membranous glomerulonephritis and .solange moll abstract membranous glomerulonephritis and crescents
Post on 14-Sep-2018
Embed Size (px)
Membranous glomerulonephritis and crescents
A 45-year-old Swiss female was diagnosed in 1992 for a seropositive rheumatoid arthritis and
treated with different regimens including NSAIDs, gold therapy, methotrexate and anti-TNF
antibodies. Since 2006, she decided to take only homeopathic therapy. On August 2009, she
was admitted to our hospital with complaints of asthenia, legs swelling and increased body
weight (6 kg in 3 months). Physical examination revealed edema of the lower extremities and
hypertension (160/90mmHg). Initial laboratory data showed high CRP value (62mg/l),
anemia (hemoglobin: 93g/l) and rapidly progressive renal failure (serum creatinine:
313mol/l on admission, 500mol/l ten days later). Proteinuria (6.3g/l) with glomerular
microhematuria and pyuria was detected on urinalysis. Other laboratory data revealed positive
antinuclear antibody (1/320) and pANCA (1/640, MPO) tests. A renal biopsy was performed,
which consisted of two cores containing 10 glomerules. Three of them showed foci of
fibrinoid necrosis with GBM rupture and extracapillary fibrin, and seven showed cellular
crescents. A diffuse moderate-to-severe interstitial inflammation composed of a mixed
infiltrate of lymphocytes, histiocytes and plasmocytes was observed. Immunofluorescence
revealed granular, global glomerular capillary wall positivity for IgG and weaker staining for
C3 and C5b-9. Electron microscopy showed subepithelial electron dense deposits, often
accompanied by GBM spikes and overlying neomembrane formation. The diagnosis of
membranous glomerulonephritis stage I-II and ANCA-associated necrotizing and crescentic
glomerulonephritis was done. Methylprednisolone and cyclophosphamide iv therapy was
begun, in association with plasmapheresis and transient hemodialysis. Treatment led to rapid
improvement in renal function (serum creatinine:120mol/l at one month) and to a significant
decline in the degree of proteinuria. Mycophenolate mofetyl was substituted for
cyclophospamide and doses of prednisone were progressively reduced. Renal function was
stable during the next following 6 months.
Only rare cases of concurrent membranous glomerulonephritis (MGN) and antineutrophil
cytoplasmic antibody (ANCA)-associated necrotizing and crescentic glomerulonephritis
(NCGN) have been reported in the literature (1). Recently, Snar et al. reported the clinical and
pathologic findings in 14 patients with MGN and ANCA-associated NCGN (2). Clinical
presentation included heavy proteinuria (mean 24-hr urine protein 6.5 g/d), hematuria, and
acute renal failure (mean creatinine 400mol/l), as observed in our case. Pathologic
evaluation revealed MGN and NCGN, with crescents involving a mean of 32% of glomeruli.
Follow-up (mean of 24.3 months) was available for 13 patients, 12 of whom were treated with
steroids and cyclophosphamide. Five patients progressed to ESRD, seven had stabilization or
improvement in renal function, and one had worsening renal function. The only independent
predictor of progression to ESRD was serum creatinine at biopsy.
In conclusion, MGN with ANCA-associated NCGN is a rare dual glomerulopathy, and
prognosis is variable.
1. Tse WY, Howie AJ, Adu D, Savage CO, Richards NT, Wheeler DC, Michael J:
Association of vasculitic glomerulonephritis with membranous nephropathy: A report of 10
cases. Nephrol Dial Transplant 1997 12:1017 1027.
2. Nasr SH, Said SM, Valeri AM, Stokes MB, Masani NN, D'Agati VD, Markowitz GS.
Membranous glomerulonephritis with ANCA-associated necrotizing and crescentic
glomerulonephritis. Clin J Am Soc Nephrol. 2009 Feb;4(2):299-308
7 croissants cellulaires
Abstract S_Moll_La Coruna 2010.pdfLa Coruna S_Moll.pdf