A case of Kaposi's sarcoma following treatment of membranoproliferative glomerulonephritis and a review of the literature

Kaposi's sarcoma (KS) is an unusual tumor principally affecting the skin of the lower extremities. Although the association between KS and renal transplant has been well documented, there are a few KS cases in the literature associated with membranoproliferative glomerulonephritis or other glomerular diseases. This report presents a patient with membranoproliferative glomerulonephritis (MPGN) who developed KS following treatment with long-term medium dose glucocorticoid and short-term additional immunosuppressives. The KS cases associated with glomerulonephritis are also reviewed. KS is a rare complication in glomerular diseases that may (or may not) be related to immunosuppression. Hence, immunosuppression treatment should be carefully planned in glomerulonephritis treatment and avoided if they are not essentially necessary.     

Minimal change glomerular disease: a paraneoplastic syndrome in two patients with bronchogenic carcinoma

Glomerulonephritis has often been reported as a paraneoplastic syndrome. In patients with carcinoma, the most common glomerular disease is membranous glomerulonephritis mediated presumably by immune complexes. Minimal change glomerular disease has been hitherto reported, to our knowledge, in only one patient with carcinoma. We report two patients with bronchogenic carcinoma in whom the simultaneous development of the nephrotic syndrome was due to minimal change glomerular disease.     

A Case of Kaposi's Sarcoma Following Treatment of Membranoproliferative Glomerulonephritis and a Review of the Literature

Kaposi's sarcoma (KS) is an unusual tumor principally affecting the skin of the lower extremities. Although the association between KS and renal transplant has been well documented, there are a few KS cases in the literature associated with membranoproliferative glomerulonephritis or other glomerular diseases. This report presents a patient with membranoproliferative glomerulonephritis (MPGN) who developed KS following treatment with long-term medium dose glucocorticoid and short-term additional immunosuppressives. The KS cases associated with glomerulonephritis are also reviewed. KS is a rare complication in glomerular diseases that may (or may not) be related to immunosuppression. Hence, immunosuppression treatment should be carefully planned in glomerulonephritis treatment and avoided if they are not essentially necessary.     

IgA nephropathy presenting clinical features of poststreptococcal glomerulonephritis

IgA nephropathy and poststreptococcal glomerulonephritis are common forms of primary glomerulonephritis in children. This paper reports a 5-year-old Omani boy who had a chance occurrence of these two different glomerular diseases. Our patient presented with clinical features of poststreptococcal glomerulonephritis and then developed recurrent macroscopic hematuria, polyarthritis, bloody diarrhea, and erythematous swelling of the penis. Renal biopsy revealed diffuse mesangial hypercellularity, with focal glomerular sclerosis, fibrous crescents, and mesangial IgA and C3 deposits, consistent with IgA nephropathy. The clinical features and differential diagnosis are outlined.     

Mesangial IgA nephropathy and idiopathic nephrotic syndrome

A 17-year-old male presented with nephrotic syndrome associated with microscopic hematuria. Renal biopsy showed only minor glomerular abnormalities (light microscopy). Immunohistology demonstrated strong mesangial deposition of IgA. Electronmicroscopy disclosed widespread effacement of foot processes in combination with isolated osmiophilic mesangial deposits. The patient responded to standard corticosteroid therapy with complete disappearance of proteinuria. Microscopic hematuria, however, persisted. Five months after steroid therapy was stopped, the nephrotic syndrome relapsed. It was again steroid-responsive with persisting microhematuria. From clinical and morphological data we conclude that the patient has concomitant idiopathic nephrotic syndrome (minimal change glomerulonephritis) and mesangial IgA glomerulonephritis. The simultaneous presence of these two diseases may give some hint as to their pathogenesis. In both, abnormalities in T cell regulation have been found. If these were indeed involved in the pathogenesis of the two glomerular diseases, a higher than expected probability for the two entities to coexist in the same patient is to be expected.     

Immunotactoid glomerulopathy with microtubular deposits, with reference to the characteristics of Japanese cases

We present the case of a 69-year-old man with nephrotic syndrome and renal insufficiency, who developed lobular glomerulonephritis. An electron microscopy examination of a renal biopsy showed microtubular structures of 24 nm in diameter in the subendothelial space and the paramesangial area. These deposits were PAS-positive and Congo red-negative, and revealed predominantly positive staining for kappa light chain. There was no evidence of diseases with highly organized glomerular deposits, such as amyloidosis, cryoglobulinemia, systemic lupus erythematosus or paraproteinemia. Therefore, the patient was diagnosed to have immunotactoid glomerulopathy (ITG). During a seven-year course he has not developed any disease known to be associated with organized glomerular immune deposits. Hence, we believe ITG occurred as a primary glomerular disease in this case. We also highlight cases of ITG with microtubular deposits that have been reported in Japan, compare these cases to previous reports, and show that the characteristics of the Japanese cases are male predominance; a high incidence of membranoproliferative glomerulonephritis (MPGN); a low incidence of monoclonal gammopathy and hematological malignancies and a higher incidence of hypocomplementemia.     

Glomerular mesangial fibrillary deposits in a patient with diabetes mellitus

Various systemic or primary glomerular diseases can result in deposition of fibrillary material in the glomerular tuft and may cause an important diagnostic challenge for the pathologists. Biopsy findings of a patient with type 2 diabetes is presented here in which striking fibrillary structures were identified in the mesangium by ultrastructural examination. The distinction between diabetic fibrillosis and fibrillary glomerulonephritis accompanying diabetic nephropathy is discussed in the setting of a literature review.     

Long-term response to peginterferon in hepatitis C virus-associated nephrotic syndrome from focal segmental glomerulosclerosis

Abstract

Hepatitis C virus (HCV) infection is a global public health problem. Chronic HCV infection is an important cause of chronic liver disease. Since the first reported association between HCV and membranoproliferative glomerulonephritis (MPGN) in 1993, HCV has been described with other types of glomerular diseases, although less frequently. Focal segmental glomerulosclerosis (FSGS) is one such glomerular disease that has been rarely reported in association with HCV. Antiviral therapy with interferon and ribavirin has been shown to be beneficial in HCV-associated MPGN. The optimal therapy of HCV-associated FSGS is not currently known. To our knowledge, long-term response to pegylated interferon monotherapy in treatment of HCV-associated FSGS has not been reported. We report an adult patient with HCV-associated FSGS who presented with nephrotic syndrome and renal failure. Treatment with pegylated interferon alfa-2a monotherapy resulted in sustained virological response with a clinical remission of nephrotic syndrome and stabilization of renal function. Patient continued to remain in clinical remission of nephrotic syndrome with stable renal function, 5 years after treatment. We also briefly review the literature on HCV-associated glomerular diseases, particularly HCV-associated FSGS.     

Unexpected IgA nephropathy during the treatment of a young woman with idiopathic dermatomyositis: case report and review of the literature

This article reports the unexpected discovery of IgA nephropathy in a 26-year-old Chinese woman 1.5 years after the onset of idiopathic dermatomyositis. The patient was taking immunosuppressive agents, prednisolone 25 mg and azathioprine 75 mg daily. Glomerulonephritis associated with idiopathic polymyositis/dermatomyositis is rare. A review of the medical literature indicates that the most common pattern seen in idiopathic polymyositis is mesangial proliferative glomerulonephritis. However, both membranous and mesangial proliferative glomerulonephritis are often seen in idiopathic dermatomyositis. It is still not clear, however, whether the humorally- mediated immune process in dermatomyositis and the cell-mediated immune process in polymyositis can explain the different patterns of occurrence of glomerular lesions in these two closely related disease entities.     

MPO-ANCA-positive crescentic necrotizing glomerulonephritis and tubulointerstitial nephritis with renal eosinophilic infiltration and peripheral blood eosinophilia

We present the case of a 67-year-old woman with myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA)-positive pauci-immune crescentic necrotizing glomerulonephritis and tubulointerstitial nephritis with renal eosinophilic infiltration and peripheral blood eosinophilia. Staining for eosinophil cationic protein indicated that activated eosinophils were involved in the tubulitis, as well as in the glomerular injury. Marked peripheral blood eosinophilia is uncommon in ANCA-positive crescentic necrotizing glomerulonephritis associated with tubulointerstitial nephritis, except in Churg-Strauss syndrome. However, our patient had no clinical history or signs of asthma, no other signs suggestive of allergic diseases, and no histologic findings of granulomas in the kidney, thus failing to fulfill the criteria for Churg-Strauss syndrome.     

Post‐transplant lymphoproliferative disorders with naso‐ and oropharyngeal manifestation

The nasopharyngeal/oropharyngeal lymphatic tissues represent the anatomical site of Epstein–Barr virus (EBV) entry. Post‐transplant lymphoproliferative disorders (PTLD) are often associated with EBV, but little is known about the characteristics of nasopharyngeal/oropharyngeal mass‐forming PTLD. Retrospective evaluation of our own PTLD database (n = 79) and the PubMed^® database (n = 61) has been performed. Sinonasal/oro‐/nasopharyngeal lymphatic masses were early lesions (n = 54/140, 38.5%), polymorphic PTLD (n = 32/140, 23%), monomorphic B‐PTLD (n = 47/140, 33.5%) and T‐PTLD (n = 7/140, 5%). One‐fourth of lesions manifested as masses in the Waldeyer's ring, and in two‐thirds of cases, swelling of tonsils was related to manifestation of benign early lesions. Tonsil infiltration by polymorphic PTLD and monomorphic PTLD was present in one‐third of cases. Extratonsillar masses were mainly monomorphic PTLD. Meta‐analysis of our data in combination with previously published data revealed that lung transplantation and young patients are at a higher risk for earlier manifestation of monomorphic PTLD. Therapy is similar to PTLD therapy strategies, in general reduced immunosuppression and chemotherapy for polymorphic and monomorphic PTLD, and diagnostic and therapeutic surgical gross tumour resection of tonsillar/adenoid lesions. In summary, it is relevant for the clinical differential diagnosis that oro‐/nasopharyngeal aggressive PTLD manifested in ~30% as tonsillar masses and >90% at extratonsillar sites.     

Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies

Dense deposit disease and glomerulonephritis with isolated C3 deposits are glomerulopathies characterized by deposits of C3 within or along the glomerular basement membrane. Previous studies found a link between dysregulation of the complement alternative pathway and the pathogenesis of these diseases. We analyzed the role of acquired and genetic complement abnormalities in a cohort of 134 patients, of whom 29 have dense deposit disease, 56 have glomerulonephritis with isolated C3 deposits, and 49 have primary membranoproliferative glomerulonephritis type I, with adult and pediatric onset. A total of 53 patients presented with a low C3 level, and 65 were positive for C3 nephritic factor that was significantly more frequently detected in patients with dense deposit disease than in other histological types. Mutations in CFH and CFI genes were identified in 24 patients associated with a C3 nephritic factor in half the cases. We found evidence for complement alternative pathway dysregulation in 26 patients with membranoproliferative glomerulonephritis type I. The complement factor H Y402H variant was significantly increased in dense deposit disease. We identified one at-risk membrane cofactor protein (MCP) haplotype for glomerulonephritis with isolated C3 deposits and membranoproliferative glomerulonephritis type I. Thus, our results suggest a critical role of fluid-phase alternative pathway dysregulation in the pathogenesis of C3 glomerulopathies as well as in immune complex-mediated glomerular diseases. The localization of the C3 deposits may be under the influence of MCP expression.     

Glomerular nephropathy associated with chronic Q fever

Of three patients with Coxiella burnetii endocarditis, two developed focal segmental proliferative glomerulonephritis (GN), and the third developed diffuse intracapillary proliferative glomerulonephritis. In one case, a good therapeutic response was followed by partial remission of the renal alterations, but 10 months later there were clinical and histological signs of active glomerular nephropathy, suggesting that the antigenic stimulus persisted. In another case, poor evolution of the infection was accompanied by clinically and histologically aggressive glomerular nephropathy, and advanced renal failure. The third patient, who had diffuse proliferative glomerulonephritis, underwent renal biopsy earlier than the other two cases, and the behavior of the nephropathy has not been aggressive to date. Immunohistopathologic study revealed a diffuse granular deposit of IgM and C3 in all three cases; the first two also presented a discrete linear IgG deposit in the capillary loops. Attempts to identify C burnetii antigen at the glomerular level by immunohistologic techniques failed in two patients. The literature on the association of chronic Q fever with glomerulonephritis is briefly reviewed.     

Lung retransplantation after posttransplantation lymphoproliferative disorder (PTLD): a single-center experience and review of literature of PTLD in lung transplant recipients.

BACKGROUND: Retransplantation in adult lung transplant recipients developing progressive bronchiolitis obliterans syndrome as a consequence of posttransplantation lymphoproliferative disorder (PTLD) therapy has not been reported in the literature. Literature on PTLD after lung transplantation is limited mostly to case reports or small case series, limiting the validity of conclusions. METHODS: Retrospective chart review of patients at our center. Analysis of pooled data published on lung transplant patients developing PTLD. RESULTS: Two patients who underwent pulmonary retransplants for PTLD have functioning grafts 23 and 36 months postoperatively, with no evidence of PTLD recurrence. Review and analysis of published data and from our center revealed that incidence of PTLD, proportion of patients with thoracic involvement, and proportion of patients who were Epstein-Barr virus seronegative before transplantation decreased continuously as a function of time from transplant. Patients developing PTLD within the first 6 months after transplantation had a clinically distinct pattern of PTLD and a significantly better survival than patients developing PTLD more than 6 months after lung transplant. CONCLUSIONS: Lung retransplantation can be considered after careful selection for lung transplant recipients developing bronchiolitis obliterans syndrome as a consequence of reduced immunosuppression for PTLD. Acquisition of PTLD and pattern of organ involvement is a continuous process as a function of time. Defining "early PTLD" as occurring in the first 6 months more accurately predicts progress and prognosis of this disease than the traditional 1 year definition of early vs late onset PTLD.     

T-cell posttransplant lymphoproliferative disorder occurring in a pediatric solid-organ transplant patient

Posttransplantation lymphoproliferative disorder (PTLD) is a serious complication seen in transplant patients as a consequence of immunosuppressant therapy. Most cases are of B-cell origin and are commonly associated with Epstein-Barr virus (EBV) infection. T-cell PTLDs are rare and only 13 pediatric T-cell PTLDs with clinicopathologic correlation have been reported previously. We present the histologic, immunophenotypic, and molecular features of a monomorphic PTLD (T-cell lymphoma) identified in a pediatric patient following orthotopic liver transplantation. The lymphoma was identified in the ileum, rectum, and mesenteric lymph nodes. In situ hybridization revealed numerous EBER-1-positive tumor cells. A current review of the literature is also discussed. Of the 14 cases of pediatric T-cell PTLD reported in the literature, only 3 (21.4%) are described as being EBV positive. Most of the reported PTLDs are monoclonal, with 9 of 11 cases (82%) showing a clonal T-cell population by gene rearrangement studies. T-cell PTLD cases appear to have a poor prognosis (11 of 14 patients died of the disease), although patients with involvement of specific anatomic sites may have a better outcome.     

Sequential occurrence of IgA nephropathy and Henoch-Schönlein purpura: support for common pathogenesis

We report a patient who developed Henoch-Schönlein purpura (HSP) 13 years after he presented with IgA nephropathy (IgAN). In both HSP and IgAN renal biopsy most commonly reveals focal proliferative glomerulonephritis on light microscopy and immunofluorescence displays mesangial IgA deposits. In addition, patients with HSP or IgAN have elevated serum IgA levels, circulating IgA immune complexes, IgA-bearing lymphocytes, immunoglobulin-producing cells, and binding of IgG to glomerular components of similar molecular weight. The occurrence of both diseases in the same patient or the same families and the presence of immune abnormalities compatible with HSP or IgAN in relatives of patients with these diseases suggest a common pathogenesis.     

Necrotic Vasculitis in a Patient with Abdominal Fibromatosis and Guillain-Barré Syndrome

The case of a 65-year-old woman presenting with Guillain-Barré syndrome is herein reported. Tomographic investigation revealed abdominal and retroperitoneal fibromatosis. During her hospitalization, renal involvement ensued, and subsequent renal biopsy demonstrated findings of crescentic pauci-immune glomerulonephritis negative for ANCA antibodies and with characteristics indicative of necrotic angiitis. The simultaneous existence of the three diseases in the same patient as well as the relation between necrotic vasculitis and G-B syndrome is speculated, and the relevant literature is reviewed.     

Necrotic vasculitis in a patient with abdominal fibromatosis and Guillain-Barré syndrome

The case of a 65-year-old woman presenting with Guillain-Barré syndrome is herein reported. Tomographic investigation revealed abdominal and retroperitoneal fibromatosis. During her hospitalization, renal involvement ensued, and subsequent renal biopsy demonstrated findings of crescentic pauci-immune glomerulonephritis negative for ANCA antibodies and with characteristics indicative of necrotic angiitis. The simultaneous existence of the three diseases in the same patient as well as the relation between necrotic vasculitis and G-B syndrome is speculated, and the relevant literature is reviewed.     

Superimposed nephritis: a separate entity among glomerular diseases?

The concomitant occurrence of two glomerular diseases in the same patient was diagnosed in seven out of 105 patients undergoing renal biopsy for suspected glomerulopathy. The most frequently associated disease was a membranous type glomerulopathy. The follow-up was characterized by a rapid deterioration of renal function and two patients were required to start a chronic hemodialysis program soon after the diagnosis. It is suggested that the observed coexisting patterns of glomerular injury do not occur on the basis of chance alone and should be considered as a separate entity in glomerular pathology. In all cases, clinical and pathologic findings were strongly suggestive for two consecutive distinct pathologic processes, thus justifying the use of the term superimposed nephritis. It is reasonable to assume that the mechanisms responsible for glomerular damage and for the evolution of the disease in superimposed nephritis are different from those regulating the corresponding glomerulonephritis when occurring alone. The high prevalence of membranous pattern in superimposed nephritis indicates that pre-existing glomerular alterations might favor an immune reaction in the subepithelial space.     

Renal oncocytoma associated with necrotizing glomerulonephritis

A case of renal oncocytoma associated with focal segmental necrotizing glomerulonephritis is described. The patient showed haematuria, mild proteinuria and arterial hypertension; the diagnosis was made after right nephrectomy performed because of the presence of a renal mass. A severe re-activation of the glomerulonephritis was observed 15 months after the nephrectomy and a steroid and immunosuppressive therapy was started. Our case is the first reported in which the removal of renal oncocytoma is not followed by the disappearance of renal symptoms, as currently reported in literature, suggesting that the two diseases are not always related.