BACKGROUND Light chain (AL) amyloidosis is a rare disease characterized by misfolded amyloid protein deposits in tissues and vital organs, and little is known about the burden of AL amyloidosis on health-related quality of life. This study aimed to quantify the burden of AL amyloidosis in terms of health-related quality of life in a diverse, community-based sample of AL amyloidosis patients. ...

There are no treatment modalities, which were proven to prevent the deposition of amyloid, proteinuria, and loss of renal function due to amyloidosis. Anti-tumor necrosis factor agents (anti-TNFs) were shown to decrease the production of serum amyloid A protein.We aimed to evaluate the long-term efficacy and safety of anti-TNFs in secondary (AA) amyloidosis patients treated in a single center.T...

Two hundred and thirty-three patients with renal amyloidosis were studied in an attempt to identify the incidence and pattern of the disease in northern India. The incidence of amyloidosis was 1.01% of 6431 post-mortems and 8.4% of 1980 renal biopsies from patients who presented with clinical evidence of glomerular disease. Two hundred and three patients (87.1%) had secondary amyloidosis, 22 (9...

BACKGROUND AND AIMS The liver is frequently involved in amyloidosis but the significance of hepatic amyloid has not been systematically studied. We have previously developed scintigraphy with 123I serum amyloid P component (123I-SAP) to identify and monitor amyloid deposits quantitatively in vivo and we report here our findings in hepatic amyloidosis. METHODS Between 1988 and 1995, 805 patien...

The amyloidoses are a group of deposition diseases in which the tissue deposits are composed of protein fibrils. These fibrils are the result of aggregation of specific precursor proteins into ordered structures which are resistant to proteolytic digestion and solubilisation. The ordered structure of the fibrils causes the deposits to be birefringent and, when histological sections are stained ...

Two major types of amyloidosis are primary amyloidosis or amyloid light chain amyloidosis and secondary amyloidosis. Although amyloidosis involves a variety of organ systems including skin, the occurrence of bullous skin lesions is rare. Little is known about the mechanism of blister formation. These blisters are often hemorrhagic and typically occur in the oral mucosa. Only a few case reports ...

BACKGROUND Cutaneous amyloidosis has been classified into primary cutaneous amyloidosis (PCA, OMIM #105250), secondary cutaneous amyloidosis and systemic cutaneous amyloidosis. PCA is the deposition of amyloid in previously apparent normal skin without systemic involvement. Amyloidosis cutis dyschromica (ACD) is a rare distinct type of PCA. Here, the unique clinical and histological findings of...

Background Carpal tunnel syndrome (CTS) is one of the most common clinical manifestations of TTR-related amyloidosis, both hereditary (ATTR), and wild type (senile systemic amyloidosis, SSA) and often precedes cardiac symptoms. The exact prevalence of CTS in light-chain amyloidosis (AL), ATTR and SSA is not known. We therefore aimed to establish prevalence, risk factors and possible association...

Amyloidosis is defined as the presence of extra-cellular deposits of an insoluble fibrillar protein, amyloid. The pulmonary involvement of amyloidosis is usually classified as tracheobronchial, parenchymal nodular, or diffuse alveolar septal. A single nodular lesion can mimic various conditions, including malignancy, pulmonary tuberculosis, and fungal infection. To date, only one case of nodula...

This study was performed in real clinical practice settings. Our paper describes two case studies which show the difficulty of differential diagnosis monoclonal gammopathies, multiple myeloma associated with AL-amyloidosis and primary AL-amyloidosis.