Renal involvement in systemic amyloidosis - An Italian retrospective study on epidemiological and clinical data at diagnosis

Franco Bergesio, Anna Maria Ciciani, Marisa Santostefano, Rachele Brugnano, Marco Manganaro, Giovanni Palladini, Anna Maria Di Palma, Marco Gallo, Pier Luigi Tosi, Maurizio Salvadori

Research output: Contribution to journalArticle

49 Citations (Scopus)

Abstract

Background. Few data are available on epidemiology and clinical picture of renal involvement in different forms of systemic amyloidosis. Methods. Patients with biopsy-proven systemic amyloidosis diagnosed in Italy between January 1995 and December 2000 were selected from 49 Nephrology and Internal Medicine Units provided they showed signs characteristic of renal involvement. Clinical and laboratory information were collected by using a specific data form for diagnosis integrated by a questionnaire on diagnostic tools. Collected data were matched both with the Italian Registry of Renal Biopsies (IRRB) and the Registry of the Italian Society of Amyloidosis (SIA) in order to approximate the incidence of the disease. Results. Of all patients, 373 were finally selected throughout Italy with an estimated mean incidence of renal amyloidosis of 2.1 per million population (p.m.p.) per year. Of those, 237 were affected from AL (primary) amyloidosis, 104 from AA (secondary) amyloidosis and 6 from AF (heredofamilial) forms. In 26 cases the type of amyloidosis remained undetermined. Among patients with AL, 36 presented an associated multiple myeloma (MM). Rheumatoid arthritis (RA) was the commonest underlying disease in AA. Median age ranged between 63 and 65 years in all groups. Males were prevalent in AL and females in AA. The main clinical features of renal involvement were represented by nephrotic syndrome and renal failure observed in 59 and 54% of cases, respectively. The presence of a lambda light chain, either in serum or urine was significantly associated to a more elevated urinary protein loss and to a reduced renal function. Patients with AA showed a worse renal function at presentation than patients with AL, possibly due to a late diagnosis and/or referral to nephrology units. Diagnosis was obtained by renal biopsy in 315 cases, by abdominal fat tissue (AFT) aspiration/biopsy in 156 patients and by other organ biopsies in 47 patients. Characterization of deposits was extremely variable among referring centres. Conclusions. Our results point to an increased incidence of renal amyloidosis observed in Italy over the period 1996-2000 with AL as the prevalent type. Characterization of amyloid deposits still remains the major diagnostic challenge of the disease. The institution of networks dedicated to rare diseases is strongly recommended in order to effectively afford this challenge.

Original languageEnglish
Pages (from-to)1608-1618
Number of pages11
JournalNephrology Dialysis Transplantation
Volume22
Issue number6
DOIs
Publication statusPublished - Jun 2007

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Amyloidosis
Retrospective Studies
Kidney
Italy
Biopsy
Nephrology
Registries
Incidence
Abdominal Fat
Delayed Diagnosis
Amyloid Plaques
Nephrotic Syndrome
Needle Biopsy
Internal Medicine
Rare Diseases
Multiple Myeloma
Renal Insufficiency
Rheumatoid Arthritis
Epidemiology
Referral and Consultation

Keywords

  • AA amyloidosis
  • Abdominal fat aspiration
  • AL amyloidosis
  • Epidemiology
  • Renal amyloidosis
  • Renal biopsy

ASJC Scopus subject areas

  • Nephrology
  • Transplantation

Cite this

Renal involvement in systemic amyloidosis - An Italian retrospective study on epidemiological and clinical data at diagnosis. / Bergesio, Franco; Ciciani, Anna Maria; Santostefano, Marisa; Brugnano, Rachele; Manganaro, Marco; Palladini, Giovanni; Palma, Anna Maria Di; Gallo, Marco; Tosi, Pier Luigi; Salvadori, Maurizio.

In: Nephrology Dialysis Transplantation, Vol. 22, No. 6, 06.2007, p. 1608-1618.

Research output: Contribution to journalArticle

Bergesio, F, Ciciani, AM, Santostefano, M, Brugnano, R, Manganaro, M, Palladini, G, Palma, AMD, Gallo, M, Tosi, PL & Salvadori, M 2007, 'Renal involvement in systemic amyloidosis - An Italian retrospective study on epidemiological and clinical data at diagnosis', Nephrology Dialysis Transplantation, vol. 22, no. 6, pp. 1608-1618. https://doi.org/10.1093/ndt/gfm041
Bergesio, Franco ; Ciciani, Anna Maria ; Santostefano, Marisa ; Brugnano, Rachele ; Manganaro, Marco ; Palladini, Giovanni ; Palma, Anna Maria Di ; Gallo, Marco ; Tosi, Pier Luigi ; Salvadori, Maurizio. / Renal involvement in systemic amyloidosis - An Italian retrospective study on epidemiological and clinical data at diagnosis. In: Nephrology Dialysis Transplantation. 2007 ; Vol. 22, No. 6. pp. 1608-1618.
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AU - Bergesio, Franco

AU - Ciciani, Anna Maria

AU - Santostefano, Marisa

AU - Brugnano, Rachele

AU - Manganaro, Marco

AU - Palladini, Giovanni

AU - Palma, Anna Maria Di

AU - Gallo, Marco

AU - Tosi, Pier Luigi

AU - Salvadori, Maurizio

PY - 2007/6

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N2 - Background. Few data are available on epidemiology and clinical picture of renal involvement in different forms of systemic amyloidosis. Methods. Patients with biopsy-proven systemic amyloidosis diagnosed in Italy between January 1995 and December 2000 were selected from 49 Nephrology and Internal Medicine Units provided they showed signs characteristic of renal involvement. Clinical and laboratory information were collected by using a specific data form for diagnosis integrated by a questionnaire on diagnostic tools. Collected data were matched both with the Italian Registry of Renal Biopsies (IRRB) and the Registry of the Italian Society of Amyloidosis (SIA) in order to approximate the incidence of the disease. Results. Of all patients, 373 were finally selected throughout Italy with an estimated mean incidence of renal amyloidosis of 2.1 per million population (p.m.p.) per year. Of those, 237 were affected from AL (primary) amyloidosis, 104 from AA (secondary) amyloidosis and 6 from AF (heredofamilial) forms. In 26 cases the type of amyloidosis remained undetermined. Among patients with AL, 36 presented an associated multiple myeloma (MM). Rheumatoid arthritis (RA) was the commonest underlying disease in AA. Median age ranged between 63 and 65 years in all groups. Males were prevalent in AL and females in AA. The main clinical features of renal involvement were represented by nephrotic syndrome and renal failure observed in 59 and 54% of cases, respectively. The presence of a lambda light chain, either in serum or urine was significantly associated to a more elevated urinary protein loss and to a reduced renal function. Patients with AA showed a worse renal function at presentation than patients with AL, possibly due to a late diagnosis and/or referral to nephrology units. Diagnosis was obtained by renal biopsy in 315 cases, by abdominal fat tissue (AFT) aspiration/biopsy in 156 patients and by other organ biopsies in 47 patients. Characterization of deposits was extremely variable among referring centres. Conclusions. Our results point to an increased incidence of renal amyloidosis observed in Italy over the period 1996-2000 with AL as the prevalent type. Characterization of amyloid deposits still remains the major diagnostic challenge of the disease. The institution of networks dedicated to rare diseases is strongly recommended in order to effectively afford this challenge.

AB - Background. Few data are available on epidemiology and clinical picture of renal involvement in different forms of systemic amyloidosis. Methods. Patients with biopsy-proven systemic amyloidosis diagnosed in Italy between January 1995 and December 2000 were selected from 49 Nephrology and Internal Medicine Units provided they showed signs characteristic of renal involvement. Clinical and laboratory information were collected by using a specific data form for diagnosis integrated by a questionnaire on diagnostic tools. Collected data were matched both with the Italian Registry of Renal Biopsies (IRRB) and the Registry of the Italian Society of Amyloidosis (SIA) in order to approximate the incidence of the disease. Results. Of all patients, 373 were finally selected throughout Italy with an estimated mean incidence of renal amyloidosis of 2.1 per million population (p.m.p.) per year. Of those, 237 were affected from AL (primary) amyloidosis, 104 from AA (secondary) amyloidosis and 6 from AF (heredofamilial) forms. In 26 cases the type of amyloidosis remained undetermined. Among patients with AL, 36 presented an associated multiple myeloma (MM). Rheumatoid arthritis (RA) was the commonest underlying disease in AA. Median age ranged between 63 and 65 years in all groups. Males were prevalent in AL and females in AA. The main clinical features of renal involvement were represented by nephrotic syndrome and renal failure observed in 59 and 54% of cases, respectively. The presence of a lambda light chain, either in serum or urine was significantly associated to a more elevated urinary protein loss and to a reduced renal function. Patients with AA showed a worse renal function at presentation than patients with AL, possibly due to a late diagnosis and/or referral to nephrology units. Diagnosis was obtained by renal biopsy in 315 cases, by abdominal fat tissue (AFT) aspiration/biopsy in 156 patients and by other organ biopsies in 47 patients. Characterization of deposits was extremely variable among referring centres. Conclusions. Our results point to an increased incidence of renal amyloidosis observed in Italy over the period 1996-2000 with AL as the prevalent type. Characterization of amyloid deposits still remains the major diagnostic challenge of the disease. The institution of networks dedicated to rare diseases is strongly recommended in order to effectively afford this challenge.

KW - AA amyloidosis

KW - Abdominal fat aspiration

KW - AL amyloidosis

KW - Epidemiology

KW - Renal amyloidosis

KW - Renal biopsy

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