequate supportive treatment: (a) blood pressure control; (b) treatment of intercurrent infections; (c) corticosteroids during major surgical procedures; (d) pay attention to the possible effect of NSAID on proteinuria and renal function. (E) In case of total renal failure or uncontrollable proteinuria: (a) consider the possibility of primary renal transplantation; (b) otherwise regular haemodialysis is indicated.
63 citations
TL;DR: Mucocutaneous manifestations were found in both primary type and myeloma-associated amyloidosis and the existence of amyloids in tongue and rectum biopsies in 17 cases and abdominal fat aspiration in 1 case and biopsie from clinically normal-looking skin also showed positive results.
Abstract: Systemic amyloidosis is a rare disorder and usually occurs in aged persons with a poor prognosis. Certain mucocutaneous symptoms and signs may act as important clues for diagnosis. Skin or tongue biopsy may also provide important information to establish a firm diagnosis. During the past 14-year period, we found 19 cases of systemic amyloidoses: 8 (42%) with primary systemic amyloidosis, 8 (42%) associated with multiple myeloma and 3 (16%) were secondary amyloidosis. Ages of the patients ranged from 52 to 71 years with a mean age of 62 years at diagnosis. Median survival was only 8 months. Mucocutaneous manifestations were found in both primary type and myeloma-associated amyloidosis. In addition to the existence of amyloid in tongue and rectum biopsies in 17 cases and abdominal fat aspiration in 1 case, biopsies from clinically normal-looking skin also showed positive results in 18 out of 19 cases.
16 citations
TL;DR: Although very rare in this age, primary systemic amyloidosis should be considered as a cause of pleural effusion, proteinuria, and congestive heart failure and should lead to further investigation.
Abstract: The case of a 30-year-old man with primary systemic amyloidosis is reported. Three months prior to admission the patient developed fever, night sweats, dyspnea, and bilateral ankle swelling. Recurrent left-sided pleural effusion led to further investigation when massive proteinuria with free monoclonal lambda chains in the urine became evident. Abdominal subcutaneous fat aspiration and renal biopsy confirmed the diagnosis of amyloidosis. Bone marrow biopsy and bone scan did not reveal multiple myeloma. Echocardiography showed a sparkling texture of the interventricular septum. Pulsed-wave Doppler recording of the left ventricular inflow profile showed the pattern of advanced cardiac amyloidosis consistent with markedly impaired diastolic heart function. Electrocardiogram-gated magnetic resonance imaging was carried out for noninvasive evaluation of cardiac function. The patient was started on repeated courses of melphalan, prednisone, and colchicine therapy. Despite increasing deterioration of renal function the therapy was tolerated quite well, and the patient is still alive 10 months after initial diagnosis. Although very rare in this age, primary systemic amyloidosis should be considered as a cause of pleural effusion, proteinuria, and congestive heart failure and should lead to further investigation.
3 citations