Rapidly progressive glomerulonephritis complicating primary AL amyloidosis and multiple myeloma

Abstract

Crescentic glomerulonephritis is a rare complication of AA amyloidosis. There are no clinical case reports of this complicating AL amyloidosis. A 67-year-old man developed rapidly progressive glomerulonephritis (RPGN) on a background of primary AL amyloidosis and IgGkappa multiple myeloma. Investigations for causes of glomerulonephritis were negative, and a renal biopsy confirmed crescentic glomerulonephritis and amyloid deposition. He progressed to end stage kidney disease (ESKD) requiring dialysis after 2 months and died 7 months after diagnosis after further treatment of multiple myeloma failed to arrest progression. We believe this to be the first clinical case report of RPGN complicating primary (AL) renal amyloidosis and multiple myeloma.

Atypical case of AL amyloidosis with urinary erythrocyte casts

“…However, and in contrast to the cases reported by Ogami and Crosthwaite, we found no evidence of glomerular segmental necrosis or cellular crescent formation [17, 18 ]. In contrast, the glomerular pathology in our case was limited to extensive mesangial and glomerular basement membrane amyloid depositions.…”

“…In contrast, the glomerular pathology in our case was limited to extensive mesangial and glomerular basement membrane amyloid depositions. In addition, our case was associated with λ-light chains, in comparison to κ-light chains in 1 of the previously published cases of AL amyloidosis [17]. Regarding bone marrow pathology, both patients had evidence of pathological infiltration with monoclonal lymphoplasmacytoid cells, whereby infiltration was less pronounced in our case (10 – 15%) in comparison to the case reported previously by Crosthwaite (38%) [17] (key features of these 3 cases are summarized in Table 1).…”

“…Two other cases of renal AL amyloidosis in association with a nephritic urinary sediment and extracapillary glomerular proliferation on renal biopsy have been reported [17, 18]. In the first report, rapidly progressive deterioration of renal function and glomerular microhematuria developed in a patient with a 4-year history of primary κ-type AL amyloidosis (without lymphoplasmacytoid bone marrow infiltration) and previously biopsy-proven myocardial involvement, who later developed multiple myeloma as evidenced by bone marrow infiltration with 38% plasma cells, a few months prior to developing renal failure.…”

“…In the first report, rapidly progressive deterioration of renal function and glomerular microhematuria developed in a patient with a 4-year history of primary κ-type AL amyloidosis (without lymphoplasmacytoid bone marrow infiltration) and previously biopsy-proven myocardial involvement, who later developed multiple myeloma as evidenced by bone marrow infiltration with 38% plasma cells, a few months prior to developing renal failure. Renal biopsy showed an acute segmental necrosis and cellular crescent formation as well as renal amyloidosis and tubulo-interstitial injury [17]. The second report described a case of AL amyloidosis and Waldenström’s macroglobulinemia in which a nephritic urinary sediment and renal failure developed.…”

Rapidly Progressive Glomerulonephritis

“…Very recently, a report has shown for the first time the appearance of RPGN complicating the course of AL amyloidosis (Crosthwaite, 2010). Cases of association of primary or secondary glomerulonephritis and anti-GBM disease or renal vasculitis have also been published, as well as cases of association of anti-GBM disease and ANCApositive renal vasculitis (Curioni, 2002;O'Connor, 2010).…”