Humans ; Immunoglobulin Light Chains ; blood ; chemistry ; immunology ; Kidney ; immunology ; pathology ; Paraproteinemias ; immunology

Humans ; Immunoglobulin Light Chains ; genetics ; immunology ; Immunoglobulin kappa-Chains ; genetics ; immunology ; Kidney ; immunology ; metabolism ; pathology ; Kidney Diseases ; immunology ; pathology ; therapy ; Mutation ; Transforming Growth Factor beta ; metabolism

OBJECTIVETo explore possible correlations between renal Th1/Th2 ratio and renal microvascular injury in children with Henoch-Sch-nlein purpura nephritis (HSPN).

METHODSThirty-two children with HSPN were enrolled. They were classified into four groups by renal pathology: HSPN class II (n=8), HSPN class IIIa (n=7), HSPN class IIIb (n=10) and HSPN class IV/V (n=7). Five patients undergoing nephrectomy due to trauma were used as the controls. INFγ, IL-4 and CD34 in the renal tissues were measured by immunohistochemical analysis. INFγ was used as a marker of Th1, IL-4 was used as a marker of Th2 and CD34 was used as a marker of microvessel. The renal microvessel density was evaluated according to the Weidner standard. The relationships among the local Th1/Th2 ratio, renal pathological grade, microvessel score and microvessel density were studied.

RESULTSImmunohistochemical analysis showed a lower expression of INFγ and a higher expression of IL-4 in the HSPN groups than in the control group. The local Th1/Th2 ratio in the HSPN groups decreased and correlated significantly with the renal pathological grade. There were significant differences among four HSPN subgroups (P<0.05). Compared with the control group, the renal microvessel density in the HSPN class II and class IIIa groups increased significantly (P<0.05), but it decreased in the HSPN class IV/V group (P<0.05). The renal microvessel scores in the HSPN class IIIa, class IIIb and class IV/V groups increased significantly compared with those in the control and the HSPN classⅡ. The increased renal microvessel scores were associated with more severe renal pathological changes. A negative correlation was found between the local Th1/Th2 ratio and the microvessel density in kidneys (r=-0.921, P<0.01).

CONCLUSIONSThe decrease of Th1/Th2 ratio in kidneys might be responsible for renal microvascular injury in children with HSPN.

Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Kidney ; blood supply ; pathology ; Male ; Microvessels ; pathology ; Nephritis ; immunology ; pathology ; Purpura, Schoenlein-Henoch ; immunology ; pathology ; Th1 Cells ; immunology ; Th2 Cells ; immunology

OBJECTIVETo investigate the pathologic features and diagnostic algorithm of light chain nephropathy (LCN).

METHODSSeven cases of LCN were studied by light microscopy, electron microscopy and immunolabeling of light chains (kappa, lambda) by immunofluorescence and immunoelectron microscopy.

RESULTSThe histopathology of 7 cases by light microscopy was variable, with 3 cases showing nodular glomerulosclerosis, 1 case showing mild to moderate mesangial proliferation, and 3 cases showing cast nephropathy with minimal glomerular change. Immunofluorescence study revealed positive staining of a single type of light chain in mesangium (nodular pattern) or along glomerular basement membrane (linear), along tubular basement membrane and around arteriolar walls in all the 7 cases. Ultrastructurally, electron-dense granular deposits were identified in mesangium, subendothelial aspect of glomerular basement membrane, outer aspect of tubular basement membrane and arteriolar walls. Immunogold labeling of light chains showed distinct labeling of a single type light chain in the granular electron-dense materials (5 cases being kappa-positive and 2 being lambda-positive).

CONCLUSIONSLCN typically shows nodular glomerulosclerosis. The ultrastructural change is characteristic and important for diagnosis. Immunolabeling of light chains by immunofluorescence and immunoelectron microscopy carries further diagnostic value, especially in cases with minimal light microscopic change.

Adult ; Aged ; Female ; Glomerulosclerosis, Focal Segmental ; immunology ; pathology ; Humans ; Immunoglobulin Light Chains ; immunology ; Immunoglobulin kappa-Chains ; immunology ; Immunoglobulin lambda-Chains ; immunology ; Kidney Diseases ; immunology ; pathology ; Kidney Glomerulus ; immunology ; pathology ; ultrastructure ; Male ; Microscopy, Fluorescence ; Microscopy, Immunoelectron ; Middle Aged

OBJECTIVETo study the clinicopathologic characteristics of IgG4-related disease in the orbital and periorbital tissue.

METHODSThe clinical manifestations and pathologic features of 17 cases of IgG4-related disease affecting the orbital and periorbital tissue encountered during the period from 2012 to 2013 were studied.

RESULTSThere were 9 male patients and 8 female patients. The age of patients ranged from 11 to 71 years (mean = 48.5 years). The main clinical manifestation was swelling of the eyelids: bilateral in 11 patients and unilateral in 6 patients. The duration of disease ranged from 5 months to 7 years (more than 2 years in 13 cases). Six patients had history of allergic disorders. In addition to orbital/periorbital involvement, the disease also affected salivary gland, lymph node, lung and kidney. The disease relapsed in 9 patients. Amongst the 8 patients treated with steroids, 5 of them achieved complete remission and the remaining 3 patients had partial remission. The IgG4 level of the 17 cases ranged from 1.49 to 14.88 g/L. Histologic examination showed pseudolymphoma pattern in 8 cases, mixed pattern in 8 cases and sclerotic pattern in 1 case. There were various degrees of lymphoplasmacytic infiltrates (with lymphoid follicle formation) and stromal fibrosis. Classical obliterative phlebitis was absent. Tissue eosinophilia was demonstrated in the 17 cases studied. Immunohistochemical study showed the presence of more than 50 IgG4-positive plasma cells per high-power field, with IgG4/IgG plasma cells ratio more than 40%.

CONCLUSIONThere are characteristic clinical manifestations, pathologic features and laboratory findings in orbital and periorbital IgG4-related disease. Thorough understanding is important in arriving at a correct diagnosis.

Adult ; Aged ; Autoimmune Diseases ; immunology ; pathology ; Child ; Female ; Humans ; Immunoglobulin G ; Kidney Diseases ; immunology ; pathology ; Lung Diseases ; immunology ; pathology ; Lymphatic Diseases ; immunology ; pathology ; Male ; Middle Aged ; Orbital Diseases ; immunology ; pathology ; Plasma Cells ; immunology

We report a case of glomerular disease with both mesangial IgA and subepithelial IgG deposits in the allograft kidney. The patient was a 36 year-old man who had received a renal allograft 1 year previously. Fifteen days before admission, he discovered a microscopic hematuria without clinical evidences of allograft rejection. Light microscopy showed diffuse increase of mesangial matrix without mesangial cell proliferation. Capillary walls were diffusely and mildly thickened. Immunofluorescence microscopy demonstrated both granular deposits of IgA in the mesangium and IgG along the capillary walls. On electron microscopy, electron-dense deposits were identified not only in the mesangium but also on the epithelial side of the glomerular basement membrane.
Adult ; Case Report ; Glomerulonephritis, IGA/*immunology/pathology ; Human ; Immunoglobulin A/*analysis ; Immunoglobulin G/analysis ; Kidney/*immunology/pathology ; Kidney Transplantation/*immunology ; Male ; Transplantation, Homologous

OBJECTIVETo study the difference in clinico-pathological features between IgA nephropathy (IgAN) and Henoch-Schonlein purpura nephritis (HSPN) in children.

METHODSThe medical data of 103 children with HSPN and 61 children with IgAN were retrospectively studied.

RESULTSThere were no significant differences in age, sex and disease course between the HSPN and IgAN groups (P>0.05). Clinical classification demonstrated that more severe conditions were found in the IgAN group than in the HSPN group and gross hematuria was more common in the IgAN group (P<0.05). Serum creatinine and cholesterol levels were higher in the IgAN group than in the HSPN group (P<0.05). Fibrinogen-related antigen deposition was more common in the HSPN group, while complement 3(C3) deposition was more common in the IgAN group. Interstitial fibrosis, tubular casts and tubular inflammatory infiltration were also more common in the IgAN group (P<0.05).

CONCLUSIONSSignificant clinico-pathological differences can be found between HSPN and IgAN in children, and these differences do not support a one disease entity hypothesis.

Child ; Child, Preschool ; Female ; Glomerulonephritis, IGA ; immunology ; pathology ; Humans ; Kidney ; pathology ; Male ; Nephritis ; immunology ; pathology ; Purpura, Schoenlein-Henoch ; immunology ; pathology ; Retrospective Studies

OBJECTIVETo study the clinicopathologic features of membranous nephropathy coexisting with IgA nephropathy.

METHODSThe renal biopsies performed in Peking University First Hospital during the period from January, 1998 to April, 2006 were retrospectively reviewed. The clinicopathologic features of 11 cases of membranous nephropathy coexisting with IgA nephropathy were studied. Electron microscopy with immunogold labeling for IgG and IgA were also performed.

RESULTSThe mean age of patients was 39.9 years. The male-to-female ratio was 1:2.9. The patients mainly presented with proteinuria. Proteinuria of nephrotic level was seen in 7 cases (63.6%). Seven cases also had associated microscopic hematuria. None of them showed evidence of renal insufficiency. Cases with secondary diseases, such as hepatitis virus infection and systemic lupus erythematosus, were excluded from the study. Histologically, vacuolation and thickening of glomerular basement membrane was seen. There was also mild mesangial hypercellularity and increase in mesangial matrix. Occasional glomeruli with crescent formation were identified in 2 cases. Immunofluorescence study showed granular staining for IgG and C3 along glomerular capillary walls, in addition to clumps of IgA deposits in mesangium. Electron microscopy revealed subepithelial and mesangial electron-dense deposits. Immunogold labeling showed IgG and IgA localized in the subepithelial and mesangial deposits respectively.

CONCLUSIONMembranous nephropathy coexisting with IgA nephropathy possesses the clinicopathologic features of both components. It might be caused by independent occurrence of the two entities.

Adult ; Female ; Glomerular Basement Membrane ; immunology ; pathology ; ultrastructure ; Glomerular Mesangium ; immunology ; pathology ; ultrastructure ; Glomerulonephritis, IGA ; complications ; immunology ; pathology ; Glomerulonephritis, Membranous ; complications ; immunology ; pathology ; Humans ; Immunoglobulin A ; metabolism ; Immunoglobulin G ; metabolism ; Kidney Glomerulus ; immunology ; pathology ; ultrastructure ; Male ; Middle Aged ; Retrospective Studies

OBJECTIVETo investigate the pathological and immunological changes of renal grafts in recipients experiencing graft rejection.

METHODSThe clinicopathologic data of 56 renal needle biopsy samples obtained from renal transplant recipients were analyzed retrospectively. The specimens were classified histopathologically according to the Banff 2009 classification system and analyzed by immunohistochemical labeling and immunofluorescence.

RESULTSIn the 56 recipients, 1 (1.79%) experienced hyperacute rejection, 8 (14.29%) had suspected acute rejection, 12 (21.43%) developed acute T-cell rejection, 6 (10.71%) had acute antibody-mediated rejection, 2 (3.57%) had acute T-cell rejection with acute antibody-mediated rejection, 12 (21.43%) had chronic active T cell-mediated rejection, 2 (3.57%) had chronic active antibody-mediated rejection, 2 (3.57%) had chronic active T cell-mediated rejection with antibody-mediated rejection, 8 (14.29%) had non-specific interstitial fibrosis and tubular atrophy, and 3 (5.36%) had normal graft function. The expression levels of immune markers CD3, CD4, CD8, CD20, GrB and perforin differed with the types of T cell-mediated graft rejection, and the positivity and expression levels of these markers tended to increased with the severity of graft rejection. The expression of C4d was positive in all cases with antibody-mediated graft rejection.

CONCLUSIONSThe pathological characteristics of the renal biopsy specimens and expression levels of the immune markers allow timely and accurate evaluation of graft rejection type to provide a reliable pathological and etiological basis for clinical treatment and prognostic assessment.

Adolescent ; Adult ; Aged ; Female ; Graft Rejection ; immunology ; pathology ; Graft Survival ; Humans ; Kidney ; immunology ; pathology ; Kidney Transplantation ; adverse effects ; Male ; Middle Aged ; Retrospective Studies ; Young Adult

Carcinoma, Renal Cell ; immunology ; pathology ; surgery ; Humans ; Keratin-19 ; metabolism ; Kidney Neoplasms ; immunology ; pathology ; surgery ; Male ; Middle Aged ; Neoplastic Cells, Circulating ; pathology ; Nephrectomy ; Vena Cava, Inferior ; pathology ; surgery