membranous glomerulonephritis and .solange moll abstract membranous glomerulonephritis and crescents

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  • Solange Moll

    Abstract

    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.

    References

    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

  • IgG

  • Abstract S_Moll_La Coruna 2010.pdfLa Coruna S_Moll.pdf

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