Myeloperoxidase-antineutrophil cytoplasmic antibody-associated crescentic glomerulonephritis in autosomal dominant polycystic kidney disease

Abstract

BackgroundAutosomal dominant polycystic kidney disease (ADPKD) is an inherited disorder that is characterized by the development of cysts in the kidneys and other organs. Urinary protein excretion is usually less than 1 g/day, and ADPKD is rarely associated with nephrotic syndrome or rapidly progressive glomerulonephritis (RPGN). To date, myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA)-associated crescentic glomerulonephritis (CrGN) has not been reported in a patient with ADPKD.Case presentationsWe report two cases of MPO-ANCA positive ADPKD. A 60-year-old Japanese woman (case 1) and a 54-year-old Japanese woman (case 2) presented with RPGN featuring severe proteinuria and microscopic hematuria. In both patients percutaneous needle biopsy of the kidney revealed MPO-ANCA-associated CrGN with a paucity of glomerular immunoglobulin staining. Each patient received intravenous methylprednisolone for 3 days, followed by oral prednisolone. Case 1 showed gradual improvement and has not progressed to end-stage renal disease (ESRD), but case 2 developed ESRD requiring hemodialysis within one month despite treatment.ConclusionThese are the first two reported cases of MPO-ANCA-associated CrGN in patients with ADPKD. Our experience suggests that serial measurement of the ANCA titer and renal biopsy should be considered for accurate diagnosis and appropriate treatment of ADPKD patients who present with proteinuria, hematuria, and rapid decline of renal function.

PKD1-associated autosomal dominant polycystic kidney disease with glomerular cysts presenting with nephrotic syndrome caused by focal segmental glomerulosclerosis

“…Visciano et al listed 29 reported cases of ADPKD with nephrotic syndrome evaluated by renal histopathological studies and illustrated that FSGS was the most common cause of nephrotic syndrome (6 out of 29 cases) [2]. A literature review by Sumida et al observed 19 cases of ADPKD with nephrotic syndrome, whose leading cause was FSGS (4 out of 19 cases) manifesting urine protein excretion of 5.8 to 14 g/day [3]. However, due to the rarity of the disease and limited number of reports, histopathology of ADPKD complicated with nephrotic syndrome has not been entirely investigated.…”

Autosomal dominant polycystic kidney disease combined with hypertrophic cardiomyopathy

“…Usually in this disorder, urinary protein excretion is less than 1 g/24 hour, while nephrotic syndrome is considered to be rare. [ 5 ] The histopathological lesions reported are variously covering focal segmental glomerulosclerosis, [ 6 ] membranous nephropathy, [ 7 ] minimal change disease, [ 8 ] crescentic glomerulonephritis, [ 9 ] immunoglobulin A nephropathy, [ 10 ] amyloidosis, [ 11 ] and mesengioproliferative glomerulonephritis. [ 12 ] It proved to be invalid of hormone empirical therapy for the patient with no sign of proteinuria reducing.…”

Rapidly Progressive Glomerulonephritis in Autosomal Dominant Polycystic Kidney Disease

“…All cases were characterized by nephrotic range proteinuria, hematuria and acute kidney injury indicated by the rapid decline in kidney function. In the most recently reported cases (38), both patients had ANCA-associated crescentic glomerulonephritis without evidence of vasculitis. In the case we presented in this review, the cause of acute kidney injury and nephrotic syndrome was attributed to crescentic glomerulonephritis rather that the patient's underlying polycystic kidney disease.…”