Rim Goucha

Atteinte rénale au cours de la maladie de Takayasu

Renal involvement in Takayasus arteritis is frequent and worsens the progression of the disease. This is primarily a renal artery stenosis causing renovascular hypertension. The glomerular disease is exceptional. This study was undertaken to determine the clinical, radiological, biological features and therapeutic response in patients with kidney disease associated with Takayasu arteritis. A retrospective chart review was conducted on 11 patients (five men and six females), with a mean age of 31.1 years (19-40 years). The discovery of kidney disease preceded the diagnosis of Takayasus arteritis in eight cases. Ten patients developed hypertension. Laboratory finding showed proteinuria in five cases of which one case was due to nephrotic syndrome. Renal failure was found in six cases including four cases in stage of terminal chronic renal failure. Impairment of the renal artery was present in nine patients, proximal in seven cases and distal in two cases, bilateral in five cases and unilateral in four cases. Narrowing renal artery was found in seven cases. The renal biopsy revealed membranoproliferative glomerulonephritis in one case and nephrosclerosis in another case. Eleven patients were followed for an average period of 155 months (3-335 months). Remission of nephrotic syndrome was concomitant with the remission of the disease. Seven patients developed outbreaks of Takayasus arteritis of which six were in care. Relapse of nephrotic syndrome was concomitant with the outbreak of the disease followed by spontaneous remission of both diseases. Improved pressure was obtained in 5 cases and worsening renal function in seven cases. Death was observed in two cases.

Pauci-immune crescentic glomerulonephritis in the Down's syndrome

Kidney disease is a rare complication in patients with the Downs syndrome. However, with increased survival, it appears that a growing number of these patients present with glomerulonephritis. Most cases have been reported as case reports and include lesions such as mesangiocapillary glomerulonephritis with hypo-complementemia, crescentic glomerulonephritis with anti-neutrophil cytoplasmic antibodies (ANCA), amyloidosis and immunotactoid glomerulopathy. We report the observation of a 38-year-old man with the Downs syndrome who presented with severe renal failure, proteinuria and microscopic hematuria evolving over two months. There was no history of congenital heart disease or urinary symptoms. Percutaneous renal biopsy revealed fibrous crescents, rupture of Bowmans capsule and peri-glomerular granuloma; there were no deposits on immunofluorescence study. Thoracic computerized tomography scan showed alveolar congestion. The patient tested negative for ANCA. At the time of reporting, the patient is on regular chronic hemodialysis. Our case illustrates a distinct entity that further expands the spectrum of renal disease known to occur in the Downs syndrome. Early detection of the renal disorders may prevent or slow down the progression.

Phéochromocytome surrénalien chez un insuffisant rénal chronique en dialyse péritonéale

Pheochromocytoma is a rare tumor responsible for paroxysmal hypertension which is difficult to control. Diagnosis is important because it represents a curable form of hypertension. Few cases of pheochromocytoma patients with end-stage renal failure were reported in the literature. These cases are specially responsible for diagnosis and therapeutic problems. We report here a case of an end-stage renal failure patient who has pheochromocytoma, he was treated by automated peritoneal dialysis. The patient is a 47-year-old man who has an IgA glomerulonephritis. On peritoneal dialysis, his blood pressure level remains high despite four antihypertensive drugs association and adequate dialysis. Furthermore, the patient suffered from headaches, sweats and palpitations. This leads to suspect pheochromocytoma. Thus, urinary excretion rates of metanephrines and normetanephrines were high. Radiographic diagnosis tests were negative but MIBG scintigraphy was able to localise the tumor in the left suprarenal gland. He had coelioscopic left adrenalectomy without complications, microscopic studies showed an hyperplasia of the adrenal medulla. Soon after surgery his blood pressure was well controlled by one antihypertensive drug. We conclude that refractory hypertension, as a possible diagnosis, is uncommon in peritoneal dialysis patients. Pheochromocytoma must be eliminated by careful evaluation.

Pronostic rénal de la néphropathie des vascularites à IgA de l’adulte: étude monocentrique, à propos de 25 cas

IgA vasculitis nephritis affects the prognosis of this disease in adult patients. This study aimed to examine the clinical characteristics of this renal involvement in adults and to identify factors influencing renal prognosis. We conducted a retrospective monocentric study of patients with histologically confirmed IgA vasculitis nephritis (rheumatoid purpura) (EULAR classification criteria) with renal involvement classified according to Pillebout classification. We analyzed renal survival and identified the factors influencing renal prognosis. Twenty-five patients were included (sex ratio M/F = 2.57), their average age at diagnosis of rheumatoid purpura was 35,76 years. Purpura was diagnosed in 100% of cases, with articular involvement in 28% of cases. Renal failure was identified in 44% of cases. The most common histological classification was IgA vasculitis nephritis (class II). Clinical remission was observed in 44% of cases and an evolution toward chronic renal failure (end-stage renal disease) in 36% of cases. Renal survival at 195 months was 57%. The identified prognostic factors were digestive involvement (p = 0.022), early renal failure (p = 0.0004), glomerular classification (P=0,001) and the severity of the histological lesions, renin-angiotensin system blocker treatment (p = 0.01) and plasma exchanges (p = 0.03). Our study shows that renal involvement during IgA vasculites can be relatively severe with poor renal prognosis. The identification of clinical and histological prognostic factors may be useful as guidance for the development of prospective therapeutic studies.La néphropathie de la vascularite à IgA conditionne le pronostic de cette affection chez l’adulte. Le but de notre étude était d’étudier les caractéristiques cliniques de cette atteinte rénale chez l’adulte et d’identifier les facteurs de pronostic rénal. Il s’agit d’une étude monocentrique rétrospective portant sur les patients ayant une vascularite à IgA (purpura rhumatoïde) (critères de l’EULAR) avec une atteinte rénale prouvée histologiquement et classée selon la classification de Pillebout. Nous avons analysé la survie rénale et identifier les facteurs de pronostic rénal. Vingt cinq patients ont été inclus (genre ratio M/F = 2,57) d’âge moyen au diagnostic du purpura rhumatoïde de 35,76 ans. Un purpura était présent dans 100% des cas avec une atteinte articulaire dans 28%. Une insuffisance rénale était présente dans 44% des cas. La classification histologique la plus fréquente était la classe II. Une rémission clinique a été observée dans 44% des cas et une évolution vers le stade terminal de l’insuffisance rénale chronique dans 36% des cas. La survie rénale à 195 mois était de 57%. Les facteurs pronostiques identifiés étaient l’atteinte digestive (p = 0,022), l’insuffisance rénale initiale (p = 0,0004), la classification glomérulaire (p = 0001) et la sévérité des lésions histologiques, le traitement par bloqueurs du système rénine angiotensine (p = 0,01) et les échanges plasmatiques (p = 0,03). Notre étude montre que l’atteinte rénale au cours des vascularites à IgA peut être relativement sévère avec un mauvais pronostic rénal. L’identification des facteurs pronostiques cliniques et histologiques pourrait guider l’élaboration d’études thérapeutiques prospectives.

Urinary mRNA analysis of biomarkers to epithelial mesenchymal transition of renal allograft

Renal allograft loss is most often a chronic process, irrespective of the mechanism at stake. In this prospective study, we studied the expression of epithelial to mesenchymal transition (EMT) markers vimentin and β-catenin by immunohistochemistry in the surveillance biopsy and measured the mRNA encoding vimentin (VIM), CD45, GAPDH and uroplakin 1a (UPK) by quantitative PCR in urinary cells in 75 renal transplant patients. The aim is to establish a simple screening test for chronic renal allograft dysfunction. We found that the value of the mRNA of vimentin and CD45 relative to the uroplakin 1a (UPK) mRNA is correlated with the score in vimentin immunostaining in routine biopsies. These biomarkers could be used as a noninvasive tool to monitor the renal graft fibrogenesis. This test could be used for early detection of fibrotic diseases of the kidney transplant.

Acute kidney injury with granulomatous interstitial nephritis and vasculitis revealing sarcoidosis

Sarcoidosis is an inflammatory disease that affects mostly the lungs and lymph glands. Renal involvement is rare and especially vasculitis. We report a case who presented an acute kidney failure and had sarcoidosis with vasculitis and nodular splenic involvement. A 35-year-old woman presenting a Lofgren syndrome was hospitalized for acute renal failure with cervical lymphadenopathy without other clinical findings. Laboratory data disclosed elevated angiotensin converting enzyme serum level. Abdominal ultrasound showed a multinodular spleen. Renal histology revealed granulomatous interstitial nephritis with necrotizing vasculitis. Outcome was favorable after the institution of high dose corticosteroids along with cyclophosphamide. Renal involvement is rare in sarcoidosis. However, the diagnostic delay should be avoided to improve the outcome.

Chronic graft versus host disease and nephrotic syndrome

Disturbed kidney function is a common complication after bone marrow transplantation. Recently, attention has been given to immune-mediated glomerular damage related to graft versus host disease (GVHD). We describe a 19-year-old woman who developed membranous glomerulonephritis after bone marrow transplantation (BMT). Six months later, she developed soft palate, skin and liver lesions considered to be chronic GVHD. Fifteen months after undergoing BMT, this patient presented with nephrotic syndrome. A renal biopsy showed membranous glomerulonephritis associated with a focal segmental glomerulosclerosis. She was started on corticosteroid treatment with good outcome.