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.
Arndt‐Gottron (S‐AG) syndrome or scleromyxedema is a scarce disease characterized by a generalized papular and sclerodermoid eruption and systemic manifestations that can lead to significant ...morbid‐mortality. Interesting, S‐AG can be associated with a paraprotein. We report an exceptional scleromyxedema case associated with a benign monoclonal gammapathy in an old woman.
Renal tubular involvement in Sjögren's syndrome (SS) often described with renal tubular acidosis, nephrogenic diabetes insipidus, or rarely with Fanconi syndrome. SS presenting with clinical features ...of Bartter's syndrome or Gitelman's syndrome is rare. We report a case of a female patient who presented an acquired Bartter syndrome with a primary SS.
Renal involvement is rare in systemic sarcoidosis. Among renal manifestations, tubulointerstitial nephritis (TIN) is the most commonly reported finding. We conducted the current study to investigate ...the clinical, laboratory, and histological features and to analyze the outcome of TIN due to sarcoidosis. We present a retrospective, single-center study of patients followed for sarcoidosis and presenting with TIN related to this systemic disease. Twenty-four patients were assessed (22 females/2 males). The mean age at diagnosis of TIN was 46.3 years. Extrarenal manifestations were dominated by thoracic involvement (95.8%), peripheral lymph nodes (54.2%), and skin lesions (33.3%). The mean proteinuria level was 0.68 g/24 h. Renal failure was diagnosed in 83.3% of cases with a median estimated glomerular filtration rate at 14.3 mL/min/1.73 m2. Nine patients presented with hypercalcemia and 12 patients with hypercalciuria. Renal biopsy was performed in 58.3% of cases. Six of the 14 patients presented with noncaseating granulomatous interstitial nephritis and eight with interstitial nephritis without granuloma. Granulomatous infiltration of renal parenchyma was complicated by vasculitis in two cases. Corticosteroid therapy was used in all patients. On follow-up analysis, four patients progressed to end-stage renal disease (ESRD) after a mean duration at 45.5 months. In the remaining patients, kidney function statistically significantly improved after one month of treatment compared to the time when the diagnosis was initially established (P = 0.031). We found that the predictive factors of progression to ESRD were multiorgan involvement (P = 0.032), advanced fibrosis F3 (P = 0.0006), and extensive interstitial granulomas (P = 0.007) and these were independently correlated with ESRD. Corticosteroid therapy seems to be effective in sarcoid TIN, but some degree of persistent renal failure is possible which can be predicted from both histologic findings and initial response to steroid therapy.
La sarcoïdose est une granulomatose multi viscérale d'étiologie inconnue qui peut revêtir des tableaux cliniques et radiologiques diverses. Les localisations cérébrales bien que rares, peuvent se ...présenter sous forme pseudo-tumorale trompeuse. Nous rapportons l'observation d'un jeune adulte Tunisien hospitalisé pour hypertension intracrânienne en rapport avec une lésion pseudotumorale radiologique qui a révélé une sarcoïdose systémique.
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.
The chordoma is a benign cartilaginous tumor whose sphenoidale localization is exceptional. This tumor has considerable difficulties of both diagnosis and treatment. We report the observation of a ...Tunisian adult who presented features of hypopituitarism set wrongly on account of a prolactinoma.
La maladie cœliaque (MC) représente une maladie auto immune touchant plusieurs organes. Elle est souvent révélée par des manifestations digestives avec une malabsorption biologique. Cependant, ...l’atteinte des séreuses peut exceptionnellement révéler cette entéropathie rendant le diagnostic difficile. Il s’agit du patient JA âgé de 63 ans admis pour exploration de syndrome de malabsorption avec des signes de polysérite à type de pleurésie, péricardite, ascite et hydrocéphalie. Le diagnostic de MC a été porté devant des signes endoscopiques sans arguments sérologiques. L’évolution était partiellement favorable à cause de la mauvaise adhésion au régime sans gluten. Notre observation est le premier cas rapporté d’une polysérite révélant la MC qui devrait être évoquée devant tout syndrome de malabsorption en l’absence de signes évocateurs.
BACKGROUND Type 1 autoimmune pancreatitis (AIP), also known as lymphoplasmacytic sclerosing pancreatitis (LPSP), is a rare cause of chronic pancreatitis, characterized by a fibro-inflammatory ...process. However, patients with AIP may have a good response to corticosteroid therapy. We describe a Tunisian patient with AIP that was confirmed to be an IgG4-related disease (IgG4-RD). CASE REPORT We describe a case of a 70-year-old man who was admitted to hospital for obstructive jaundice and abdominal pain. Serum liver function tests were abnormal and upper abdominal computed tomography (CT) imaging showed diffuse pancreatic swelling and strictures of the main pancreatic duct without any focal lesion. Pancreatico-biliary magnetic resonance imaging (MRI) showed a thickened rim surrounding the pancreatic duct Serum IgG4 levels were elevated, resulting in a diagnosis of IgG4-related AIP. The patient showed a good clinical, biochemical, and radiological response following steroid therapy in combination with azathioprine. CONCLUSIONS The diagnostic workup of IgG4-RD is complex and usually requires a combination of clinical examination, imaging, and serological analysis. As this case report has demonstrated, IgG4-RD should be considered in patients who present with pancreatitis or AIP, because of the favorable response to steroid therapy, particularly when treatment is initiated early.
All these data were in keeping with LN. ...it was possible to make a diagnosis of systemic lupus erythematosus (SLE) with both renal and cerebrovascular involvement. The treatment included captopril, ...prednisolone, diltiazem and Vitamin E. Two years after, there was normalization of blood pressure, regression of proteinuria, and decrease of the serum creatinine (111 μmol/L) with eDFG of 49 mL/min. Since that, she was only followed up for two years then she never consulted. ...our patient presented a posterior arachnoids cyst. ...ADPKD may remain asymptomatic for a longtime2 as in both of our cases. ...ultrasonography is the procedure of choice in the workup of patients with ADPKD.
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