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Background: Dermatomyositis (DM) is a systemic autoimmune disease characterized by distinct skin lesions and a clinically heterogeneous constellation of systemic manifestations. This disease poses a challenge to clinicians because of its rarity, diverse clinical presentations, and variable organ involvement, resulting from an autoimmune attack on affected organs, which could be triggered by environmental factors in genetically susceptible individuals. Renal involvement is rare, with immunoglobulin M (IgM) nephropathy yet to be reported in patients with DM. Case presentation: A 38‐year‐old man was admitted to Shariati Hospital, affiliated with Tehran University of Medical Sciences, with proximal weakness of the upper and lower extremities that had developed in the preceding month after receiving the Sinopharm COVID‐19 vaccine. The patient was diagnosed with DM based on the heliotrope rash, Gottron's papules, progressive proximal muscle weakness, and paraclinical findings. IgM nephropathy developed subsequently, diagnosed by light and immunofluorescence microscopy. Conclusion: We describe the first case of IgM nephropathy in a DM patient following COVID‐19 vaccination. This phenomenon requires further investigation into the possible crosslinks between the pathogenesis of IgM nephropathy with DM and the COVID‐19 vaccine. Diagnosing renal complications in DM patients promptly and accurately can help to achieve the best outcomes. [ABSTRACT FROM AUTHOR]

Abstract A 13-year-old Chinese girl attended to our Pediatric Dermatology Unit for the appearance of itchy targetoid lesions on the trunk, face and upper limbs. A skin biopsy showed histological findings typical of erythema multiforme minor. A month earlier she was admitted for the onset of a nephrotic syndrome and the renal biopsy showed an IgM nephropathy with a diffuse mesangial cell proliferation. There was no medical history of recent infections, fever, muscle or joint pain, drugs intake related to erythema multiforme and viral serology were negative. The role of antibodies in erythema multiforme could be more relevant than suspected and the severity of erythema multiforme was reported to be proportional to the antibody-mediated complement-dependent cytotoxicity, supporting the potential pathogenetic role for humoral immunity in this subtype of erythema multiforme. We reported the first association of erythema multiforme and IgM nephropathy in a pediatric patient providing an additional hint that an antibody-mediated process, rather than T-cell cytotoxicity, might represent the main pathogenetic mechanism in certain subtypes of erythema multiforme.

A 13-year-old Chinese girl attended to our Pediatric Dermatology Unit for the appearance of itchy targetoid lesions on the trunk, face and upper limbs. A skin biopsy showed histological findings typical of erythema multiforme minor. A month earlier she was admitted for the onset of a nephrotic syndrome and the renal biopsy showed an IgM nephropathy with a diffuse mesangial cell proliferation. There was no medical history of recent infections, fever, muscle or joint pain, drugs intake related to erythema multiforme and viral serology were negative. The role of antibodies in erythema multiforme could be more relevant than suspected and the severity of erythema multiforme was reported to be proportional to the antibody-mediated complement-dependent cytotoxicity, supporting the potential pathogenetic role for humoral immunity in this subtype of erythema multiforme. We reported the first association of erythema multiforme and IgM nephropathy in a pediatric patient providing an additional hint that an antibody-mediated process, rather than T-cell cytotoxicity, might represent the main pathogenetic mechanism in certain subtypes of erythema multiforme. [ABSTRACT FROM AUTHOR]

Abstract ICPIs were effective for primary and metastatic foci of lung adenocarcinoma, but their repeated use provoked a late relapse of IgM nephropathy and lethal lesions in pancreas and lung. ICPIs should be used carefully in cases of immune‐related disease.

Abstract Background IgM nephropathy is a rare disease with variable clinical presentations and is an unusual cause of nephrotic syndrome. Histopathological findings typically include mesangial hypercellularity with IgM and complement deposition, though the spectrum may range from normal glomeruli through to focal and segmental glomerulosclerosis. Thromboembolism is a well recognised complication of nephrotic syndrome, but cerebral venous sinus thrombosis is rarely described. Case presentation This is the case of a 23-year-old male presenting with the nephrotic syndrome, whose initial renal biopsy was consistent with minimal change disease. Complete remission was achieved with prednisone, however multiple relapses and steroid dependence prompted re-biopsy, the results of which were more consistent with IgM nephropathy. His last relapse was complicated by cerebral venous sinus thrombosis. He then received rituximab and a weaning course of prednisone to again enter remission. Conclusions This case highlights the need to consider IgM nephropathy in the differential diagnosis of nephrotic syndrome. Additionally, it emphasises the risk of thrombosis in patients with severe nephrosis.

We present a case with a rare variant of glomerulonephritis, IgM nephropathy, which occurs mainly with nephrotic syndrome. The clinical features of this variant of kidney damage are characterized; the pathogenetic and the transformation of this form of nephritis into focal segmental glomerulosclerosis are discussed. The development of severe nephrotic syndrome at the beginning of the disease, the formation of secondary steroid resistance have confirmed this hypothesis and have justified the treatment with cyclosporin A aimed at the recovery of the function of the podocyte with remission of nephritis.

ICPIs were effective for primary and metastatic foci of lung adenocarcinoma, but their repeated use provoked a late relapse of IgM nephropathy and lethal lesions in pancreas and lung. ICPIs should be used carefully in cases of immune‐related disease. [ABSTRACT FROM AUTHOR]

Background: Mesangioproliferative glomerulonephritis (MesPGN) is a common morphological pattern that encompasses several groups of renal diseases including IgA nephropathy (IgAN), IgM nephropathy (IgMN), lupus nephritis (LN), C1q nephropathy (C1qN) and other entities. Objectives: The aim of this study was to analyze the pathological findings and the clinical features of cases of MesPGN seen at the king Abdulaziz University, in Saudi Arabia. Patients and Methods: A total of 750 percutaneous native renal biopsies were seen at our institution from January 2000 to December 2011. All the cases diagnosed as MesPGN on light microscopy (LM) were retrieved from the archives of pathology. The pathological features and the clinical data of these cases were reviewed. The clinical data was available for 80 cases only. Results: A total of 103 cases (14%) met the inclusion criteria for the diagnosis of MesPGN. The most common diagnostic entity was IgMN (46.6%) followed by IgAN (30%) along with few cases of class II LN, C1qN, minimal change disease (MCD), Alport’s syndrome, focal segmental glomerulosclerosis (FSGS), thin basement membrane disease (TBMD), and fibrillary glomerulonephritis. The most common clinical presentation was nephrotic syndrome seen in 71% of 80 cases, followed by hematuria (14%). Histologically, focal mesangial proliferation was seen in 62% while diffuse proliferation was seen in 38% of the cases. Conclusion: Mesangioproliferative glomerulonephritis is an important cause of nephrotic syndrome in young adults in the western region of Saudi Arabia. Future studies from the region are needed to elucidate the clinical relevance of mesangial cell proliferation to the end stage kidney diseases.

The spectrum of biopsy-proven glomerular disease was studied from a single center in Northwestern India, among adolescents aged 13-19 years. From January 2009 to December 2012, a total of 177 patients with biopsy-proven glomerular disease were studied. The same pathologist reported all the biopsy specimens after subjecting to light, immunofluorescence, and electron microscopy. The clinical profile and laboratory findings of the patients were correlated with the histopathological spectrum of glomerular diseases. Males formed 71.19% (n = 126) and the remaining 28.81% (n = 51) were females. Lupus nephritis had a strong female predominance, whereas minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) had a male predominance. Nephrotic syndrome was the indication for biopsy in 65% of the cases. Rapidly progressive renal failure and acute nephritis were the next common indications accounting for 14% and 7%, respectively. FSGS was the most common glomerular disease in adolescents (n = 45, 25.4%). The next common were MCD and lupus nephritis each contributing 21.6% and 10.7%, respectively. Primary glomerular diseases accounted for 84.75% (n = 150) of the total. The remaining 15.25% (n = 27) were attributed to secondary glomerular diseases, of which lupus nephritis was the most common, i.e., in 70.4% patients (n = 19). FSGS was the most common histology in adolescent nephrotic participants (37%). MCD was the next common, found in 31% of nephrotic patients. Electron microscopy changed the diagnosis made by light microscopy and immunofluorescence in 5.6% cases only, and it confirmed the diagnosis in another 21.6%. Kidney biopsy in adolescents is a safe procedure. The spectrum of glomerular diseases in adolescents is different from that seen in adults and smaller children. [ABSTRACT FROM AUTHOR]

The authors previously investigated progressive glomerulonephropathy in 2- to 11-year-old common marmosets and characterized age-related changes of the renal glomeruli and development of tubulointerstitial lesions. In this study, immunoglobulin deposition and ultrastructural changes of the glomeruli were investigated in 5 young marmosets from 6 months to 3 years of age with pre-onset or early glomerulonephropathy. In all animals, the foot processes of podocytes were effaced, and IgM was deposited into the glomeruli. In glomeruli without glomerular basement membrane (GBM) alteration, IgM was the only immunoglobulin type deposited in the glomeruli. In cases with more advanced lesions of reticulation and thickening of GBM, IgA and IgG deposits were also observed. Therefore, the authors conclude that IgM may be the primary or earliest immunoglobulin deposited in this nephropathy, whereas IgA and IgG deposition may be connected to the progression of the glomerular lesions. IgM deposition and foot process effacement of podocytes occur early in the life of affected marmosets. [ABSTRACT FROM AUTHOR]

Context: IgM nephropathy (IgMN) is a relatively less recognized clinico-immunopathological entity in the domain of glomerulonephritis , often thought to be a bridge between minimal change disease and focal segmental glomerulosclerosis. Evidence Acquisitions: Directory of Open Access Journals (DOAJ), Google Scholar, Pubmed (NLM), LISTA (EBSCO) and Web of Science has been searched. Results: IgM nephropathy can present as nephritic syndrome or less commonly with subnephrotic proteinuria or rarely hematuria. About 30% patients respond to steroids whereas others are steroid dependent / resistant. They should be given a trial of Rituximab or stem cell therapy. Conclusions:IgM nephropathy (IgMN) is an important and rather neglected pathology responsible for renal morbidity in children and adults in developing countries as compared to developed nations with incidence of 2-18.5% of native biopsies. Abnormal T-cell function with hyperfunctioning suppressor T-cells are believed to be responsible for this disease entity. Approximately one third of the patients are steroid responders where as the remaining two thirds are steroid resistant or dependent. Therapeutic trials including cell therapies targeting suppressor T-cells are required.

Background: Immunoglobulin M nephropathy (IgMN) is an idiopathic glomerulonephritis (GN) usually presenting clinically as steroid resistant/dependent nephrotic syndrome (NS) with pathology of mesangial proliferative GN or focal and segmental glomerulosclerosis with diffuse predominant mesangial IgM deposits. Not much information is available about its natural history. This is the first Indian study to our knowledge on IgMN in adults and adolescents. Materials and Methods: We evaluated renal biopsies performed at our center between January,′04 to September,′09. Biopsies of all adolescents and adults were evaluated for IgMN and we studied their age, gender distribution, blood pressure (BP), disease duration, steroid/immunosuppressive management and serial serum creatinine (SCr), urinary proteins, and BP values. Patients with other systemic diseases/infections and children were excluded. Results: IgMN constituted 4.3% of 2702 adult renal biopsies. No significant gender predilection was noted. Males presented at average age of 23.1 years, females at 30 years. Steroid-dependent NS was the commonest presentation noted in 75% followed by steroid-resistant NS. Hypertension was noted in 10% patients. Mesangial proliferative GN (MePGN) was commonest histopathological finding noted in 74.4%, followed by focal segmental glomerulosclerosis (FSGS) in 16.2%, and minimal change disease (MCD) in 9.4% biopsies. Sole IgM deposits were noted in 88.5%. All MCD, 35.6% MePGN reached remission, FSGS progressed to renal failure by 1 year. Hypertension, proteinuria, interstitial fibrosis, and FSGS were bad prognosticators. Conclusions: This is the first Indian study of IgMN in adults and adolescents carried out over a period of 5.8 years, which has shown that hypertension, proteinuria, and interstitial fibrosis at presentation have bad prognosis.

Background: It has been suggested that C1q and immunoglobulin M (IgM) nephropathy are variants of minimal change nephrotic syndrome (MCNS). Many researchers believe that these two conditions signify a worse prognosis for children with MCNS in comparison with immunofluorescence (IF)-negative MCNS. The aim of our study was to determine the prognostic significance of C1q nephropathy and IgM nephropathy in children with MCNS. Methods: Fifty-five children with MCNS who had been biopsied over the course of 24 years at our institution were retrospectively categorized into three groups on the basis of IF microscopy findings: IF-negative MCNS (29/55 patients), MCNS with IgM nephropathy (19/55 patients), and MCNS with C1q nephropathy (7/55 patients). Clinical characteristics at disease presentation, clinical course, and renal outcome were compared between groups during the median follow-up period of 16.9 years (minimum 1.0, maximum 31.1 years). Results: No statistically significant differences in clinical characteristics at disease presentation, clinical course, and renal outcome were found. Children with IgM nephropathy, C1q nephropathy, and IF-negative MCNS were clinically indistinguishable. Conclusions: We concluded that C1q or IgM nephropathy variants do not seem to signify a worse prognosis in children with MCNS in comparison with IF-negative MCNS. [ABSTRACT FROM AUTHOR]

Implication for health policy/practice/research/medical education: Much has been published on the epidemiology and clinicopathological characteristics of IgM nephropathy, but there is little information on the etiology, pathogenesis and specific therapy of the disease. Controversy still shrouds the definition and nosologic status of the disease. Well-coordinated and concerted international efforts and collaboration between researchers in the developing and developed countries are needed to make further progress on the above aspects of the disease. [ABSTRACT FROM AUTHOR]

Background: Reactivation of hepatitis B virus (HBV), characterized by increased levels of serum HBV DNA, abnormal liver function and hepatic failure, is a frequent complication of immunosuppressive therapy and chemotherapy in patients with HBV infection. However, reactivation of occult HBV infection with immunosuppressive therapy or chemotherapy is rare.Case Presentation: A 77-year-old man was diagnosed with nephrotic syndrome and IgM nephropathy with unclear pathogenesis. Liver function was normal, HBV-related serum markers were negative and HBV DNA titer was below the upper limits of normal. Two months following the start of prednisone therapy for his nephrotic syndrome, laboratory tests revealed a substantial increase in serum transaminase levels (ALT: 490 IU/L; AST: 149 IU/L) and an elevation of HBV DNA level (3.42×10(6) copies/ml). We tested stored kidney tissue for HBsAg and HBcAg using immunohistochemistry and found the sample to be HBcAg positive, allowing us to confirm the etiology of nephropathy as an occult HBV infection. The cause of the hepatitis was thought to be HBV reactivation, so we immediately administered lamivudine. One month after the initiation of daily lamivudine treatment, laboratory tests revealed that serum levels of transaminases had improved (ALT: 35 IU/L; AST: 17 IU/L). Patient examination one year later showed that HBeAg had decreased with a concomitant increase of HBeAb, the quantity of HBV DNA was undetectable, and liver function and renal function had stabilized.Conclusion: This is the first report describing HBV reactivation in an occult HBV infection patient treated with oral prednisone for nephrotic syndrome. HBV-associated antigen should be regularly tested for in patients with unknown etiological glomerulonephritis in areas with high HBV viral popular and even in those with no clinical evidence for diagnosis of HBV. [ABSTRACT FROM AUTHOR]

Copyright of Nefrologia is the property of Revista Nefrologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Nefrologia is the property of Revista Nefrologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Context: IgM nephropathy (IgMN) is a relatively less recognized clinico-immunopathological entity in the domain of glomerulonephritis, often thought to be a bridge between minimal change disease and focal segmental glomerulosclerosis. Evidence Acquisitions: Directory of Open Access Journals (DOAJ), Google Scholar, Pubmed (NLM), LISTA (EBSCO) and Web of Science has been searched. Results: IgM nephropathy can present as Nephrotic syndrome or less commonly with subnephrotic proteinuria or rarely hematuria. About 30% patients respond to steroids whereas others are steroid dependent / resistant. They should be given a trial of Rituximab or stem cell therapy Conclusions: IgM nephropathy (IgMN) is an important and rather neglected pathology responsible for renal morbidity in children and adults in developing countries as compared to developed nations with incidence of 2-18.5% of native biopsies. Abnormal T-cell function with hyperfunctioning suppressor T-cells are believed to be responsible for this disease entity. Approximately one third of the patients are steroid responders where as the remaining two thirds are steroid resistant or dependent. Therapeutic trials including cell therapies targeting suppressor T-cells are required. [ABSTRACT FROM AUTHOR]

Background: We analyzed the impact of immunoglobulin M (IgM) positivity on the relapse-free interval post completed course of cyclophosphamide (CYC) treatment in patients with steroid-dependent nephrotic syndrome (SDNS) and minimal change disease (MCD). Methods: This was a retrospective chart review of all children who received CYC for SDNS and MCD between 1988 and 2009. Patients were divided into three groups based on kidney biopsy: MCD without immunoglobulin M (IgM) positivity (IgM−), MCD with IgM-positive immunofluorescence (IF) only (IgM+), and MCD with IgM-positive IF and electron-dense deposits on electron microscopy (IgM++). The relapse-free time interval to the first relapse post-CYC therapy or up to 48 months of follow-up (if no relapse occurred) was used for survival analysis. Results: Forty children aged 1.5-12.3 years (15 were IgM−, 16 were IgM+, 9 were IgM++) received a cumulative CYC dose of 175 ± 30 mg/kg. The overall relapse-free survival time was 75 % at 12 months, 64 % at 24 months, 59 % at 36 months, and 56 % at 48 months, with no significant differences between the IgM groups ( p = 0.80). Conclusions: Based on our results, we conclude that more than 50% of our SDNS patients with MCD remained relapse-free 4 years post-CYC treatment. No significant difference in the response to CYC was observed between patients with or without IgM positivity. [ABSTRACT FROM AUTHOR]

Kidney biopsy is an investigation for diagnosis and prognosis of a variety of nephritides. It also influences therapeutic options. Immunofluorescence (IMF) greatly adds in identifying the pathologies which may not be obvious on light microscopy (L/M), such as IgM, IgA nephropathy, pauci-immune glomerulonephritis, and anti-glomerular basement membrane disease. We present here data of 170 pediatric kidney biopsies from July 2005 to December 2009 from Department of Nephrology and Hypertension, Lady Reading Hospital, Peshawar, Pakistan. The study was undertaken to see whether IMF would alter the histological pattern of pediatric kidney biopsies and to compare these data with an earlier data from our department of 415 pediatric kidney biopsies done over 7-year period from 1998 to 2005, which were analyzed with L/M alone. Out of 170 kidney biopsies using L/M and IMF, IgM turns out to be most common pattern (20%%), followed by minimal change disease (MCD) (17.05%%), focal and segmental glomerulosclerosis (FSGS) (15.88%%), chronic sclerosing glomerulonephritis (Chr. sclerosing GN) (12.35%%), mesangio proliferative glomerulonephritis (MPGN) (7.65%%), mesangio capillary glomerulonephritis (MCGN) (6.47%%), membranous glomerulonephritis (Mem. GN) (5.29%%), IgA nephropathy (5.29%%), cresentic glomerulonephritis (Cres. GN) (3.53%%), lupus nephritis (2.96%%), and others (3.53%%). Comparing these results of 170 cases with 415 renal biopsies without IMF, IgM dominated the histological pattern in IMF group whereas MCD followed by FSGS and MPGN were prominent in group without IMF. Therefore, variation in the overall histological pattern with IMF technique proved statistically significant ( p < 0.0001). Addition of IMF has altered the frequency of MCD, a change from 24%% (100/415) to 17%% (29/170), FSGS from 18.3%% (76/415) to 15.88%% (27/170), and MPGN from 17.35%% (72/415) to 7.65%% (13/170). [ABSTRACT FROM AUTHOR]

Immunoglobulin (Ig) M nephropathy is defined by electron-dense mesangial deposits and mesangial IgM visible by immunofluorescence (IF) without other histopathologic and immunofluorescent microscopic abnormalities. Certain patients have only immuno-positive (IgM+) IF. Children presenting with steroid-dependent or steroid-resistant nephrotic syndrome have a high prevalence of IgM+ IF with or without electron-dense deposits. We reviewed the clinical course of children with steroid-dependent or steroid-resistant nephrotic syndrome who underwent renal biopsy at Texas Children‘s Hospital from 1989 to 2006 to further characterize IgM+ IF in children with nephrotic syndrome. Of the 55 children with steroid-resistant or -dependent minimal change disease (MCD), 23 had IgM+ IF. Of these 23 children, 61% had microscopic hematuria at presentation, 48% (11/23) were steroid-dependent, and 48% (11/23) steroid-resistant (one underwent biopsy prior to steroid therapy). We compared the efficacy of adjuvant treatment with cyclophosphamide and cyclosporine: 18% initially treated with cyclophosphamide obtained remission, while 55% had no response; 83% obtained subsequent remission with cyclosporine. Of those initially treated with cyclosporine, 88% obtained complete or partial remission. IgM+ IF may be surrogate marker for the severity of MCD. Based on our results, children with MCD and IgM+ IF have a better response to cyclosporine than cyclophosphamide. [ABSTRACT FROM AUTHOR]

Aim: Renal abnormalities have been reported in ankylosing spondylitis (AS). The purpose of this study was to elucidate the nature of glomerulonephropathy in AS. Methods: Two hundred and sixty-six patients with definite AS diagnosed at Taipei Veterans General Hospital, Taiwan, were enrolled. Urinary analysis and renal biopsy were performed in this study. The nature of glomerulonephropathy in AS was analyzed. Results: Twelve out of 266 (4.5%) patients had AS-associated nephropathy manifesting as haematuria alone (11 patients) or haematuria and proteinuria (1 patient). Renal biopsy in seven patients showed IgA nephropathy in two cases, mesangial nephropathy with isolated C3 deposits in three cases, and IgM nephropathy in two cases, one of which had accompanied infectious endocarditis. Conclusion: AS-associated nephropathy is not uncommon and mesangial nephropathy is the most common form. Microscopic haematuria and proteinuria are the most common manifestations of renal involvement. [ABSTRACT FROM AUTHOR]

Familial Mediterranean fever (FMF) is the most common periodic syndrome characterized by various clinical manifestations associated with self-limited auto-inflammatory process. Amyloidosis is its most common renal complication that can be prevented with colchicine treatment. In the last years, further clinical and histological features of FMF nephropathy have been established, such as the associations with vasculitic diseases and various types of glomerulonephritis. IgM nephropathy (IgMN) is an uncommon histological entity that is characterized by prominent diffuse mesangial deposition of IgM. Herein, we present a 10-year-old Turkish female child suffering from FMF in which non-nephrotic proteinuria is due to IgMN other than amyloidosis. [ABSTRACT FROM AUTHOR]

Treatment of steroid-resistant nephrotic syndrome (SRNS) remains a challenge to pediatric nephrologists. Recently, intravenous cyclophosphamide (IVCPM) infusion was shown to be effective, safe, and economical for the treatment of SRNS, particularly minimal change disease (MCD), as it results in more sustained remissions, longer periods without proteinuria, and fewer significant side effects at a lower cumulative dose. A prospective study was conducted to evaluate IV-CPM infusions in the management of children with SRNS secondary to MCD or IgM nephropathy. Five patients with SRNS (4 IgM nephropathy and 1 MCD) received six monthly IV-CPM infusions at a dose of 500 mg/m[sup2]. No patient achieved complete or sustained remission. Three patients attained partial remission, which was not sustained for more than 1 month post therapy. One patient progressed rapidly to end-stage renal disease during treatment. Side effects included vomiting in four patients and alopecia in one patient. Conclusion: IV-CPM pulse therapy at a dose of 500 mg/m[sup2] is unsuccessful in obtaining complete or sustained remission in children with SRNS secondary to IGM nephropathy or MCD. Further randomized controlled studies with higher doses are required. [ABSTRACT FROM AUTHOR]

Abstract Background: The aim of the present study was to investigate to what extent IgM nephropathy in children with minimal change nephrotic syndrome (MCNS) and diffuse mesangial hypercellularity (DMH) evolves to focal segmental glomerulosclerosis (FSGS). Methods: Tissues from renal biopsies were examined by light microscopy (LM), immunofluorescence (IF) and, in four cases, by electron microscopy (EM). From a total of 352 nephrotic children, 121 had renal biopsy results as steroid dependent or resistant. A diagnostic renal biopsy was also perfomed in 331 children with non-nephrotic proteinuria and/or hematuria. A second renal biopsy was performed in 16 children whose renal function was impaired during the follow up. The clinical course of IgM-positive children was compared with that of IgM-negative children. Results: Of the 121 nephrotic children with renal biopsy, 85 were MCNS. Twenty were IF positive mainly for IgM, six of whom (30%) presented evolution to FSGS, while of the remaining 65 IF-negative children, only three (4.6%) presented evolution to FSGS. Of the total 331 children with non-nephrotic proteinuria and/or hematuria, 139 were diagnosed as IgA–IgG nephropathy, 44 had positive IF for IgM and 148 were IF negative. Of the 44 children IF positive for IgM, seven (15.9%) presented evolution to FSGS, while none of the 148 IF-negative children presented evolution to FSGS. The follow-up time for all children ranged from 1 to 14 years. Conclusions: Of IgM nephropathy patients with MCNS and DMH, a significant percentage develop impaired renal function, due to the evolution of FSGS, as revealed by repeat biopsy during long-term follow up. [ABSTRACT FROM AUTHOR]

Background. Pure diffuse mesangial hypercellularity (DMH), in its primary form, is a relatively rare histological finding and few data exist in the literature regarding its clinical course and prognosis in nephrotic adults with this diagnosis. [ABSTRACT FROM PUBLISHER]

IgM nephropathy is a relatively rare cause of idiopathic nephrotic syndrome. It was initially described by van de Putte, then by Cohen and Bhasin in 1978, as a distinctive feature of mesangial proliferative glomerulonephritis. It is typically characterized by diffuse IgM deposits on the glomeruli and diffuse mesangial hypercellularity. Little is known about the pathogenesis and treatment of this disease. We describe a patient who presented with nonspecific symptoms of epigastric pain, nausea, and early satiety. Abdominal imaging and endoscopies were unremarkable. She was found to have significant proteinuria (6.4 g/24 hours), hyperlipidemia, and edema consistent with a diagnosis of nephrotic syndrome. Kidney biopsy was performed and confirmed an IgM nephropathy. Less than 2 weeks after her diagnosis of IgM nephropathy, she presented with an acute cerebellar stroke. Thrombophilia is a well-known complication of nephrotic syndrome, but a review of the literature failed to show an association between IgM nephropathy and acute central nervous system thrombosis. [ABSTRACT FROM AUTHOR]

Immunoglobulin M nephropathy (IgMN) is an idiopathic glomerulonephritis characterized by diffuse deposits of IgM in the glomerular mesangium. However, its renal prognosis remains unknown. We compared renal outcomes of IgMN patients with those of patients with minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), or mesangial proliferative glomerulonephritis (MsPGN) from a prospective observational cohort, with 1791 patients undergoing native kidney biopsy in eight hospitals affiliated with The Catholic University of Korea between December 2014 and October 2020. IgMN had more mesangial proliferation and matrix expansion than MsPGN and more tubular atrophy and interstitial fibrosis than MCD. IgMN patients had decreased eGFR than MCD patients in the earlier follow-up. However, there was no significant difference in urine protein or eGFR among all patients at the last follow-up. When IgMN was divided into three subtypes, patients with FSGS-like IgMN tended to have lower eGFR than those with MCD-like or MsPGN-like IgMN but higher proteinuria than MsPGN-like IgMN without showing a significant difference. The presence of hypertension at the time of kidney biopsy predicted ≥20% decline of eGFR over two years in IgMN patients. Our data indicate that IgMN would have a clinical course and renal prognosis similar to MCD, FSGS, and MsPGN. [ABSTRACT FROM AUTHOR]

Background. IgM nephropathy (IgMN) was first described in 1978 but whether IgMN is a distinct entity has not yet been confirmed. We tried to clarify the characteristics of IgMN on the basis of clinical and histological findings of patients who were treated and investigated by us. Methods. We evaluated 90 patients (group A) with mesangial proliferative glomerulonephritis (MesPGN) who exhibited more dominant mesangial deposits of IgM than of IgG and IgA. To clarify the significance of the IgM mesangial deposits, we divided these patients into two subgroups: group A1 comprised 30 patients with diffuse and global mesangial IgM deposits and group A2 comprised the other 60 patients, with focal segmental mesangial IgM deposits. Ninety Mes-PGN patients without predominant IgM deposits, excluding those with IgA nephropathy were studied as controls (group B). Results. Group A had significantly higher levels of serum IgM than group B ( P < 0.01). The mean values for glomerular filtration rate (GFR), serum albumin, and CH50 in group A were significantly lower than those in group B ( P < 0.01). Proteinuria in group A was significantly more severe than in group B ( P < 0.05). Group A had a significantly higher incidence of adhesion ( P < 0.01) and duplication ( P < 0.05) of capillary loops than group B. They also demonstrated a significantly higher incidence of glomerular deposition of C1q and/or C4,C3, and fibrinogen than group B ( P < 0.01). Conclusion. These findings suggest that the mesangial deposition of IgM is useful for assessing glomerular damage or renal dysfunction in MesPGN patients, regardless of the amount of mesangial IgM deposits. Furthermore, MesPGN with predominant IgM deposition, which includes both diffuse global and focal segmental deposition of IgM, should be referred to as IgM nephropathy. [ABSTRACT FROM AUTHOR]

Idiopathic nephrosis (IN), which includes minimal change (MCD), diffuse mesangial proliferation (DMP) and focal segmental glomerular sclerosis (FSGS), is classically characterized by the absence of significant deposits by immunofluorescence microcopy (IF), except for the focal lesions of segmental sclerosis and/or hyalinosis of FSGS, which fix IgM and C3 antiserums. Since IF is available in most centres, an increasing number of unexpected findings has been reported. In order to evaluate the clinical significance of the glomerular deposits revealed by IF in some instances, we reviewed the renal biopsy findings of 222 consecutive children presenting with IN and in whom IF microscopy was available. By light microscopy, 122 patients showed MCD, 10 DMP, and 90 FSGS with DMP (11 cases) or without (79 cases). By IF, 125 specimens were negative and served as controls; 54 showed mesangial IgM deposits, 24 mesangial IgG deposits (associated with Clq deposits in 16), 15 scattered granules of C3 and 4 predominant deposits of mesangial IgA. We correlated these findings with initial response to steroid therapy and outcome and could find no significant difference between the various categories defined by IF and the control group. Repeat biopsies, performed in 21 cases, showed the persistence of deposits in 11 and their transformation in 10. The particular problem raised by the patients who present with IN and mesangial IgA deposits is discussed. Our results demonstrate that patients presenting with IN and 'positive IF', whether showing IgM, IgG and Clq, C3 or IgA, do not represent distinct clinicopathological entities. [ABSTRACT FROM AUTHOR]

Mice have been found to be susceptible to spontaneous renal localization of immune deposits. However, the significance of these immune deposits is still debated. We investigated the immunopathogenesis of a naturally occurring glomerulopathy associated with progressive proteinuria and glomerulosclerosis in 75 BALB/c mice. The mice were divided into five groups of 15 and killed at the age of 1, 3, 6, 12, or 18 months for laboratory and renal pathologic studies. These mice showed persistently increasing serum levels of immune complexes, a marked increase of glomerular immune deposits which were capable of fixing C3, and interstitial infiltration of lymphocytes and plasma cells, followed by the occurrence of proteinuria, mesangiopathy, and glomerulosclerosis. Our findings suggest that an immune system mediated process occurred in the kidneys of the mice tested. [ABSTRACT FROM AUTHOR]

IgM nephropathy is one of the rare causes of glomerulonephritis, characterized by a clinical picture ranging from asymptomatic urinary disorders to proteinuria severe enough to cause nephrotic syndrome, and is more frequently seen in children and adolescents. Under a light microscope, there are minor changes and focal segmental glomerulosclerosis (FSGS), like mesangial proliferation in various degrees. Although IgM nephropathy is considered a refractory variant of minimal change disease (MCD), there is no agreement on the treatment of IgM nephropathy, and it seems that the success of treatment with steroids is not as effective as the treatment of MCD. In this report, we will present our experience with the treatment of a case of a patient with primary IgM nephropathy with steroids and cyclosporine. In this case, edema and ascites completely disappeared, and complete remission was obtained within 2 months of treatment with a combination of prednisolone and cyclosporine. We can suggest that a combination of cyclosporine and a steroid, together with recommendations generally made in nephrotic syndrome, can be useful in the absence of contraindications for the treatment of IgM nephropathy, which is rarely seen in adults. [ABSTRACT FROM AUTHOR]

IgM nephropathy presents with refractory nephrotic syndrome and its treatment is a significant challenge for pediatricians. We present two patients with IgM nephropathy and frequently relapsing nephrotic syndrome treated with rituximab and subsequently mycophenolate mofetil. Both showed complete remission, which 24 to 30 months later, was still maintained. The role of mycophenolate mofetil therapy in maintaining remission after successful treatment of rituximab in IgM nephropathy needs to be examined. [ABSTRACT FROM AUTHOR]

Objective: To find out the incidence and natural history of IgMN in India. Methods: Renal biopsies of children ≤12 y age of last 6 y were retrospectively evaluated. Their clinical and biochemical presentation were correlated. Patients with systemic diseases/disorders were excluded from the study. Results: Immunoglobulin M nephropathy (IgMN) constituted 11.9% ( n = 28) of 236 renal biopsies. Mean age was 10 y, predominant in boys ( n = 24), most of the patients presented with proteinuria and edema. The most common associated histopathological finding was mesangial proliferative glomerulonephritis (MePGN) in 60.7% ( n = 17) followed by minimal change disease (MCD) in 28.6% ( n = 8) and focal segmental glomerulosclerosis (FSGS) in 10.7% ( n = 3). In 85.7%, IgM appeared as the sole immunoglobulin deposit mainly in mesangial regions, followed by accompanied C3 in 3.6% ( n = 1) and C1q + C3 in 10.7% ( n = 3) biopsies. Conclusions: IgMN was observed in 11.9% biopsies with commonest morphology of MePGN followed by MCD and FSGS; proteinuria was bad prognosticator in addition to FSGS and co-deposition of other immunoglobulins had no significance. [ABSTRACT FROM AUTHOR]

Objective: To report a case with the diagnosis of IgM nephropathy and familial Mediterranean fever (FMF). Clinical Presentation and Intervention: A 9-year-old boy was admitted to our hospital with recurrent abdominal pain since the age of 4 years. Laboratory investigations revealed a sedimentation rate of 88 mm/h, C-reactive protein: 83.2 mg/l (0–10 mg/l), white blood cell count: 12,700/mm3, fibrinogen: 622 mg/dl (200–400 mg/dl) and serum amyloid A: 186 mg/l (0–5.8 mg/l). Urinalysis revealed +2 proteinuria. A 24-hour urinary protein excretion was 12 mg/m2/h. M694V homozygous mutation was identified in exon 10. Percutaneous renal biopsy showed mesangial cell proliferation and increased mesangial matrix in the glomeruli, without amyloid accumulation. Immunofluorescence study showed IgM (+1) and C1q (+1) deposits. Treatment with 1 mg/day colchicine was started. Six weeks later, proteinuria had disappeared and the patient was asymptomatic. Conclusion: This case illustrates the unusual association of FMF with non-amyloid glomerulopathy. Glomerular diseases such as IgM nephropathy may be seen as a manifestation of FMF. Copyright © 2008 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background: IgM nephropathy is a rare disease with variable clinical presentations and is an unusual cause of nephrotic syndrome. Histopathological findings typically include mesangial hypercellularity with IgM and complement deposition, though the spectrum may range from normal glomeruli through to focal and segmental glomerulosclerosis. Thromboembolism is a well recognised complication of nephrotic syndrome, but cerebral venous sinus thrombosis is rarely described.Case Presentation: This is the case of a 23-year-old male presenting with the nephrotic syndrome, whose initial renal biopsy was consistent with minimal change disease. Complete remission was achieved with prednisone, however multiple relapses and steroid dependence prompted re-biopsy, the results of which were more consistent with IgM nephropathy. His last relapse was complicated by cerebral venous sinus thrombosis. He then received rituximab and a weaning course of prednisone to again enter remission.Conclusions: This case highlights the need to consider IgM nephropathy in the differential diagnosis of nephrotic syndrome. Additionally, it emphasises the risk of thrombosis in patients with severe nephrosis. [ABSTRACT FROM AUTHOR]

Antiphospholipid syndrome (APS) is a disorder characterized by antiphospholipid antibody positivity, arterial or venous thrombosis, and fetal loss. In APS, renal as well as vascular and glomerular involvement is observed. Systemic lupus erythematosus and other connective tissue diseases should be excluded to diagnose primary APS. Immunoglobulin M (IgM) nephropathy is characterized by single or dominant IgM deposition in glomerular mesangium. It often presents with hematuria and proteinuria. In a 45-year-old female patient admitted to our clinic with diabetes mellitus and proteinuria, fundus examination did not reveal diabetic retinopathy but a high anticardiolipin IgM and venous thrombosis in the upper extremity were observed. Renal biopsy revealed IgM nephropathy. The patient was diagnosed with primary APS and IgM nephropathy. Cyclophosphamide and steroid treatment was started. Her proteinuria decreased as a result of the treatment. Although, it is reported in the literature that primary APS coexists with other glomerulonephritis, we did not detect coexistence of primary APS and IgM nephropathy. [ABSTRACT FROM AUTHOR]