In immunoglobulin light-chain (L) amyloidosis the cause of death is most commonly due to cardiac involvement. Cardiac amyloidosis is frequently diagnosed by postmortem examination. There are many reports of diagnoses of cardiac amyloidosis established by autopsy of cardiac tissue. We report four patients whom we could diagnose as having cardiac amyloidosis by echocardiogram and electrocardiogra...

There are two major forms of amyloidosis, primary amyloidosis (AL) and secondary amyloidosis. AL amyloidosis results from deposition of immunoglobulin light chains or their fragments. One such example is AL amyloidosis associated with multiple myeloma, in which overproduced immunoglobulin light chains get deposited onto tissues, leading to tissue dysfunction. Amyloidosis in the intestines can p...

BACKGROUND Amyloidosis includes a group of diseases characterized by the extracellular deposition of various fibrillary proteins that can autoaggregate in a highly abnormal fibrillary conformation. The amyloid precursor protein of systemic light-chain (AL) amyloidosis is comprised of monoclonal light chains that are due to plasma cell dyscrasia. The clinical presentation of patients with AL amy...

The cardiovascular system is a common target of amyloidosis. This review presents the current clinical and diagnostic approach to amyloidosis, with the emphasis on cardiovascular involvement. It summarises recent nomenclature, classification, and pathogenesis of amyloidosis. In addition, non-invasive possibilities are discussed, together with endomyocardial biopsies in the diagnosis of cardiac ...

Although amyloidosis of the respiratory tract is well recognized, pleural involvement is very rare with only two cases being reported in the past. We report a case of primary amyloidosis with pleural effusion and suggest that pleural involvement and pleural effusion be added to the classification of pulmonary amyloidosis, and that amyloidosis be added to the list of causes of a pleural effusion.

BACKGROUND Amyloidosis cutis dyschromica is a rarely documented variant of cutaneous amyloidosis. To date, only 26 cases have been reported. OBJECTIVE The purpose of this study was to improve the clinical and histopathological data for this variant of amyloidosis and to highlight the immunohistochemical features of the disease. The published cases were also reviewed. METHODS We performed a ...

BACKGROUND Amyloidosis is a heterogeneous disease caused by deposition of amyloid fibrils in organs and thereby interfering with physiological functions. Hardly any incidence data are available and most survival data are limited to specialist clinics. METHODS Amyloidosis patients were identified from the Swedish Hospital Discharge and Outpatients Registers from years 2001 through 2008. RESU...

Introduction. Secondary renal amyloidosis due to tuberculosis is a debilitating disease with considerable mortality and morbidity due to renal failure and other manifestations of both amyloidosis and renal failure. Most patients with amyloidosis have been adequately treated with DOTS (Directly observed treatment, Short Course strategy). The aimof our study was to analyze the epidemiological and...

Respiratory involvement with amyloidosis typically involves the tracheobronchial tree or lung parenchyma. We describe a patient with systemic amyloidosis who was respirator-dependent because of extensive amyloid infiltration of the diaphragm, with no evidence of other pulmonary amyloidosis. Diaphragmatic myopathy from amyloid should be considered in respiratory failure in amyloidosis.

OBJECTIVE Phenotype II in familial Mediterranean fever (FMF) is the onset of amyloidosis before the onset of FMF with its typical attacks, or as an isolated finding in a member of an FMF family. Its presence was investigated by looking for proteinuria among the asymptomatic relatives of patients with FMF complicated by amyloidosis and among the asymptomatic relatives of patients with juvenile c...