Nondiabetic renal disease in patients with type 2 diabetes

Diabetic nephropathy (DN) is one of the major complications of type 2 diabetes mellitus (T2DM). The diagnosis of DN is mostly clinical. Kidney biopsy is indicated only if nondiabetic renal disease (NDRD) is suspected. This study is aimed to assess the prevalence of NDRD and to determine predictor and prognostic factors of DN, NDRD. It was a retrospective analytic study including T2DM patients in whom renal biopsies were performed at our department from 1988 to 2014. Seventy-five patients were included. Mean age was 52.7 years with sex ratio at 1.56. Renal biopsy findings were isolated NDRD in 33 cases, NDRD superimposed on DN in 24 cases, and isolated DN in 18 cases. Most common NDRD found were focal segmental glomerulosclerosis (21%) and membranous nephropathy (19%). Multivariate analysis showed that the absence of ischemic heart disease [odds ratio (OR) = 0.178, 95% confidence interval (CI) = 0.041–0.762], absence of peripheral vascular disease (OR = 0.173, 95% CI = 0.045–0.669), and presence of hematuria (OR = 7.200, 95%CI = 0.886–58.531) were independent predictors of NDRD. 24 patients reached end-stage renal disease 55% in DN group, 16% in DN associated to NDRD group, and 30% in NDRD group. The prevalence of NDRD found in our study confirmed usefulness of renal biopsy in patients with T2DM, especially in those without degenerative complications, hypertension, and insulin therapy

Encapsulating peritoneal sclerosis after kidney transplantation: Success of medical treatment

Encapsulating peritoneal sclerosis (EPS) is an infrequent but serious complication of long-term peritoneal dialysis (PD). EPS may become clinically apparent when patients are on PD (classical EPS) or after undergoing kidney transplantation (post-transplantation EPS). This presentation of EPS seems to occur shortly after kidney transplantation in former PD patients. In this report, we present our experience in our first case of patient diagnosed with EPS after kidney transplantation

Facteurs pronostiques de l’atteinte rénale au cours du Purpura Rhumatoïde de l’adulte au Centre Hospitalier et Universitaire de Tunis / Pronostic factors of Henoch Schönlein nephritis in Tunis university hospital: Pronostic factors of Henoch Schönlein nephritis in Tunis university hospital

Context and objective. IgA vasculitis is a leukocytoclastic vasculitis with IgA deposits characterized by the association of cutaneous, articular and digestive involvements. Renal involvement worsens the pronostic of the disease. The main objective of this work was to identify the risk factors of end-stage renal failure in Tunisian adults with IgA vasculitis. Methods. From 1975 to 2017, patients with IgA vasculitis and nephritis were studied retrospectively. All the patients benefited from a renal biopsy classified histologically according to Pillebout. Results. Thirty-four adult patients (mean age at nephritis onset: 39 ± 17.6y) mainly men, were included. Risk factors for progression to end stage renal disease were: edema (p=0.002), oligoanuria (p=0.003), initial renal impairment (p=0.001), anemia (p=0.010), hyperuricemia (p=0.015), class IV (p=0.018), crescents (p=0.018) and interstitial fibrosis (p=0.017). Conclusion. The outcome of renal involvement during IgA vasculitis is relatively poor. Renal involvement conditions the long-term prognosis of the disease. The identification of clinical, biological and histological risk factors of end stage renal failure would improve its management. Contexte et objectif. Le purpura rhumatoïde est une vascularite leucocytoclasique à dépôts d’IgA caractérisée par l’association de signes cutanés, articulaires et digestifs. L’atteinte rénale fait la gravité de la maladie. Le principal objectif de ce travail était d’identifier les facteurs prédictifs de la maladie rénale chronique stade 5 chez des patients ayant un Purpura Rhumatoïde avec atteinte rénale. Méthodes. Nous avons étudié de façon rétrospective les observations de patients ayant présenté un purpura rhumatoïde avec atteinte rénale colligés de 1975 à 2017. Les lésions histologiques ont été classées selon la classification de Pillebout. Résultats. Nous avons colligé 34 patients adultes atteints de Purpura Rhumatoïde avec atteinte rénale. L’âge moyen des patients était de 39 ± 17,6 ans avec une prédominance masculine. Les facteurs prédictifs de la Maladie Rénale Chronique stade 5 étaient : les œdèmes (p=0,002), l’oligoanurie (p=0,003), l’insuffisance rénale initiale (p=0,001), l’anémie (p=0,010), l’hyperuricémie (p=0,015), la classe histologique IV (p=0,018), les croissants (p=0,009) et la fibrose interstitielle (p=0,017). Conclusion. L’atteinte rénale au cours du Purpura Rhumatoïde conditionne le pronostic à long terme. Sa recherche doit être systématique afin de prévenir ou de ralentir l’évolution vers la maladie rénale chronique stade 5

Renal amyloidosis in ankylosing spondylitis: A monocentric study and review of literature

Secondary renal amyloidosis (RA) is the most common type of renal involvement in ankylosing spondylitis (AS). We assessed the epidemiologic and clinico-biological profile of AS patients with RA, to analyze treatment modalities and prognostic aspects, and to determine predictive factors of RA during AS. This was a retrospective study including 13 cases of RA among 212 cases who presented with AS, during the period from 1978 to 2006. The median age of the patients at the time of diagnosing AS was 47 years (range: 19–67). There were 11 males and two females. RA onset was diagnosed after a mean follow-up of 144.6 months (range: 10–505) from the AS diagnosis. We noted erosive peripheral arthritis, lumbar stiffness with bamboo spine, and coxitis in 23.1%, 76.9%, and 30.8% of cases, respectively. Nephrotic syndrome was found in eight patients (61.5%). At the time of diagnosing RA, six patients had renal failure. Amyloid deposits were histologically proven by salivary gland biopsy in six cases (46.1%) and by renal biopsy in seven cases (53.8%). Four patients received a long-course treatment with colchicine but with a good outcome only in two cases. In our series, AS was more severe among patients with RA. Four predictive factors of RA were identified: smoking (P = 0.04), erosive peripheral arthritis (P = 0.002), bamboo spine (P = 0.001), and biologic indicators of inflammation (P = 0.0001). High erythrocyte sedimentation rate was identified as the only independent risk factor of RA during AS (P = 0.0001). Renal function as well as urinalysis should be monitored at regular intervals to detect early renal involvement during AS

New familial cases of karyomegalic interstitial nephritis with mutations in the FAN1 gene

Abstract Background Karyomegalic interstitial nephritis (KIN) is a rare disease entity first described by Burry in 1974. The term KIN was introduced by Mihatsch et al. in 1979. KIN is characterized by chronic tubulointerstitial nephritis associated with enlarged tubular epithelial cell nuclei, which leads to a progressive decline of renal function. The prevalence of this disease is less than 1% of all biopsies, and its pathogenesis is unclear. KIN results from mutations in FAN1 (FANCD2/FANCI-Associated Nuclease 1), a gene involved in the DNA damage response pathway, particularly in the kidney. In this study, we report two Tunisian consanguineous families with KIN caused by mutations in the FAN1 gene. Methods Direct sequencing of the coding regions and flanking intronic sequences of the FAN1 gene was performed in three affected members. Three prediction programs (Polyphen-2 software, SIFT, and MutationTaster) were used to predict the functional effect of the detected variations. Results Two causative frameshift variants in the FAN1 gene were identified in each family: The previously described frameshift mutation c.2616delA (p.Asp873ThrfsTer17) and a novel mutation c.2603delT (p.Leu868ArgfsTer22) classified as "pathogenic" according to the American College of Medical Genetics and Genomics (ACMG) guidelines. Conclusion To our best knowledge, this is the first Tunisian study involving familial cases of KIN with mutations in the FAN1 gene. We hypothesize that these findings can expand the mutational spectrum of KIN and provide valuable information on the genetic cause of KIN