1/35. Successful treatment with steroid pulse therapy in a case of immunotactoid glomerulopathy with hypocomplementemia.We report a case of immunotactoid glomerulopathy with severe hypocomplementemia. The patient was a 47-year-old woman who presented with pitting edema, proteinuria, and hypertension. Serological testings were negative or within normal limits except for hypocomplementemia. There were no findings of hematopoietic diseases, cryoglobulinemia, and systemic lupus erythematosus. The renal biopsy specimen showed membranoproliferative glomerulonephritis with numerous periodic acid-Schiff (PAS)-positive deposits. Under electron microscopy, however, microtubular structure was shown in the mesangial matrix and the subendothelial and subepithelial spaces of the peripheral capillary loops. These histological features were compatible with those of immunotactoid glomerulopathy. Although conventional oral steroid therapy failed to have an effect on proteinuria and hypocomplementemia over 3 months, steroid pulse therapy brought dramatic relief: complete remission of proteinuria and normalization of hypocomplementemia. These findings suggest that intensive immunosuppressive therapy may cure a kind of immunotactoid glomerulopathy with hypocomplementemia.- - - - - - - - - - ranking = 1keywords = cryoglobulinemia (Clic here for more details about this article) |
2/35. Diffuse recidivant alveolar hemorrhage in a patient with hepatitis c virus-related mixed cryoglobulinemia.A case of diffuse and recidivant alveolar hemorrhage is presented in a patient with hepatitis c virus-related type II mixed cryoglobulinemia with membranoproliferative glomerulonephritis. The patient was a 48-year-old white woman who suffered several outbreaks of pulmonary hemorrhage refractory to treatment with steroids, cyclophosphamide, azathioprine, plasmapheresis and interferon-alpha. The patient also presented persistent increased titers of immune complexes and rheumatoid factor with no histological hepatic alterations. Some considerations about evolution and treatment are given according to the updated physiopathology of this disease.- - - - - - - - - - ranking = 5keywords = cryoglobulinemia (Clic here for more details about this article) |
3/35. Membranoproliferative glomerulonephritis type I, mixed cryoglobulinemia and lymphoma in the absence of hepatitis c infection.Chronic hepatitis c virus infection has been linked to cryoglobulinemia, membranoproliferative glomerulonephritis, and malignant B-cell lymphoproliferation, suggesting a possible pathogenetic link between these disorders. We report a patient with the latter clinical triad in the absence of hepatitis c infection. We postulate that the persistent and dysregulated immunologic activity associated with chronic antigen stimulation, inflammation and/or B-cell malignancy induces nephritogenic autoantibodies, including cryoglobulins, that produce a similar clinical syndrome in genetically susceptible individuals. copyright copyright 1999 S. Karger AG, Basel- - - - - - - - - - ranking = 5keywords = cryoglobulinemia (Clic here for more details about this article) |
4/35. The effect of combination therapy with interferon and cryofiltration on mesangial proliferative glomerulonephritis originating from mixed cryoglobulinemia in chronic hepatitis c virus infection.Cryofiltration, which has developed from double filtration plasmapheresis (DFPP) with a cooling unit, is an on-line technique to remove cryoglobulin. We report on a patient who suffered from progressive edema and renal insufficiency caused by cryoglobulinemic membranoproliferative glomerulonephritis (MPGN), probably due to chronic hepatitis c virus (HCV) infection. To remove cryoglobulins and terminate the HCV infection, we utilized combination therapy with cryofiltration and interferon-alpha injection with corticosteroids. interferon-alpha was capable of decreasing proteinuria but not diminishing cryoglobulin. Additional cryofiltration could remove cryoglobulin to an undetectable level. This combination therapy was partially successful to reduce proteinuria and prevent the progressive deterioration of renal function. The major adverse effects of this therapy were bleeding and myelosuppression. We conclude that this combination therapy may be effective and should be considered as treatment for cryoglobulinemic MPGN.- - - - - - - - - - ranking = 4keywords = cryoglobulinemia (Clic here for more details about this article) |
5/35. Membranoproliferative glomerulonephritis and demyelinating neuropathy caused by type II mixed cryoglobulinemia associated with HCV infection.Type II cryoglobulinemia is sometimes observed in patients with HCV infection. It is also well known that some cases with membranoproliferative glomerulonephritis (MPGN) possess cryoglobulinemia, which can induce neuropathy. We treated a 73-year-old woman with hepatitis c virus (HCV) who was admitted to our department because of proteinuria. She was diagnosed as MPGN according to renal biopsy. Her ankle jerk was absent with reduced touch sensation and vibration below the knees; sural nerve biopsy revealed demyelinating neuropathy. Administration of prednisolone resulted in improved proteinuria. cryoglobulinemia associated with HCV infection seemed to induce MPGN and demyelinating neuropathy.- - - - - - - - - - ranking = 6keywords = cryoglobulinemia (Clic here for more details about this article) |
6/35. Cryofiltration and oral corticosteroids provide successful treatment for an elderly patient with cryoglobulinemic glomerulonephritis associated with hepatitis c virus infection.A 75-year-old man was admitted due to nephrotic syndrome, purpura on the legs, which was associated with hepatitis c virus (HCV), and type II mixed cryoglobulinemia. Renal biopsy revealed features of cryoglobulinemic glomerulonephritis. Since the patient was elderly and the HCV genotype was Ib, interferon-alpha for reducing HCV was not indicated. Four sessions of cryofiltration and the administration of corticosteroids improved the proteinuria and renal function strikingly without adverse effects. This case demonstrates that an elderly patient who has nephrotic syndrome caused by cryoglobulinemic glomerulonephritis associated with HCV can be treated safely by cryofiltration with low doses of oral corticosteroids.- - - - - - - - - - ranking = 1keywords = cryoglobulinemia (Clic here for more details about this article) |
7/35. The benefit of long-term interferon alfa therapy for symptomatic mixed cryoglobulinemia (cutaneous vasculitis/membranoproliferative glomerulonephritis) associated with chronic hepatitis c infection.The efficacy of long-term interferon therapy for chronic hepatitis c infection with symptomatic mixed cryoglobulinemia has not clearly been defined. We describe a patient with chronic hepatitis c, symptomatic mixed cryoglobulinemia (cutaneous vasculitis), and membranoproliferative glomerulonephritis (MPGN) who responded clinically, biochemically, and virologically to a 1-year course of interferon therapy. Interferon side effects were minimal. Relapse occurred when interferon was discontinued, and suppressive maintenance interferon therapy was required for clinical, biochemical, and virologic remission. During the 5th year of maintenance therapy, she developed potential side effects that necessitated discontinuation of interferon treatment. After treatment stoppage, a clinical, biochemical, and virologic remission was maintained for more than 1 year. However, the potential side effects, which included eye irritation, arthralgias, myalgias, fatigue, insomnia, memory loss, and depression, persisted. Ophthalmologic, rheumatologic, and neurologic evaluations were nondiagnostic. Psychometric testing revealed dementia and mood disorder. Because the disabling symptoms persisted after 9 months, a health-related quality of life assessment was carried out with the SF-36 survey. Compared with healthy individuals and patients with chronic hepatitis c, our case had a lower health-related quality of life assessment on six out of seven scales and on four out of seven scales of the SF-36 survey, respectively. This case report indicates that long-term maintenance interferon therapy was effective in the treatment of symptomatic mixed cryoglobulinemia and its renal complications and resulted in a clinical, biochemical, and virologic sustained response. It is postulated that the side effects of long-term interferon therapy in this setting may be problematic.- - - - - - - - - - ranking = 7keywords = cryoglobulinemia (Clic here for more details about this article) |
8/35. interferon-alpha in combination with ribavirin as initial treatment for hepatitis c virus-associated cryoglobulinemic membranoproliferative glomerulonephritis.Mixed cryoglobulinemia (MC) and glomerulonephritis are the most important extrahepatic manifestations of chronic hepatitis c virus (HCV) infection. In HCV-infected patients with MC, renal involvement worsens the overall prognosis because of a high incidence of infection or cardiovascular disease. The relationship between MC and HCV infection has prompted the use of antiviral therapy. Two patients with chronic HCV infection, type-II MC and membranoproliferative glomerulonephritis (MPGN), presenting as nephrotic syndrome were treated with interferon (IFN)-alpha (3 MU 3 times per week) and ribavirin (15 mg/kg daily) for 6 months. Laboratory tests included measurement of anti-HCV antibodies, HCV rna, and HCV genotyping, and characterization of circulating cryoglobulins. A pretreatment renal biopsy was performed, and the histopathologic lesions were scored according to the index of disease activity. viremia and cryoglobulinemia were suppressed in both patients. However, a complete remission of proteinuria was observed in 1 patient only. The evaluation of the renal biopsy specimens revealed a mild MPGN (activity score: 5/24) in the patient with remission of proteinuria and a severe MPGN (activity score: 15/24) in the patient who maintained a nephrotic-range proteinuria. Although a fully satisfactory treatment is not yet available, we feel that a reasonable therapeutic strategy for HCV-infected patients with MC nephritis could be as follows: (1) antiviral treatment alone for patients with a low-grade kidney involvement, and (2) a short-term course of steroids and cytotoxic drugs followed by antiviral therapy for acute exacerbations and/or rapidly progressive GN.- - - - - - - - - - ranking = 2keywords = cryoglobulinemia (Clic here for more details about this article) |
9/35. Type I membranoproliferative glomerulonephritis in an hiv-infected individual without hepatitis c co-infection.Type I membranoproliferative glomerulonephritis (MPGN) is an uncommon manifestation of human immunodeficiency virus (hiv)-associated renal disease in patients co-infected with hepatitis c virus (HCV). We describe a case of Type I MPGN in an hiv-positive diabetic man with nephrotic-range proteinuria and renal insufficiency who was not co-infected with HCV. Tubuloreticular inclusions were present but there was no evidence for either cryoglobulinemia or cryoglobulin deposits in the kidney. This finding suggests that Type I MPGN may represent a reaction of the kidney to hiv independent of the effects of HCV co-infection. Clinical suspicion must be maintained for Type I MPGN in all hiv infected patients presenting with significant proteinuria regardless of HCV infection status.- - - - - - - - - - ranking = 1keywords = cryoglobulinemia (Clic here for more details about this article) |
10/35. Membranoproliferative glomerulonephritis associated with low-grade B cell lymphoma presenting in the kidney.Low-grade B cell lymphoma of mucosa-associated tissue type (MALToma) rarely may involve the kidney. Membranoproliferative glomerulonephritis (MPGN) is an uncommon complication of B cell lymphoma and may be related to cryoglobulin and/or immunoglobulin synthesis by a secretory B cell clone. We report 2 patients with the novel renal biopsy findings of coexistent MALToma and MPGN. Both subjects presented with nephrotic proteinuria and renal insufficiency. One patient had a serum M protein (IgG K) but neither individual had any other clinical or serologic evidence of systemic disease, including hematolymphoid malignancy, autoimmune disease, cryoglobulinemia, or hepatitis c viral infection. Both renal biopsies demonstrated MPGN type I with immunoglobulin deposits that in 1 case showed light chain restriction (IgM K). Electron microscopy disclosed corresponding glomerular electron dense deposits in subendothelial locations. Both biopsies also contained atypical interstitial lymphoid infiltrates comprising marginal zone (centro-cyte-like) cells that infiltrated tubules and showed extra-capsular extension. Immunostains demonstrated a predominantly B cell population that lacked expression of CD5 and cycline D1, and gene rearrangement studies confirmed the presence of a monoclonal B cell population in both cases. These findings indicate that low-grade B cell lymphoma in the kidney may be an unexpected finding in patients with nephrotic syndrome related to MPGN. Immunophenotypic and gene rearrangement studies are important ancillary tools for the evaluation of atypical lymphoid infiltrates in kidney biopsies.- - - - - - - - - - ranking = 1keywords = cryoglobulinemia (Clic here for more details about this article) |
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