Steroid-induced scleroderma renal crisis in an at-risk patient.

Scleroderma renal crisis (SRC) is a major complication of systemic sclerosis (SSc). We present a case of a patient who died of complications attributable to SRC in the context of recently diagnosed SSc. We review the typical clinical and laboratory features of SSc present in our patient and highlight those that identify greater risk of progression to SRC.

Amyloid load and clinical outcome in AA amyloidosis in relation to circulating concentration of serum amyloid A protein.

Background: Reactive systemic (AA, secondary) amyloidosis occurs in chronic inflammatory diseases, and most patients present with nephropathy. The amyloid fibrils are derived from the circulating acute-phase reactant serum amyloid A protein (SAA), but the relation between production of fibril precursor protein, amyloid load, and clinical outcome in AA and other types of amyloidosis is unclear.

Methods: We studied amyloidotic organ function and survival prospectively for 12-117 months in 80 patients with systemic AA amyloidosis in whom serum SAA concentration was measured monthly and visceral amyloid deposits were assessed annually by serum amyloid P component scintigraphy.

Transthyretin Ile73Val is associated with familial amyloidotic polyneuropathy in a Bangladeshi family. Mutations in brief no. 158. Online.

Amyloidosis is characterised by the extraceullular deposition of certain different proteins in a distinctively abnormal fibrillar conformation. All types of amyloid fibril share remarkably similar structural and biophysical properties despite substantial chemical heterogeneity among their respective precursor proteins. Hereditary amyloidosis associated with genetically determined protein variants is rare, but is extremely important as a model for studying the pathogenesis of amyloidosis generally.