Amyloidosis: A Clinical Overview
Rheumatic Disease Clinics of North America, April 29, 2013
Hazenberg BPC - The importance of typing amyloid with confidence, the usefulness of imaging techniques, the principles of treatment, and the need for well-planned treatment monitoring during follow-up are discussed.
amyloidosis is the name for protein-folding diseases characterized by extracellular deposition of a specific soluble precursor protein that aggregates in the form of insoluble fibrils.
The classification of amyloidosis is based on the chemical ...
Tocilizumab Treatment for Nephrotic Syndrome Due to Amyloidosis in Behcets Disease
Renal Failure, May 20, 2013
Redondo-Pachon MD et al. - Renal involvement is an unusual but significant Behcet´s disease (BD) complication and AA amyloidosis appears to be the most common etiology. IL-6 is a pro-inflammatory cytokine with an important role in AA amyloidosis development. Tocilizumab (TCZ) is a humanized anti-IL-6 receptor antibody that has emerged as an effective and specific treatment in AA amyloidosis secondary to chronic inflammatory disorders. The authors report on a ...
Urinary Cytopathology in Primary Bladder Amyloidosis
Acta Cytologica, April 29, 2013
Toll AD et al. - amyloidosis results from the accumulation of unique extracellular proteins which are not able to be degraded via the usual mechanism of lysosomal proteolysis. Isolated collections of amyloid within the bladder are extremely uncommon, and a cytopathologic description in voided urine has not been described to date. The authors' results show the difficulty of diagnosing urinary amyloid in the absence of clinical suspicion. Further, the presence of urinary amyloid is ...
AA Amyloidosis Associated With Systemic-Onset Juvenile Idiopathic Arthritis
American Journal of Kidney Diseases, May 14, 2013
Saha A et al. - The authors report a 12-year-old boy with nephrotic syndrome due to renal AA amyloidosis. The AA amyloidosis was associated with a 3-year history of systemic-onset juvenile idiopathic arthritis. The presence of serum amyloid A protein was confirmed by laser microdissection of Congo Red–positive glomeruli and vessels followed by liquid chromatography and tandem mass spectrometry; this analysis excluded hereditary and familial amyloidosis. Aggressive ...
Chronic diarrhea as the presenting feature of primary systemic AL amyloidosis: serendipity or delayed diagnosis
BMC Gastroenterology, May 2, 2013
Wang C et al. - A 43-year-old man with chronic diarrhea and weight loss was referred to the authors' hospital. Prior to his presentation, extensive evaluation including an exploratory laparotomy was carried out and did not yield any valuable findings. An echocardiography performed after repeated episodes of orthostatic hypotension revealed infiltrative cardiomyopathy. Moreover, biopsies of the terminal ileum revealed amyloid deposition confirmed by Congo Red staining. ...
Localized Amyloid Light-Chain Amyloidosis and Extramedullary Plasmacytoma of the Mitral Valve
Annals of Thoracic Surgery, May 6, 2013
Roumy A et al. - An unusual case of localized amyloid light-chain (AL) amyloidosis and extramedullary plasmacytoma of the mitral valve is described. The worsening of a mitral regurgitation led to investigations and surgery. The valve presented marked distortion and thickening by type AL amyloid associated with a monotypic CD138+ immunoglobulin lambda plasma cell proliferation. Systemic staging showed a normal bone marrow and no evidence of amyloid deposition in other localizations. ...
Clinical presentation, treatment, and prognosis of periocular and orbital amyloidosis in a university-based referral center
Clinical Ophthalmology, May 8, 2013
Aryasit O et al. - The purpose was to present the demographic data, clinical presentation, and treatment options, and to evaluate prognosis, for periocular and orbital amyloidosis in patients at the Songklanagarind Hospital, Thailand. Periocular and orbital amyloidosis presented with a variety of symptoms, depending on the location of the disease. A mass lesion was the most common symptom. The intent of the treatment modalities was to spare function.
Cardiac amyloidosis induces up-regulation of Deleted in Malignant Brain Tumors 1 (DMBT1)
Cardiovascular Pathology, May 16, 2013
Muller H et al. - The purpose of this study was to examine the role of innate defense components, i.e., Deleted in Malignant Brain Tumors 1 (DMBT1) and the complement system, in different types of cardiac amyloidosis. Up-regulated DMBT1 and complement activation in cardiac amyloidosis may be part of the activated pathways induced by protein aggregation and the consecutive inflammatory reaction.
Senile Systemic Amyloidosis: Clinical Features at Presentation and Outcome
Journal of the American Heart Association, April 29, 2013
Pinney JH et al. - The authors report the clinical presentation, natural history, and prognostic features of ATTRwt compared with cardiac-isolated AL amyloidosis and calculate the probability of disease diagnosis of ATTRwt from baseline factors. Factors at baseline associated with a worse outcome in ATTRwt are positive troponin T, a pacemaker, and NYHA class IV symptoms. The age of the patient at diagnosis and NT pro-BNP level can aid in distinguishing ATTRwt from AL amyloidosis.
Successful treatment of lichen amyloidosis using a CO2 surgical laser
Dermatologic Therapy, May 6, 2013
Norisugi O et al. - Two patients with lichen amyloidosis (LA) had been treated with topical corticosteroids, but with no effect on the eruptions. The present authors then started treating the affected area by superficial ablation using a CO2 surgical laser (LASER 30C, Lumenis Inc., Yokneum, Israel) at a setting of 10–15 watts with a 0.12-second pulse duration, 0.36-second rest duration, and 5-mm laser spot size. The present authors treated the patients twice a month ...