Tubulointerstitial nephritis is a dominant feature of hereditary apolipoprotein A-I amyloidosis

Renal apolipoprotein A-I amyloidosis: a rare and usually ignored cause of hereditary tubulointerstitial nephritis.

Apolipoprotein A-I amyloidosis is a rare, late-onset, autosomal dominant condition characterized by systemic deposition of amyloid in tissues, the major clinical problems being related to renal, hepatic, and cardiac involvement. Described is the clinical and histologic picture of renal involvement as a result of apolipoprotein A-I amyloidosis in five families of Italian ancestry. In all of the ...

Vimentin is a dominant target of in situ humoral immunity in human lupus tubulointerstitial nephritis

OBJECTIVE In lupus nephritis (LN), severe tubulointerstitial inflammation (TII) predicts progression to renal failure. Severe TII is associated with tertiary lymphoid neogenesis and in situ antigen-driven clonal B cell selection. The autoantigen(s) driving in situ B cell selection in TII are not known. This study was undertaken to identify the dominant driving autoantigen(s). METHODS Single C...

D25V apolipoprotein C-III variant causes dominant hereditary systemic amyloidosis and confers cardiovascular protective lipoprotein profile

Apolipoprotein C-III deficiency provides cardiovascular protection, but apolipoprotein C-III is not known to be associated with human amyloidosis. Here we report a form of amyloidosis characterized by renal insufficiency caused by a new apolipoprotein C-III variant, D25V. Despite their uremic state, the D25V-carriers exhibit low triglyceride (TG) and apolipoprotein C-III levels, and low very-lo...

karyomegalic tubulointerstitial nephritis: a rare cause of chronic kidney disease

Tubulointerstitial lupus nephritis.

BACKGROUND Isolated or predominant tubulointerstitial lupus nephritis is rare. CASE PRESENTATION Here we report the case of a thirty eight years old male who was diagnosed with systemic lupus erythematosus (SLE) according to clinical and laboratory criteria and presented with impaired renal function and non nephrotic range proteinuria. Renal biopsy revealed normal glomeruli but interstitial m...