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Emphysematous pyelonephritis is a rare and severe infectious complication characterized by the presence of gas in the renal parenchyma, excretory cavities and surrounded tissues. It is due to the development of non-anaerobic gasifier bacteria. We report a new rare case of emphysematous pyelonephritis in a kidney transplant recipient, particular by its occurrence in a non-functional graft and its exceptional association with emphysematous cystitis.

Unexplained deep vein thrombosis may justify screening for antineutrophil cytoplasmic antibody‐associated vasculitis as it can be an unusual presentation of this disease.

BACKGROUND AND AIMS Acute interstitial nephritis (AIN) represents an important cause of acute kidney injury. The most common etiology of AIN is drug-induced disease. Definitive diagnosis of AIN requires renal biopsy. Despite this, there is a lack of consensus regarding precise histological diagnostic criteria, and the clinical significance of common histological findings is uncertain. The aim of this study was to explore the histological characteristics of drug-induced AIN and to evaluate their impact on the renal outcome. METHOD We conducted a retrospective study that included patients with biopsy proven drug-induced AIN, followed in the department of nephrology of Charles Nicolle hospital in Tunis during the period between 1990 and 2019. Histological diagnosis of AIN was based on the presence of an interstitial inflammatory cell infiltrate with or without fibrosis. RESULTS During the period of the study, we collected 69 cases of biopsy proven AIN. The diagnosis of drug-induced AIN was performed in 23 patients (33%), with a medium age of 35 years. In eight patients (35%), nonsteroidal anti-inflammatory drugs were the culprit, followed by antibiotics in six patients (26%) and other drugs in three cases (13%). In six patients (26%), no single drug could be identified as the culprit, and these were classified as multidrug causes. The presence of acute kidney disease was the main indication for the kidney biopsy. All cases were characterized by a prominent tubulointerstitial inflammatory cell infiltrate. Interstitial infiltration was mild in three cases (13%), moderate in five cases (17%) and severe in 15 cases (65%). The interstitial infiltrate consisted predominantly of lymphocytes. The presence of eosinophils has been noted in 10 cases (43%). Interstitial granulomas were noted in two patients (8%). Interstitial edema and tubulitis were observed in 13 (56%) and 6 cases (26%), respectively. In seven patients (30%) interstitial fibrosis was present, and four patients (17%) had mild-to-moderate tubular atrophy, whereas tubular necrosis was present in seven cases (30%). After a mean follow up of 24 months, eight patients (35%) progressed to chronic kidney disease. Histological factors associated with poor renal outcome, were interstitial fibrosis (P ˂ 0.001) and tubular atrophy (P = 0.01). Patients with interstitial edema (P ˂ 0.001) or tubulitis (P = 0.005) had a better renal prognosis. CONCLUSION Renal biopsy is required to establish a definitive diagnosis of drug-induced AIN. Nevertheless, it is not carried out in a systematic way. The composition of cells in the interstitial infiltrate may at times be helpful in determining the etiology of AIN. In particular, the presence of a considerable proportion of eosinophils favors a diagnosis of drug-induced AIN. Moreover, during drug-induced AIN, histological data have a great prognostic value. In our study, the main histological prognostic factors were interstitial fibrosis, tubular atrophy, interstitial edema and tubulits. Further studies are needed to validate these results and establish a score based on histological data in order to predict renal outcome during drug-induced AIN.

Proliferative glomerulonephritis with monoclonal immunoglobulin deposits is a new disorder with undefined treatment modalities. We propose cyclophosphamide‐bortezomib‐dexamethasone and autologous stem cell transplantation as a therapeutic protocol.

Introduction: Mycophenolate Mofetil (MMF) is an immunosuppressive drug usually used in kidney transplants to prevent rejection. It has various adverse effects such as leucopenia, anemia, diarrhea but Mouth ulcers are rarely reported. Methods: We present a case report of MMF-induced mouth ulcers in an African patient. Case Report: A 41-year-old African-male patient has painful oral ulcers which developed 5 months after kidney transplantation. The immunosuppressive maintenance regimen comprised Steroids, Tacrolimus and MMF. Results: These ulcers were firstly related to a fungic or viral infection so the patient was prescribed Fluconazole and Aciclovir without any improvement. Then, Tacrolimus blood level was checked and it was in a therapeutic range. Finally, we decide to stop MMF and the ulcers healed quickly. Discussion: Oral ulcers are frequently seen complications in immunosuppressant patient but are rarely described with MMF. These ulcers can become large and very painful and degrade patient's life quality. So when infections causes are excluded, we have to keep in mind that these ulcers can be a drug adverse effect.

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.

Introduction Meme si l’association entre infection a Cytomegalovirus (CMV) et rejet aigu (RA) apres transplantation renale (TR) est connue depuis plusieurs annees, les liens entre ces 2 complications demeurent toujours debattus. L’objectif de notre etude etait de rapporter notre experience en ce qui concerne l’association de ces 2 complications. Description Nous avons mene une etude longitudinale, retrospective, analytique, incluant les patients transplantes du rein entre 1986 et 2018. Methodes Notre population etait subdivisee en deux groupes : un groupe A comportant les TR compliquees d’au moins un episode de RA et un groupe B incluant celles qui n’ont jamais ete compliquees de RA. Resultats Parmi les 620 patients transplantes dans notre centre au cours de la periode d’etude, 129 (20,8 %) ont presente 149 episodes de RA (20 patients ont developpe un 2eme episode). L’infection a CMV etait plus frequente dans le groupe RA(+) (35,5 % contre 16,1 % dans le groupe RA(-), p 3 mois), p = 0,0016. Des signes histologiques de CMV ont ete notes dans 5 des 45 biopsies du greffon realisees pour suspicion de RA. L’infection a CMV etait un facteur de risque independant de survenue de RA apres etude multivariee (RR = 2,275 ; p = 0,0002). Trois episodes d’infection a CMV sont survenus precocement apres un episode de RA. Le delai etait de 20, 23 et 30 jours apres l’instauration du traitement antirejet. Conclusion L’infection a CMV est consideree comme un facteur de risque de survenue de RA apres TR. Elle doit etre prevenue pour diminuer l’incidence du RA essentiellement tardif. Par ailleurs, une infection a CMV peut compliquer un RA traite du fait de l’intensification de l’immunosuppression.

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.

Background and Aims Mesangial deposits Ig A was described the first time in 1968 by Berger and Hinglais. It remains the most common primary glomerulonephritis worldwide. It is often idiopathic but can also be secondary. The aim of our study is to describe the epidemiologic characteristics, the incidence and the anatomopathological features of 501 IgA nephropathy (IgA N) patients. Method It is a retrospective mono-centric study including patients having IgA N in the renal biopsy done in our department among a period of 17 years. Results We analyzed data of 8427 patients who underwent renal biopsy. 81% had glomerular nephropathy with 7.3% (501) IgA N. A male-to-female ratio of 2.27. The average age was 28.7 years. IgA N was primary in 80.2% cases and secondary in 17.8% cases. The most frequent secondary IgA N was rheumatoid purpura (74.8%). There was a male predominance in Berger‘s disease as well as in rheumatoid purpura. Berger’s disease was more common in adults, whereas rheumatoid purpura was more common in children. The main indication of renal biopsy was proteinuria with hematuria in 23.2% of cases and nephrotic syndrome in 23.8%. The association of non-nephrotic proteinuria, hematuria, arterial hypertension and renal injury was found in 9.3% whereas isolated macroscopic hematuria only in 6.4% of cases. According to HAAS classification, HAAS 3 was the most frequent. OXFORD classification used only from 2010, and M1, S1, E0, T0 and M1, S1, E0, T2 were the most frequent. Glomerular lesions were associated to tubulo interstitial and vascular lesions in 48.2% of cases. Conclusion IgA nephropathy is the most common glomerular disease and a frequent cause of end stage renal disease. Because of a clear increase of it’s incidence in our country and the delay in the diagnosis, a systematic screening of urines is needed in our country as it’s done in Singapore and Japan.

Background and Aims Anti-Glomerular basement membrane disease « anti-GBM » is a rare autoimmune disease. It most often results in a rapidly progressive glomerulonephritis syndrome associated with intra-alveolar hemorrhage. The diagnostic confirmation is histological, by the demonstration of linear deposits of immunoglobulins (IgG) along the glomerular basement membrane. The aim of our study was to describe its epidemiological, clinical, biological, immunological, histological, and evolutionary characteristics and to identify the different prognostic factors for predicting patient survival and renal survival. Method It was a retrospective, descriptive and analytical study including patients over 16 years old with anti-GBM disease proved by kidney biopsy and followed up over a period of 32 years (January 1985 to July 2017), in Internal Medicine Department of Charles Nicolle Hospital of Tunis. Results We collected 32 patients with a sex ratio H/F = 1.13. The mean age at diagnosis was 42 years old with extremes of 18 to 81 years old. The most frequent extra-renal manifestations were pulmonary (53%), neurological (12.5%) and ocular (6%) manifestations. Hematuria associated with proteinuria was constantly found. The latter was nephritic in 31% of patients. All patients had renal insufficiency on admission, oligo-anuric in 60% of cases. The use of extra-renal treatment at admission was necessary in 75% of patients. On the immunological level, the search for anti-MBG antibodies, performed in 20 patients, was positive in 65% of cases. All our patients had undergone a renal biopsy puncture with direct immunofluorescence study. Diffuse extra-capillary glomerulonephritis was observed in 93% of patients. Corticotherapy was initiated in 27 cases, associated with plasma exchange in 21 cases and cyclophosphamide in 17 cases. Nine of our patients died in the first year. Renal outcome was marked by partial remission in 2 cases and end-stage renal failure in 19 cases. In univariate analysis, an age greater than 60 years and the occurrence of respiratory distress were predictors of death. Oligo-anuria and need dialysis on admission were predictive factors for progression to end-stage renal failure. Conclusion Anti-GBM disease is a serious illness. Current therapeutic modalities have significantly improved patient survival. These must be intensive and rapid in order to hope for a favorable renal evolution whose prognosis remains reserved.

Background and Aims Hypertension is a common manifestation during systemic lupus erythematosus (SLE). Its mechanism is multifactorial and microthromboses of renal arterioles seem to be the most important mechanism. The objective of our study is to identify the histological and evolutionary characteristics of patients with lupus nephritis (LN) presenting with hypertension. Method A retrospective study of 85 patients followed for LES with lupus nephritis documented by a renal biopsy collected in 17 years and presenting with hypertension. Results Among 174 patients with LN, eighty-five (48.58%) are hypertensive. A sex ratio F / H of 6.08. The mean age of LN diagnosis was 36.4 years old [13 -75 years old]. The average time to onset of hypertension was 25.8 months [0-204 months]. Malignant hypertension was present in 12% of patients. Antiphospholipid Antibody Syndrome (APLS) was found in 35.3% of cases. Renal biopsy showed LN class II in 2 cases, class III in 8 cases, class IV in 43 cases, class V isolated in 8 cases and class VI in 3 cases. Vascular lesions were arteriolosclerosis in 40% of cases and thrombotic microangiopathy (TMA) lesions in 17.6% of cases. The treatment was essentially based on blockers of the renin angiotensin system, either as monotherapy or in combination with calcium channel blockers, beta blockers or central antihypertensives. The evolution was marked by the occurrence of cerebrovascular accidents associated in 7 cases with APLS and coronary artery disease in 2 cases. Renal evolution was marked by total and durable remission in 27.5%, chronic renal disease in 31.7%, and end-stage renal failure in 40.8% of cases. Blood pressure was balanced in 40,5 % of cases and unbalanced in 59,5% of cases. Conclusion In our lupus patients, hypertension was common, associated with severe glomerular and vascular lesions and a rather severe renal prognosis.

Background and Aims Systemic Lupus Erythematosus (SLE) is associated with an increased risk of cardiovascular morbidity and cardiovascular mortality. The risk of cardiovascular events is 1.3–2.7 times higher in SLE patients than in the general population, and even higher in patients with lupus nephritis (LN). Traditional risk factors as well as SLE-specific and treatment-related factors all contribute to the increased risk of cardiovascular disease. The primary aim of the present study was to evaluate cardiovascular risk factors, morbidity and mortality in patients with LN. Method This is a retrospective study of patients over the age of 16, with LN proved by kidney biopsy and followed up in our department over a period of 17 years. The diagnosis of lupus was made according to criteria of The American College of Rheumatology revised in 1997. Demographic, clinical and para-clinical data were collected from medical observations. Results We collected 155 women and 19 men with a sex ratio F / H of 8.2. The mean age at the time of the discovery of LN was 32.6 years [15-45 years]. Overall median follow-up time was 81.2 months. Renal symptomatology was dominated by proteinuria noted in all patients with an average proteinuria at 3.3 g / 24h, associated to a nephrotic syndrome in 68% of patients, hematuria was present in 69% of patients and renal failure was present in half of cases with an average serum creatinine of 110 µmol / l. At the time of diagnosis of LN, hypertension was noted in 48.9% of cases, diabetes in 2.8% of cases and obesity in 57.4% of cases with an index average body mass of 28.5 Kg / m2. Smoking was reported in 17.2% of the cases. The average cholesterol level was 5,5±2,1 mmol/l, the average triglycerid level was 2,5±1,1 mmol/. Antiphospholipid syndrome was found in 14.9% of cases. We performed 243 renal biopsies with 174 initial and 69 iterative biopsies. The histological lesions were polymorphic dominated by LN class IV (54.3%), arteriolosclerosis was observed in 47.7% and lesions of thrombotic microangiopathy in 29.8%. Corticosteroid therapy was prescribed in all patients combined with immunosuppressive therapy in 54.6% of cases. The overall survival of the patients at 10 years was 85%. During follow-up, cardiovascular complications found in our series were mainly strokes (6.3%) and coronary insufficiency (5.2%) and transient ischemic attack (6.9%). After a univariate analysis, the additional cardiovascular risk factors identified in our study were antiphospholipid syndrome (p = 0.01), renal failure (p = 0.01), long-term corticosteroid therapy (p = 0.009), the chronicity of the disease (evolution of lupus> 10 years) (p = 0.014), proliferative forms (p=0.001), arteriolosclerosis (p=0.0002) and lesions of thrombotic microangiopathy (p=0.018). Survival in patients without cardiovascular risk factors was better (96% vs 88%). Conclusion In conclusion, in addition to traditional risk factors SLE patients have several disease related risk factors that explain increase cardiovascular disease. A careful control for this risk factors is essential to continuously improve survival in SLE.

Background and Aims Crystal-induced kidney disease refers to kidney injury caused by intratubular crystal deposition. The most common forms of crystalline nephropathy encountered in renal pathology are nephrocalcinosis and oxalate nephropathy. The purpose of our study is to determine the epidemiological and clinical characteristics of hyperoxaluria diagnosed from renal biopsy. Method We retrospectively reviewed all kidney biopsy specimens evaluated at renal pathology laboratory, from 1976 to 2019. The biopsy specimens were received from multiple medical department and medical centers. We studied 8900 biopsy specimens and we were focused on patients whose diagnosis of hyperoxaluria was made from renal biopsy Results We identified 25 cases (15 children and 10 adults) with a sex ratio H / F of 0.9. Mean age at diagnosis was 17.2 years old [4 months-73 years old]. Most patients were offspring of consanguineous mating (14 of 25) with intermarriage of first-degree cousins being the most common pattern. A family history of chronic kidney disease was found in 13 patients: indeterminated nephropathy (n = 6) and renal stone (n = 5) and primary hyperoxaluria (n=2). Among our patients, five had a history of urolithiasis. One patient had a history of chronic diarrhea related to Crohn's disease and one patient had a history of cephalic pancreatectomy and ileal resection. Initial symptoms and signs were dominated by renal failure (n = 25) with mean creatinine of 789.5 μmol / l [306-1832μmol / l], associated with proteinuria in 10 patients and hematuria in 11 patients. Arterial hypertension was present in 4 patients. Oligo anuria was reported in 4 patients without dilation of the urinary excretory pathways. In our patients, the diagnosis of crystalin nephropathy was revealed by renal biopsy. In one case, the diagnosis was made after renal transplant. In 4 cases the diagnosis was made by postmortem kidney biopsy. In all cases, the kidney biopsy specimen showed extensive intratubular crystal deposition and tubulointerstitial mononuclear cell infiltration with features of tubular injury and interstitial fibrosis. Examination of histologic slides showed colorless refractile crystals of polygonal appearance. Multicolored birefringence under polarized light identified these crystals as calcium oxalate. After different investigations (genetic and biological analysis), the diagnosis of hyperoxaluria was confirmed. Hyperoxaluria was primary in 23 patients and secondary in 2 patients. Conclusion Hyperoxaluria is a rare condition, often serious, involving renal prognosis and sometimes life-threatening, especially in early-onset forms. Early diagnosis and treatment should be done as soon as possible to slow the progression to end-stage renal failure. In patients with renal insufficiency, the diagnosis of hyperoxaluria is difficult. Renal biopsy can help when clinical and radiological data are not sufficient.

Introduction Le rejet aigu (RA) est une complication immunologique redoutable apres transplantation renale (TR). L’objectif de notre etude etait de determiner les aspects epidemiologiques et demographique du RA dans une population de transplantes renaux. Description Nous avons mene une etude longitudinale, retrospective, analytique, incluant les 620 patients transplantes du rein entre 1986 et 2018. Methodes Notre population etait subdivisee en deux groupes : un groupe A comportant 129 TR (20,8 %) compliquees d’au moins un episode de RA et un groupe B incluant 491 TR qui n’ont jamais presente de RA. Resultats On a note 149 episodes de RA chez 129 patients (20 patients, soit 15,5 %, ont presente un 2e episode) dont 70,5 % etaient precoces (≤ 3 mois) et 57,7 % au cours du premier mois post TR. L’incidence globale du RA etait de 2,43/100 patients/annee. Une baisse de l’incidence du RA a ete notee au fil du temps passant de 55,4 % (entre 1986 et 1990) a 4,4 % (entre 2010 et 2018) (p Conclusion Le RA est une complication multifactorielle. Il doit etre prevenu, diagnostique et traite a temps pour eviter les sequelles a court, moyen et long terme.

Introduction La microangiopathie thrombotique (MAT) serait frequente au cours de la nephropathie a IgA (NIgA), jusqu’a 53 % de prevalence. La presence de MAT influence-t-elle la valeur pronostique du score MESTC ? Description Nous avons selectionne les cas de NIgA biopsies entre1995 et 2017. Nous avons inclus les patients âges de plus de 16 ans ayant au moins une lesion de MAT a leur biopsie renale. Les NIgA secondaires et les vascularites a IgA ont ete exclues. Methodes Le test Chi2 a ete utilise pour l’etude d’association du MESTC avec la dialyse [IC = 95 %, p Resultats Cinquante dossiers ont ete inclus : âge = 34 ans ± 10 ; proteinurie = 3,2 g/24H ± 1,8 ; clairance creatinine mediane = 23 mL/min/1,73 m2. Des lesions M1, E1, S1, T1, T2, C1 et C2 ont ete observees respectivement dans 60 %, 8 %, 88 %, 16 %, 70 %, 10 % et 14 % des cas. Le score cumule MESTC etait egal a un, deux, trois, quatre, cinq, six et sept respectivement dans 8 %, 12 %, 30 %, 32 %, 8 %, 10 % et 0 % des cas. La dialyse etait significativement associee a : la fibrose interstitielle (T1 ou T2) (p = 0,02 ; RR = 8,3) en particulier T2 (p Fig. 1 ). Conclusion Le score cumule MESTC et la presence de lesion T2 sont des elements pronostiques eventuels en cas de NIgA primitive avec MAT.

Introduction: Vitamin D deficiency is frequently observed among dialysis patients. Previous studies suggested that 50 to 90% of end-stage renal disease patients are deficient in vitamin D. In Tunisia, studies regarding hypovitaminosis D in patients on dialysis are not numerous. Actually, many data support the use of native vitamin D in hemodialysis (HD) patients. In Tunisia, using native vitamin D is not part of therapeutic habits of all dialysis centers. Objectives: The aim of this study was to determine the prevalence of vitamin D deficiency in patients with chronic kidney disease stage 5 undergoing HD and to evaluate the effect of oral cholecalciferol supplementation, in intact parathormone (iPTH), serum calcium and serum phosphorus. Patients and Methods: We conducted a pre-experimental study among HD patients. Monthly oral supplementation with Cholecalciferol, was instituted for six months. Results: Forty-three participants were included. The mean 25-hydroxy vitamin D concentration was 17.89 ng/mL. Vitamin D deficiency was observed in 83.7% of our patients. We observed a significant increase in 25-hydroxy vitamin D and calcium levels and a significant decline in iPTH levels. No evidence of toxicity, nor severe hypercalcemia or hyperphosphatemia was noted. Conclusion: The supplementation with cholecalciferol seems reasonable and well tolerated in HD patients if reasonable doses are used with regular monitoring.

BackgroundPrimary membranous nephritis (PMN) is an autoimmune disease induced by the deposit of antibodies (Ab) to the phospholipase receptor A2 receptor (PLA2R) on podocytes. In this context, we aimed to assess the relationships between anti-PLA2R Ab, PLA2R rs4664308 SNP, PLA2R mRNA levels and PMN susceptibility and outcome.MethodsSixty-eight PMN patients, 30 systemic lupus erythematosus (SLE) patients with secondary MN and 30 healthy control subjects served for anti-PLA2R Ab measurement by ELISA and PLA2R rs4664308 SNP genotyping by a commercial real-time PCR. Twenty patients with tubulo-interstitial nephritis (TIN) were used as controls for renal PLA2R mRNA quantification in PMN patients from kidney biopsies. PLA2R mRNA quantification was carried-out by real-time PCR after RNA extraction.ResultsForty-three (63.2%) PMN patients received initial therapy consisting of alternating monthly cycles of corticosteroids and cyclophosphamide. Twelve (17.6%) patients had resistant PMN to initial therapy and were consecutively treated by cyclosporine or tacrolimus. Anti-PLA2R Ab were positive in 54 (79.4%) PMN patients, while all SLE patients and controls were negative, pConclusionAnti-PLA2R Ab and renal PLA2R mRNA could be useful markers for PMN outcome predicting. The PLA2R rs6446308 SNP is associated with PMN susceptibility in Tunisians.

Introduction La prevalence de la microangiopathie thrombotique (MAT) au cours de la nephropathie a IgA (NIgA) varie beaucoup selon les etudes (de 1,5 a 53 %). Deux etudes recentes viennent de l’evaluer a 20 %. Description Nous avons determine la prevalence et l’incidence annuelle de la MAT au cours de la NIgA primitive dans un centre de reference et nous avons degage les caracteristiques epidemiologiques en cas de MAT. Methodes Nous avons selectionne les dossiers de tous les adultes biopsies et suivis a notre centre pour NIgA primitive de 1995 a 2017. Ont ete exclus de cette etude, les patients suivis pour moins de 3 mois et chez qui une NIgA secondaire n’a pas ete elimine. Nous avons ensuite etudie les dossiers des patients ayant au moins une lesion de MAT a la biopsie renale. Resultats En 22 ans, le diagnostic de NIgA a ete porte chez 519 patients, dont 238 suivis a notre centre. Il s’agissait d’une NIgA primitive dans 186 cas, associee a une lesion de MAT dans 50 cas ; soit une prevalence de la MAT au cours de la NIgA primitive de 26,8 %. L’incidence etait de 2,5 cas/an, elle augmente a 3,5 cas/an a partir de 2005. Le groupe avec MAT avait un âge moyen de 34 ans [18-63] et un sexe ratio H/F de 1,94. Vingt huit pourcent des patients etaient issus d’un mariage consanguin, 50 % etaient tabagiques, 30 % avaient un niveau socio-economique bas et 38 % etaient depourvus de couverture sanitaire. La survie renale moyenne etait de 2,5 ans avec une mediane egale a un an. Conclusion La MAT est une lesion frequente au cours de la NIgA primitive. Une etude de l’impact clinique et pronostique d’une telle lesion est necessaire pour pouvoir discuter de leur eventuelle place dans la classification d’Oxford.

Introduction L’infection par le virus de l’hepatite C (VHC) est une pathologie systemique qui peut s’associer a des manifestations extrahepatiques, notamment l’atteinte renale. Methodes Le but de notre etude etait de decrire les differentes atteintes renales objectivees a la biopsie renale associees au cours infection par le VHC. Etude retrospective menee de 1992, date de debut de pratique de serologie du VHC, a 2018, qui a inclus les patients porteurs de VHC et qui ont beneficie d’une ponction biopsie renale. Resultats obtenus ou attendus Nous avons collige 18 patients, sexe ratio H/F 2,6, d’âge moyen de 56,1 ans [21–73 ans]. Seulement 2 patients etaient connus VHC positifs avant la decouverte de l’atteinte renale. Les signes extra-renaux etaient : une neuropathie peripherique chez 7 patients, une cytolyse chez 2 patients. l’hypertension arterielle etait retrouvee dans 83,3 % des cas. La proteinurie etait positive dans 94,4 % des cas, la proteinurie moyenne etait de 3,6 g/24 h associee dans la moitie des cas a un syndrome nephrotique, l’hematurie etait positive dans 83,3 % des cas, l’insuffisance renale etait retrouvee dans 72,2 % des cas avec une creatinine moyenne de 300,4 μmol/l. l’anemie etait notee chez 14 patients, une hypergamma-globulinemie a base large etait retrouvee chez 7 patients. Les lesions histologiques observees etaient dominees par la glomerulonephrite membranoproliferative (GNMP) type I dans 11 cas associee a une cryoglobulinemie mixte dans 2 cas, les lesions de hyalinose segmentaire et focale etaient observees chez 3 patients, une glomerulonephrite extra-membraneuse chez 2 patients et une glomerulonephrite aigue chez un patient. Conclusion Le depistage de l’atteinte renale chez les patients porteurs de VHC et inversement la recherche d’une infection a VHC en cas d’une atteinte glomerulaire doivent etre systematiques afin d’etablir une strategie therapeutique specifique associee a un traitement anti viral qui permettrait de controler la maladie renale.

Introduction La pancytopenie est une urgence medicale pouvant mettre en jeu le pronostic vital vue le risque infectieux et le risque hemorragique. La prise en charge therapeutique doit etre rapide et depend essentiellement de l’etiologie sous-jacente. Le but de ce travail etait de determiner les caracteristiques cliniques, biologiques, etiologiques, therapeutiques et evolutives des pancytopenies chez des malades hospitalises dans un service de medecine interne. Patients et methodes Nous avons mene une etude retrospective et descriptive portant sur les dossiers de 52 patients ayant presentes une pancytopenie et ayant ete hospitalises dans notre service. Resultats Il s’agissait de 16 hommes et 36 femmes dont l’âge moyen etait de 48 ans avec des extremes allant de 19 ans a 83 ans. L’examen clinique avait objective un syndrome anemique dans 32 cas (61 %), une fievre dans 18 cas (34 %), une splenomegalie dans 16 cas (30 %) et une hepatomegalie dans 10 cas (19 %). Un syndrome hemorragique etait observe chez 12 patients (23 %). Sur le plan biologique, les autres manifestations associees a la pancytopenie etaient un syndrome inflammatoire biologique dans 18 cas (34 %), une hypergammaglobulinemie dans 14 cas (26 %) et une perturbation de bilan hepatique dans 7 cas (13 %). L’anemie etait de type normochrome normocytaire dans 35 cas (67 %), microcytaire dans 11 cas (21 %) et macrocytaire dans 6 cas (11 %), avec un taux d’hemoglobine moyen a 6,7 g/dL (extremes allant de 2,2 g/dL a 11,4 g/dL). Le taux de plaquettes moyen etait de 71800 elements/mm3, avec des extremes allant de 7000/mm3 a 145000/mm3. Le taux moyen des leucocytes etait de 2790 elements/mm3 avec des extremes allant de 570/mm3 a 3800/mm3. Une neutropenie etait notee dans 22 cas (42 %) et une lymphopenie dans 33 cas (63 %). La ponction sternale a ete realisee dans 28 cas (53 %) et la moelle etait riche d’aspect normal dans 11 cas (39 %). L’enquete etiologique a revele un hypersplenisme secondaire a une hypertension portale dans 13 cas (25 %). Une neoplasie a ete diagnostiquee dans 7 cas (13 %) et etait a type de : myelome multiple (2 cas), leucemie aigue (3 cas), lymphome (1 cas) et liposarcome myxoide (1 cas). Une cause infectieuse a ete retrouvee dans 6 cas (11 %) et il s’agissait d’une leishmaniose viscerale dans deux cas, une endocardite infectieuse dans un cas, une septicemie a staphylocoque dans deux cas et un sepsis a point de depart urinaire dont le germe n’a pu etre identifie dans un cas. La pancytopenie etait en rapport avec un lupus erythemateux systemique dans 6 cas (11 %). L’etude cytologique de la Ponction sternale a permis de retenir le diagnostic d’un syndrome myelodysplasique dans 2 cas (3 %) et d’un deficit vitaminique dans 4 cas (7 %). Une origine medicamenteuse a ete retenue dans 5 cas (9 %). Un syndrome hemolytique et uremique a ete mis en evidence dans un cas. Aucune cause n’a ete retrouvee dans 8 cas (15 %). Dix-sept patients (32 %) ont necessite la transfusion par des culots globulaire, 4 patients par des culots plaquettaires (7 %) et un patient par du plasma frais congele. Huit patients sont decedes (15 %), dont 5 suite a un etat de choc septique (9 %), 2 suite a etat de choc hemorragique (3 %) et un par une cause indeterminee. Conclusion La pancytopenie presente un motif frequent de consultation en medecine interne, le plus souvent revelee par un syndrome anemique et plus rarement par des complications infectieuses ou hemorragiques. L’examen cle reste la ponction sternale qui va guider la demarche diagnostique et etiologique.

Introduction La frequence de l’atteinte renale au cours de syndrome de Sjogren (SS) varie selon les series de 2 % a 67 %. L’atteinte la plus typique est l’acidose tubulaire distale. Les autres atteintes qui peuvent ete retrouvees, sont l’acidose tubulaire proximale, le syndrome de Gitelman et le syndrome de Bartter dont nous rapportons une nouvelle observation. Observation Il s’agissait d’une femme âgee de 60 ans, qui a consulte pour un syndrome sec buccale et oculaire evoluant depuis 6 ans associe a des paresthesies des 2 membres inferieurs et des crampes musculaires. Les explorations faites ont conclues a un SS devant une xerostomie, xerophtalmie, un break up time altere, des anticorps antinucleaire type anti SSA positif et une sialadenite lymphocytaire stade 3 de Chisholm a l’etude anatomopathologique de la biopsie de glandes salivaires accessoires. Par ailleurs, elle presentait a la biologie une hypokaliemie a 3 mmol/l confirmee a 2 reprises avec une hyperkaliurese a 68 mmol/24H associees et une hypercalciurie a 7,74 mmol/24H et a une alcalose metabolique. Le diagnostic de syndrome de Bartter associee au SS a ainsi ete retenu. La patiente a ete traitee par une supplementation potassique par voie orale, gel larmes, larmes artificielles et de la Bromhexine avec une evolution favorable. Conclusion Les formes secondaires de syndrome de Bartter sont exceptionnelles. Quelques cas ont ete rapportes dans la litterature et etaient secondaires a : un SS, une prises d’aminosides, une tuberculose et une sarcoidose. Le diagnostic est biologique, confirme devant l’association d’une hypokaliemie, une alcalose metabolique, une hyperkaliuerese et une hypercalciurie.

Introduction La glomerulonephrite a depots non organises non Randall d’immunoglobuline monoclonale (PGNMID) est une gammapathie monoclonale de signification renale de decouverte recente. Elle est rare et son etiopathogenie n’est pas encore connue. Nous rapportons un nouveau cas de PGNMID associe a un myelome multiple. Observation Mme SC âgee de 57 ans, hypertendue, operee pour cancer du sein gauche, pour lequel elle a recu une chimiotherapie, une radiotherapie locoregionale et une hormonotherapie adjuvante, etait admise pour bilan etiologique d’œdemes des membres inferieurs evoluant dans un cadre d’alteration de l’etat general. Elle rapportait par ailleurs des epigastralgies, et un amaigrissement non chiffre. L’examen physique avait objective une hypertension arterielle a 150/80, des œdemes des membresinferieurs blancs mous gardant le godet et une hematurie a trois croix a la bandelette urinaire. Le bilan biologique avait montre un syndrome inflammatoire, une insuffisance renale aigue avec une clearance a 18,3 ml/mn (MDRD), une anemie hypochrome microcytaire, un syndrome nephrotique, une proteinurie de 24H a 7,5 g et une gammapathie monoclonale de type IgG kappa avec diminutiondes IgA. Le bilan phosphocalcique etait sans anomalies. Au cours de l’hospitalisation, la fonction renale de la patiente s’est rapidement degradee avec apparition d’une oligoanurie, necessitant le recours a des seances d’hemodialyse. La ponction sternale avait objective une plasmocytose a 12 %. Le diagnostic de myelome multiple symptomatique stade 3B selon Durie et Salmon a ete pose. Devant le syndrome nephrotique avec insuffisance renale, une ponction biopsie renale (PBR) a ete realisee. La PBR avait montre une glomerulonephrite membranoproliferative, avec a l’immunofluorescence des depots non organises d’IgG, kappa et de C3, rouge congo negatifs, de localisation glomerulaire exclusive, cadrant avec une PGNMID. La cryoglobulinemie etait negative. Le taux serique de C3 etait bas. Notre patiente a eu un traitement d’induction par bortezomib, dexamethasone et thalidomide, suivi d’une autogreffe de cellules souches hematopoietique. Des les premieres semaines de traitement, une amelioration de la fonction renale et de l’albuminemie etait notee, avec une reduction de la proteinurie et reprise de la diurese. Une reponse hematologique a ete objectivee au myelogramme et a l’ immunoelectrophorese. Discussion La PGNMID est une gammapathie monoclonale de signification renale rare, le plus souvent constituee d’une immunoglobuline entiere, IgG3 kappa. Pour retenir le diagnostic, il faut eliminer d’abord les autres glomerulonephrites a depots monoclonaux d’immunoglobulines : amylose, maladie de Randall et cryoglobulinemie. Notre patiente, aux antecedents de cancer du sein, presente une PGNMID associee a un myelomemultiple. Dans la litterature, l’association documentee de la de PGNMID avec une hemopathie maligne ou une neoplasie solide n’est pas frequente : seulement six cas de myelome multiple associe et cinq cas de neoplasie solide associee (2 cancer du colon, 2 cancers anorectaux, un cancer du sein avec un cancer de la vessie) ont ete decrits. La reprise de la diurese de notre patiente, la normalisation de son bilan renal et de son albuminemie ainsi que la normalisation de l’immunoelectrophorese et du myelogramme traduisent une remission hematologique et renale. Conclusion La PGNMID est une entite de decouverte recente, qu’il faut evoquer devant un syndrome nephrotique. La confirmation du diagnostic repose sur la PBR. Un traitement ciblant le clone B sous-jacent, parfois non detectable par les techniques usuelles, est indispensable pour preserver le pronostic renal.

Introduction Les atteintes glomerulaires au cours du myelome multiple (MM) sont variees (amylose AL, maladie de Randall). Les proprietes intrinseques des immunoglobulines sont responsables de la diversite des lesions renales. La glomerulonephrite membranoproliferative type I (GNMP) n’est que rarement decrite au cours du myelome. Nous rapportons un cas de GNMP en rapport avec un MM. Observation Il s’agit d’un patient âge de 52 ans hypertendu sous quadritherapie, suivi depuis mars 2014 pour un MM a IgG Kappa stade IIIa. Il a ete traite par 4 cycles de thalidomide-dexa puis autogreffe de moelle osseuse en mars 2015. L’evolution etait favorable avec persistance d’une reponse stable. En mars 2016, apparition d’un syndrome nephrotique (SN) impur par l’hematurie et l’insuffisance renale avec une creatininemie a 445 umol/L et proteinurie a 12 g/24 h, concomitant a une rechute de son MM. Devant le SN survenant dans le cadre de cette dysglobulinemie, une biopsie des glandes salivaires accessoires a ete pratiquee et n’a pas montre de depots amyloides. La ponction biopsie renale a conclu a une GNMP type I associee a d’importantes lesions tubulo-interstitielles sans stigmates de tubulopathie myelomateuse. A l’immunofluorescence presence de depots granuleux parietaux parsemes dans tous les glomerules d’IgG et C3 associes a des depots endomembraneux d’IgM, C1q et chaines legeres a predominance Kappa. Le dosage du complement C3 serique etait abaisse. Le bilan immunologique Anticorps antinucleaires etait negatif. Le traitement specifique du MM a ete repris et l’evolution etait marquee par l’amelioration progressive des chiffres de creatininemie atteignant 162 umol/L en decembre 2017 et baisse significative de la proteinurie a 1,75 g/24 h. Discussion Ce cas combine des facteurs pronostiques propres au MM et a la GNMP. Les Criteres de mauvais pronostic (CMP) propres au MM sont la masse (stade IIIB), hypoalbuminemie et la presence d’anomalies cytogenetiques de mauvais pronostic. Les CMP propres a la GNMP : sont l’insuffisance renale, le SN intense et l’hypertension arterielle severe. L’instauration conjointe d’une therapeutique immunosuppressive urgente a renverse le pronostic et a modifie le tournant evolutif de la maladie chez notre patient. Conclusion La GNMP type I est rare au cours du MM. Chez notre patient elle a bien evolue sous traitement specifique. Ceci doit encourager la pratique de la PBR devant toute atteinte renale glomerulaire secondaire au MM.