[Fibrillary glomerulonephritis]. / La glomérulonéphrite fibrillaire.

Fibrillary glomerulonephritis (FGN) is a glomerular disease described since 1977, with a prevalence in renal biopsies of less than 1%. It presents as renal failure, proteinuria, haematuria and hypertension in middle-aged adults. It is defined histologically, using light microscopy, which reveals organised deposits of fibrils measuring around 20nm, which are negative for Congo red staining. Electron microscopy, the first gold standard for diagnosis, has now been superseded by immunohistochemistry using the anti-DNAJB9 antibody. The discovery of this molecule has revolutionised the diagnosis of GNF, thanks to its excellent sensitivity and specificity (98% and 99% respectively). The association of GNF with hepatitis C virus, autoimmune diseases, neoplasia or haemopathy is debated. Renal prognosis is guarded, with 50% of patients progressing to end-stage renal failure within 2 to 4years of diagnosis. In the absence of randomised controlled trials, the recommended treatment is based on nephroprotective measures, corticosteroid therapy and possibly a second-line immunosuppressant such as rituximab. After renal transplantation, recovery or recurrence is possible. The pathophysiology of the disease is still poorly understood, and further studies are needed.

[A rare cause of AA amyloidosis: Hereditary epidermolysis bullosa]. / Une cause rare de l'amylose AA : les épidermolyses bulleuses héréditaires.

INTRODUCTION: Recessive dystrophic epidermolysis bullosa is a rare genetic condition characterized by fragile skin and mucous membrane, caused by mutations in the COL7A1 gene. AA amyloidosis is a rare complication of these genodermatosis. OBSERVATIONS: Two patients with recessive dystrophic epidermolysis bullosa, generalized severe in the first case and generalized intermediate in the second case, developed at the age of 38 and 28, respectively, nephrotic syndrome. The diagnosis of secondary renal amyloidosis was confirmed by renal biopsy in the first case and by minor salivary gland biopsy in the second case. Death occurred 2 months after diagnosis in both cases. CONCLUSION: Renal involvement is quite common in AA amyloidosis in patients with recessive dystrophic epidermolysis bullosa. Nephrotic syndrome and rapid decline in renal function renal are characteristic features. The prognosis is poor due to underlying conditions and the lack of an etiological treatment.

[Minimal change nephropathy and IgA deposits associated with a Sezary syndrome]. / Néphropathie à IgA et LGM au cours d'un syndrome de Sézary.

INTRODUCTION: The Sézary syndrome (SS) is an aggressive form of cutaneous T-cell lymphoma (CTCL) requiring a rapid diagnosis due to its poor prognosis. CASE REPORT: We report the first case of an eighty-nine-year-old woman who presented with concomitant Sezary syndrome and anasarca, revealing a nephrotic syndrome caused by a minimal change nephropathy associated with immunoglobulin A (IgA) deposits. Scarce literature described rare cases associating these two entities (nephrotic syndrome and nephropathy). However, the nephrotic syndrome was delayed from disease onset, secondary to immunosuppressive treatment of SS, or due to the weaning of SS therapy. Thus, the direct link between the glomerular lesion and the cutaneous lymphoma was difficult to establish. However, the synchronous occurrence of both SS and glomerulopathy in our patient, along with Sezary cells in both urines (urinary cytology) and biopsy, and resolution of nephropathy after treatment of SS, support the likely attributability of SS in glomerulopathy. CONCLUSION: Practitioners must acknowledge the possible occurrence of glomerular involvement in SS.

[Vaccine recommendations for children with idiopathic nephrotic syndrome]. / Recommandations vaccinales pour les enfants avec un syndrome néphrotique idiopathique.

The specific treatment of idiopathic nephrotic syndrome is based on corticosteroid therapy and/or steroid-sparing immunosuppressive agents in children who are steroid-dependant or frequent relapsers (60-70 %). Patients have an increased infectious risk not only related to the disease during relapses (hypogammaglobulinemia and urinary leakage of opsonins) but also to treatments (corticosteroids or immunosuppressive agents) in period of remission. Vaccination is therefore particularly recommended in these patients. Potential vaccine risks are ineffectiveness, induction of vaccine disease and relapse of idiopathic nephrotic syndrome. Only live vaccines expose to the risk of vaccine disease: they are in general contra-indicated under immunosuppressive treatment. The immunogenicity of inactivated vaccines is reduced but persists. The immunogenic stimulus of vaccination may in theory trigger a relapse of the nephrotic syndrome. Nevertheless, this risk is low in the literature, and even absent in some studies. The benefit-risk ratio is therefore in favor of vaccination with respect to the vaccination schedule for inactivated vaccines, with wide vaccination against pneumococcus and influenza annually. Depending on the context and after expert advice, immunization with live vaccines could be discussed if residual doses/levels of immunosuppressive treatments are moderate and immunity preserved.

[A patient with rheumatoid arthritis presenting a nephrotic syndrome: A case report]. / Un syndrome néphrotique chez un patient porteur d'une polyarthrite rhumatoïde : à propos d'un cas.

A fifty-one years-old patient with a history of rheumatoid arthritis of recent diagnosis is hospitalized for exploration of a rapidly progressive anasarca state. First analysis discovered an impure nephrotic syndrome (acute renal failure, hematuria) and massive glomerular proteinuria. Auto-medication by nonsteroidal anti-inflammatory drug was revealed. Renal biopsy showed minimal glomerular disease and acute tubular necrosis. Corticosteroid use permitted a normalization of proteinuria and renal recovery was obtained. Literature review showed renal impairment occurring in rheumatoid polyarthritis. Minimal glomerular disease is rare but can be associated with rheumatoid arthritis. This disease, associated with the use of nonsteroidal anti-inflammatory drug, may be responsible of the patient condition.

[Coronary involvement and nephrotic syndrome in systemic lupus: A case report]. / Atteinte coronarienne et syndrome néphrotique au cours du lupus systémique : à propos d'une observation.

INTRODUCTION: Heart failure during systemic lupus erythematosus has various causes. CASE REPORT: A 29-year-old female presented with a systemic lupus flare and a nephrotic syndrome, followed by cardiogenic shock requiring extra-corporeal membranous oxygenation. Ventricular dysfunction was related to massive myocardial infarction due to an anterior interventricular artery thrombosis and an underlying atheroma. The young age and the absence of chest pain were not suggestive of coronary artery disease initially. Coronary thrombosis was probably favored by the nephrotic syndrome, in which the arterial thrombotic risk is increased. CONCLUSION: Coronary artery disease should be systematically evoked in the presence of ventricular dysfunction in patients with systemic lupus, including when they are young and in the absence of chest pain. Nephrotic syndrome should be identified as a risk factor for arterial thrombosis.

[Up to date of pathophysiology mechanism of idiopathic nephrotic syndromes: Minimal change disease and focal and segmental glomerulosclerosis]. / Actualité sur les mécanismes physiopathologiques des syndromes néphrotiques idiopathiques : lésions glomérulaires minimes et hyalinose segmentaire et focale.

Idiopathic nephrotic syndrome represents up to 30% of adult glomerulopathies. However, its prognosis according to remission, relapse and renal failure remains unchanged since the 80s and prediction remains difficult. Physiopathology of adult idiopathic nephrotic syndrome is complex and multifactorial, including immunologic and environmental factors and a putative permeability-circulating factor, still unknown. In this point of view, we propose to summarize actual knowledge about idiopathic minimal change disease and focal and segmental glomerulosclerosis physiopathology.

[Berger's disease associated to an adalimumab-treated rheumatoid arthritis]. / Maladie de Berger au cours d'une Polyarthrite Rhumatoïde traitée par Adalimumab.

Berger's disease is characterized by deposits of immunoglobulin A in the glomerular mesangium. We report a case of a patient who was treated by adalimumab for a rheumatoid arthritis (RA). The patient is 45 years old and is treated for RA since 1996. Adalimumab was started after the failure of several treatments. Biological and clinical response to adalimumab were excellent. A nephrotic syndrome was diagnosed during the patient follow-up. Adalimumab was stopped since it was suspected to be responsible of these symptoms. Berger's disease was diagnosed thanks to a renal biopsy. The nephrotic syndrome was treated with corticosteroids, then tocilizumab was used to treat RA. TNF-alpha inhibitors are well known for inducing kidneys' adverse reactions (ADR). Usually, they appear shortly after the beginning of a treatment. Adalimumab has already been described in studies for inducing similar kidneys' adverse drug reactions. These ADR are often associated with systemic disease outbreak. It is difficult to assert that adalimumab or RA was responsible of the ADR that we noticed in our patient. It is usually admitted that these ADR are uncommon when the RA is controlled.

[IgA nephropathy: Unusual forms]. / Formes rares de néphropathie à IgA.

IgA nephropathy can have various initial presentation and evolutive characteristics. In this article, specific forms of IgA nephropathy are described, such as hypertensive emergency, nephrotic syndrome, rapidly progressive glomerulonephritis, monotypic IgA deposits, or IgA nephropathy associated with inflammatory diseases. Identification of these specific forms is needed to better characterize and treat these rare pathologies.

[Pulmonary embolism as a presentation of nephrotic syndrome]. / Embolie pulmonaire révélant un syndrome néphrotique.

BACKGROUND: Nephrotic syndrome (NS) in adults is defined by proteinuria>3g/24h or 50mg/kg/d, hypoproteinemia<60g/24h and hypoalbuminemia<30g/L. The final diagnosis is guided by the histopathology evidence when a renal biopsy is possible. The consequences of NS are multiple: high blood pressure, undernutrition, infections and a hypercoagulable state. OBSERVATION: We report the case of a patient presenting with thromboembolic disease, occurring in the absence of other thromboembolic risk factors, which revealed NS with spontaneously favorable evolution. CONCLUSION: Thromboembolic disease in NS is frequent but underestimated and may remain underdiagnosed. Thorough investigation - including serum protein levels and testing for proteinuria - are essential in thromboembolism, as is excluding cancer or another cause. The treatment of thromboembolic disease in NS is based on anticoagulation for as long as the NS persists. There is no consensus about primary prophylaxis but an albumin level below 20g/L should be considered as a risk factor of thrombosis and prophylactic anticoagulation should be started.

[Membranous nephropathy: New insights in therapeutic approach]. / Traitement de la glomérulonéphrite extramembraneuse.

Membranous nephropathy is one of the leading causes of nephrotic syndrome in adults, evolving to 30 % end-stage renal disease after 10 years, in the absence of specific treatment. In 2009, the M-type phospholipase A2 receptor (PLA2R), a podocyte membrane glycoprotein, was identified as the first autoantigen involved in more than 70 % of primitive membranous nephropathy. Many studies have reported that high titers of PLA2R antibodies are correlated with a lower risk of spontaneous or immunosuppressant-induced remission, a higher risk of nephrotic syndrome and of progression to end-stage renal disease. Treatment is still challenging and controversial because of potential toxicity and lack of a reliable prognosis marker. In the past, the 2012 Kidney Disease: Improving Global Outcomes (KDIGO) clinical practice guidelines recommended immunosuppressive therapies as steroids and alkylating agents or cyclosporine in patients with persistent nephrotic syndrome or impaired renal function. Recent studies and one multicentric randomised controlled trial brought clear evidence to support the use of rituximab in these patients: rituximab regimen induces immunological and clinical remission in patients with membranous nephropathy, with a high safety profile. However, they have provided important data on the impact of PLA2R antibodies assessment as a prognostic biomarker in patients with membranous nephropathy. The next step will be the integration of this biomarker in KDIGO guidelines and the recommendation of rituximab as a first line immunosuppressive therapy in patient with persistent nephrotic syndrome due to membranous nephropathy.

[Lower limb arterial thrombosis in a nephrotic syndrome]. / Thrombose artérielle du membre inférieur sur un syndrome néphrotique.

Thromboembolic complications are frequent in the nephrotic syndrome. Arterial localizations have been rarely reported. There is no consensus on their management; it depends on the location and hypercoagulable state. We report a case of acute lower limb ischemia occurring in children with a history of nephrotic syndrome, complicated by toes necrosis. The diagnosis was made by Doppler ultrasonography. Thrombectomy was performed and the patient received an anticoagulant treatment. Local cares have improved the local state, avoiding surgical necrosectomy.

[Nephrotic Syndrome in Adult Patients--Etiology and Complications]. / Das nephrotische Syndrom beim Erwachsenen--Ursachen und Komplikationen.

The nephrotic syndrome (NS) in adult patients is a rare entity. It is characterized by a tetrad consisting of edema, proteinuria, hypoalbuminemia and hypercholesterolemia. NS can be caused by intrinsic glomerular disorders or secondary damage to the glomerulus triggered by systemic diseases, infections or drugs. The responsibility of the primary care physician is to distinguish NS from other edematous disorders. Patients should be referred to a nephrologist for further diagnosis and treatment. NS is associated with various complications. Affected individuals are prone to infections and may suffer from thrombophilia, acute renal failure and Vitamin D deficiency. Immunosuppressive therapy of the underlying disorder may cause late onset complications such as skin cancer or urothelial carcinoma. Aside from causative therapy adequate management of these complications is crucial for the treatment of patients suffering from NS.

[Disseminated tuberculosis revealing IgA nephropathy with nephrotic syndrome : A case report]. / Néphropathie à IgA compliquée d'un syndrome néphrotique révélée par une tuberculose disséminée.

A 27-year-old man without any medical history presented concomitantly a pulmonary and urinary tuberculosis and a nephrotic syndrome with hematuria and renal failure. The renal biopsy showed increased mesangial matrix, few focal segmental lesions, and IgA deposits confirming the diagnosis of IgA nephropathy. Nephrotic syndrome remission occurred quickly after antituberculous treatment. The association between tuberculosis and IgA nephropathy has been previously reported in 9 patients. Renal outcome was always favorable with antituberculous treatment. No relapse occurred, with a maximal follow-up of 42 months. Here, we discuss this singular association and previous similar cases.

[Histologic kidney lesions during pure nephrotic syndrome in adults in Conakry]. / Lesions histologiques renales au cours du syndrome nephrotique pur de l'adulte a Conakry.

INTRODUCTION: Nephrotic syndrome represents a significant part of chronic kidney disease in Black Africa. Our work aimed to determine the frequency and type of kidney lesions in an adult carrying pure nephrotic syndrome. PATIENTS AND METHODS: We conducted a prospective and descriptive study (2003-2004), 40 patients were recruited on the basis of the presence of a pure nephrotic syndrome. Proteinuria was plentiful, selective to albumin greater than 3 g/24h, not accompanied by hematuria, or high blood pressure, or kidney failure. Cases of bacterial, parasitic and viral infections (hepatitis B, C, HIV) were excluded. By following contra-indications of renal biopsy, samples were taken under local anesthesia by means of percutaneous lumbar and fixed in two tubes, one containing "Michel's medium" and the other 12% Formalin. These techniques and readings were carried out in the pathological anatomy Laboratories of Nantes (France) and Conakry (Guinea). RESULTS: There were 24 men and 16 women with an average age of 26.2 ± 8 years [range: 20-51]. Clinical symptoms were dominated by weight gain characterized by edema. Proteinuria was between 3-3.5 g/24h in 16 (40%); between 3.6-5 g in 2 cases (5%) and greater than 5 g/24h in 22 cases (55%). The number of glomeruli was on average 11 ± 9 [range: 3-36]; glomerular permeability was on average 10.4 ± 10. Renal impairment was glomerular in 22 cases, tubulointerstitial in 12 cases and vascular in 6 cases. Immunofixation was positive in 30/40 cases for IgA IgG IgM; in 26 cases for C1q C3; in 4 cases for C1q C3 C4; and finally for fibrin in 28 cases. Histological renal lesions were FSGS (40%), MDC (35%), MGN (5%), MPGN (5%) and undetermined (15%). CONCLUSION: A regular practice of renal anatomopathological examination "on the spot" will lead us to carefully assess the causes of kidney failure associated with nephrotic syndrome.

INTRODUCTION: Le syndrome néphrotique représente une part importante d'insuffisance rénale chronique en Afrique noire. Notre travail avait pour but de déterminer la fréquence et le type de lésions histologiques rénales chez un adulte porteur d'un syndrome néphrotique pur. PATIENTS ET MÉTHODES: Au cours d'une étude prospective et descriptive (2003­2004), 40 patients ont été sélectionnés sur la base de la présence d'un syndrome néphrotique pur. La protéinurie était abondante, sélective à l'albumine, supérieure à 3 g/24h, non accompagnée d'hématurie, ni d'hypertension artérielle, ni d'insuffisance rénale. Les cas d'infections bactériennes, parasitaires et virales (hépatites B, C, HIV) ont été volontiers exclus. En respectant les contre indications de la biopsie rénale, les prélèvements ont été effectués sous anesthésie locale, par voie lombaire percutanée, puis fixés dans deux tubes dont un contenait du « liquide de Michel ¼ et l'autre du Formol à 12%. Les techniques et lectures ont été effectuées aux Laboratoires d'anatomie pathologique de Conakry (Guinée) et de Nantes (France). RESULTATS: Il s'agissait de 24 hommes et de 16 femmes âgés en moyenne de 26,2 ± 8 ans [20­51]. La symptomatologie clinique était dominée par une prise de poids marquée par les œdèmes. La protéinurie était comprise entre 3­3,5 g/24h dans 16 cas (40%); entre 3,6­5 g dans 2 cas (5%) et supérieure à 5 g/24h dans 22 cas (55%). Le nombre de glomérules était en moyenne de 11 ± 9 [3­36]; la perméabilité des glomérules était en moyenne de 10,4 ± 10. L'atteinte rénale était glomérulaire dans 22 cas, tubulo-interstitielle dans 12 cas et vasculaire dans 6 cas. L' immunofixation a été positive dans 30 cas /40 pour les IgA IgG IgM; dans 26 cas pour C1q C3; dans 4 cas pour C1q C3 C4; et en fin pour la fibrine dans 28 cas. Les lésions histologiques rénales étaient une HSF (40%), une LGM (35%), une GEM (5%), une GNMP (5%) et indéterminée (15%). CONCLUSION: Une pratique régulière de l'examen anatomo-pathologique rénal «sur place" nous amènerait à apprécier judicieusement les causes d'insuffisance rénale en rapport avec un syndrome néphrotique.

[A multicenter study on profile of hypertension in focal segmental glomerulosclerosis in Tunisia]. / Profil de l'hypertension artérielle dans la hyalinose segmentaire et focale (HSF). Étude multicentrique de 116 cas en Tunisie.

UNLABELLED: Hypertension in focal segmental glomerulosclerosis is frequent and responsible for the progression of the disease. It could be a circumstance of the diagnosis of FSG or a complication of the nephrotic syndrome. PURPOSE: To determine the prevalence of hypertension among patients with FSG diagnosed in Tunisia and to describe the profile of patients with FSG having hypertension in contrast with who do not. PATIENTS AND METHODS: It was a retrospective multicentric study based on 116 patient files having FSG located in 5 specialized centers in Tunisia. RESULTS: The prevalence of hypertension among our patients was 41%, with a feminine predominance, their mean age was 36.34 ± 15.71 years. The systolic blood pressure among the patients with hypertension was 153.18 mmHg. The nephrotic syndrome was impure due to hypertension in 14.5% of the cases. The patients affected by hypertension were more obese. Proteinuria was higher among those not having hypertension than those with it, who score an average value of 5.67 ± 4.51 g/24h, with an insignificant difference. Serum creatinine at presentation was significantly higher among patients with hypertension. Vascular lesions were present at the renal biopsy among 39.45% of patients affected by hypertension, associated with renal failure in 58.50% of patients. The etiopathogenic treatment of FSG was essentially based on steroids full dose. CONCLUSION: Hypertension is often present in FSG and its' treatment must be as soon as possible in order to slow the progression of kidney chronic disease.

[Growth in steroid-responsive nephrotic syndrome in Cote d'Ivoire]. / La croissance staturale des enfants ayant un syndrome néphrotique cortico-sensible en Côte d'Ivoire.

We realized one of the first observational studies in sub-Saharan Africa whose aim was to analyze the growth in steroid-responsive nephrotic syndrome. This was a retrospective study, involving 30 children followed for nephrotic syndrome in pediatric nephrology unit of the University Hospital of Yopougon (Abidjan) from 28.02.2005 to 19.12.2010. Each child was followed for two years and we are interested in demographic characteristics and the evolution of size. At the end of our study, it appears that: the mean age was 89.33 ± 43.41 months (7.44 years) with a sex ratio of 3,28. At the end of the two years of follow-up, patients had received a median dose of steroids 6151 ± 3832 mg/m(2), 9111 ± 1376 mg/m(2) and 8664 ± 5379 mg/m(2), respectively, for a relapse or no, two and at least three relapses. Fourteen patients had received vitamin D therapy and calcium. At the end of follow-up, four children had growth retardation. An average gain in Z-score was noted at the end of follow-up in girls and boys respectively +0.06 and +0.36. The growth retardation observed in our study was not associated with different growth periods (P=0.116), gender (P=0.548) and the mean Z-scores observed at the end of follow-up was not significantly different between the sexes (P=0.26). Growth retardation observed was not related to the cumulative dose administered (P=0.15), number of relapse (P=1.000).

[Thymic disease associated with nephrotic syndrome: a new case with membranous nephropathy and literature review]. / Les syndromes néphrotiques para-thymiques : revue de la littérature à propos d'une observation.

INTRODUCTION: Malignant thymoma or thymic hyperplasia is associated with various autoimmune diseases. Renal disease has rarely been reported in this condition. We report a new case with improvement of renal disease after thymectomy. CASE REPORT: A 77-year-old-women with nephritic syndrome was found to have associated thymic mass. Renal pathology showed membranous nephropathy. The thymic mass pathology showed a B2 type thymoma. After thymectomy the nephrotic syndrome improved. CONCLUSION: Glomerulopathy can be secondary to an acquired thymic disease. Membranous nephropathy but also other glomerular diseases can be observed often presenting with nephritic syndrome. Despite the rarity of this association this clinical observation underlines that a thymoma should be searched in the presence of a glomerulopathy. The glomerulopathy can be improved by the treatment of the thymoma.

[AL amyloidosis]. / Amylose AL.

AL amyloidosis belongs to the group of conformational diseases. It is the most common type of amyloidosis with an estimated 500 new cases per year in France. It is due to a small and usually indolent plasma cell clone which synthesizes an unstable, misfolded monoclonal immunoglobulin light chain that is prone to aggregate and form amyloid fibrils. Non-invasive biopsy such as abdominal fat aspiration or minor salivary gland biopsy should be performed to confirm the diagnosis and if negative, involved tissues have to be examined. Clinical presentation is very diverse, as AL amyloidosis can affect almost any organ or tissue in the body, other than the brain. The kidney is the most frequent organ involved, whereas heart disease characterized by restrictive cardiomyopathy is the most severe. Early diagnosis, before advanced cardiomyopathy, is essential for improving outcome. The association of alkylating agent and high-dose dexamethasone is effective in almost two-thirds of patients. Combinations of proteasome inhibitors, dexamethasone, and alkylating agents achieve high response rates. Close monitoring of clonal and organ response is mandatory to guide therapy changes and duration. New treatments designed to eliminate amyloid deposits are under development.