A Proliferative Glomerulonephritis Secondary to a Monoclonal IgA (original) (raw)
Related papers
American Journal of Kidney Diseases, 2011
Proliferative glomerulonephritis with monoclonal immunoglobulin G (IgG) deposits (PGNMIGD) is a recently recognized glomerular disease. Light microscopy usually resembles membranoproliferative glomerulonephritis. Glomerular deposits are mostly IgG3 ; however, unlike in the usual forms of monoclonal immunoglobulin deposition disease, extraglomerular deposits are absent. If PGNMIGD is secondary to the glomerular deposition of circulating monoclonal IgG, it is expected to recur in kidney allografts with the same pattern of monoclonal IgG deposition. We reviewed our kidney biopsy files between January 1, 2003, and January 4, 2010, and identified 21 biopsy specimens with PGNMIGD, mostly with glomerular IgG3 deposits. Of the 21 biopsy specimens, 4 were from kidney allografts; 2 were recurrent and the other 2 were de novo diseases. Recurrent PGNMIGD develops rapidly, causing proteinuria. This rapid recurrence of PGNMIGD in kidney allografts provides further proof that PGNMIGD is secondary to the glomerular deposition of circulating monoclonal IgG. Am J Kidney Dis. 58 :276-281.
NDT Plus, 2010
A 78-year-old woman developed acute-onset nephrotic syndrome. A renal biopsy showed mild mesangial proliferative glomerulonephritis. Immunofluorescence studies revealed granular IgG3-λ deposits within the mesangial area and along the glomerular capillary walls. Electron microscopy showed mesangial and subendothelial granular electron-dense deposits. The pattern of deposition was predominantly mesangial. Serum or urine monoclonal proteins were not detected. Middle-dose steroid therapy induced a rapid remission of nephrotic syndrome. We consider that this is the first case of steroid-responsive nephrotic syndrome due to an extremely rare glomerular disease, proliferative glomerulonephritis with monoclonal IgG deposits associated with pure mesangial proliferative features.
Human pathology, 2003
Morphological examination of 2 renal biopsy specimens obtained from a 69-year-old woman with a nephrotic syndrome, high blood pressure, and a reduced glomerular filtration rate revealed, in ultrastructural study, a type of a glomerulonephritis with fibrillar deposits in a subendothelial position which were unusual in their immunoglobulin components (mainly IgM). The fibrillar components were of irregular size, 13 to 18 nm in diameter and presented a very particular "barbed wire" morphological aspect, not hitherto described. Diffraction studies and image analysis, revealed spiraled fibrils with regular alternating elements that we suggest may correspond to IgM molecules. The clinical (isolated renal symptoms) and laboratory (traces of 3 monoclonal components in the serum and 2 normal bone marrow biopsy specimens) data provided no evidence of hematopoietic malignancy, viral hepatitis or cryoglobulinemia.
Mayo Clinic proceedings, 2015
To study the hematologic characteristics of proliferative glomerulonephritides (GNs) from nonorganized glomerular monoclonal immunoglobulin (MIg) deposition (MIPG). The pathology database at Mayo Clinic (Rochester, Minnesota) was used to find patients with MIPG who underwent a kidney biopsy between January 1, 2008, and December 31, 2013. Retrospective medical record review was conducted in the identified cohort (N=60). The median patient age was 56 years (interquartile range, 47-62 years) and the estimated glomerular filtration rate was 36 mL/min/1.73 m(2) (interquartile range, 22-52 mL/min/1.73 m(2)). Most patients had IgG MIg deposits (90%; 54 of 60) and a membranoproliferative pattern (48%; 29 of 60). A circulating nephropathic MIg was detected by serum immunofixation (SIFE(+)) in 20% (12 of 59) and by abnormal serum free light chain ratio (sFLCR(+)) in 21% (12 of 56). The subsets of SIFE(+) and sFLCR(+) incompletely overlapped. The nephropathic clone was found by bone marrow tes...
Mesangial IgA deposits indicate pathogenesis of anti-glomerular basement membrane disease
Molecular medicine reports, 2012
Anti-glomerular basement membrane (anti-GBM) disease is characterized by crescentic glomerulonephritis with immunoglobulin G (IgG) autoantibodies to the non-collagenous (NC1) domain of α3(IV) collagen presenting along the GBM. The patient clinically manifests with rapidly progressive glomerulonephritis (RPGN) with pulmonary hemorrhage (Goodpasture syndrome). In rare cases, other immunocomplexes of IgA or IgM are involved, but their specificities have not been determined. We report a rare case of a 31-year-old female who was diagnosed as having anti-GBM disease with extensive IgA deposits in the mesangium. This patient presented heavy hematuria, proteinuria with increasing creatinine, but no lung hemorrhage. Renal biopsy showed crescentic glomerulonephritis (type Ⅰ) with strong IgA (3+) as lump and branch shape. Therapies with pulse methylprednisolone, plasmapheresis and cyclophosphamide administration were less effective. This case is different from the present type Ⅰ crescentic glo...
ANCA-associated crescentic glomerulonephritis with mesangial IgA deposits
American Journal of Kidney Diseases, 2000
Antineutrophil cytoplasmic autoantibodies (ANCA) are commonly associated with a necrotizing and crescentic glomerulonephritis (GN) that is pauci-immune, with few or no glomerular immune complex deposits detectable by immunofluorescence (IF) or electron microscopy (EM). Immunoglobulin A (IgA) nephropathy may also be manifest as a crescentic GN, but it is characterized by mesangial immune complex deposits containing IgA and is rarely associated with myeloperoxidase (MPO)-or proteinase 3 (PR3)-specific ANCA when an enzyme immunoassay is used to detect these antibodies. This report describes six patients with severe crescentic GN with mesangial IgA deposits by IF and mesangial electron-dense deposits by EM in patients with positive ANCA serological test results (four patients, anti-PR3; one patient, anti-MPO; one patient, anti-PR3 and anti-MPO). Patients presented with acute or progressive renal insufficiency, hematuria, proteinuria (nephrotic range in two patients), and hypertension. Three patients had evidence of systemic vasculitis: two patients at initial presentation and one patient later in the clinical course. Renal biopsy specimens showed crescents in greater than 50% of glomeruli in all cases, but only mild, focal and segmental mesangial and endocapillary hypercellularity, more typical of ANCA-associated crescentic GN than of crescentic IgA nephropathy without associated ANCA. Semiquantitative analysis of mesangial and endocapillary cellularity performed on renal biopsy slides from these six patients and from eight ANCA-negative patients with IgA nephropathy and crescents in greater than 50% of glomeruli showed significantly greater hypercellularity in the ANCA-negative cases. Three of five ANCA-positive patients for whom follow-up clinical data were available showed improved renal function after treatment with cyclophosphamide and corticosteroids and have not developed end-stage renal disease 17, 20, and 25 months postbiopsy. The remaining two patients were dialysis dependent at the time of biopsy and have remained so despite treatment with cyclophosphamide and corticosteroids. The findings suggest an overlap syndrome of ANCA-associated crescentic GN and IgA nephropathy that resembles the former both histologically and in its potential to respond to aggressive therapy if detected relatively early in its course.
Immunopathologia Persa, 2016
Anti-neutrophil cytoplasmic antibody (ANCA)-associated crescentic glomerulonephritis is an important cause for rapidly progressing glomerulonephritis. It is generally classified under pauci-immune glomerulonephritis. However, 12%-18% of ANCA-associated crescentic glomerulonephritis show immune-complex deposits causing a diagnostic dilemma. We report 2 cases of ANCA-mediated glomerulonephritis with associated immune-complex deposits. First case is a 19-year-old female patient presented with fever and bilateral lower limb purpura since one day. Immunologic work-up was normal except positivity for cytoplasmic or c-ANCA by indirect immunofluorescence (IF). Kidney biopsy showed presence of segmental cellular crescent with fibrinoid necrosis. IF showed strong fine granular positivity for IgG, IgA, C3, C1q, kappa and lambda along the glomerular capillary walls. Second case is a 20-year-old male presented with low grade fever for last one month and vomiting for last two days. Immunologic work-up was unremarkable except positivity for cytoplasmic or c-ANCA by indirect IF. Kidney biopsy showed 14 glomeruli of which 8 glomeruli showed cellular crescents. IF for IgG, IgA, C3, kappa and lambda was done, which showed strong fine granular positivity along the glomerular capillary walls. Both the cases were treated with intravenous methylprednisolone and oral prednisone on a weaning regimen, and monthly therapy of intravenous cyclophosphamide. The maintenance phase consisted of mycophenolate mofetil and oral prednisone administrated in alternate-day low-dose regimen. Both the patients are on regular follow-up and are doing well.These immunecomplexes act synergistically with ANCA to cause more severe damage to the kidneys with a poorer outcome. Thus a prompt diagnosis and management of these patients is crucial.