M. Hajji

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.

Atypical hemolytic uremic syndrome: A monocentric adult Tunisian study and review of literature

Atypical hemolytic uremic syndrome (aHUS) is characterized by microvascular thrombosis resulting in thrombocytopenia, hemolytic anemia, and multiorgan dysfunction. It is associated with genetic or acquired disorders of regulatory components of the complement system. For our study, we collected data from 16 patients diagnosed with aHUS between January 2010 and January 2014. The mean age was 33.6 years. The female-to-male ratio was 3. The median follow-up duration was 27 ± 3.5 months. The most common clinical presentation was hypertension. Renal involvement was noted in all cases. Ten patients had extrarenal manifestations. Semi-quantitative dysfunction of the alternative pathway of complement was found in all cases. A genetic study was not available for our patients. During the acute stage, all patients received plasma therapy, and among them, seven required dialysis and five were still on dialysis at the time of discharge. One patient underwent renal transplantation. None of our patients received eculizumab perfusion. The renal survival was inversely correlated to young age (<30 years) (P = 0.001), presence of anti-factor H antibodies (P = 0.003) and serum creatinine at diagnosis >5 mg/dL (P = 0.02). Mortality rate was significantly correlated to young age (<30 years old) (P = 0.01). Collecting multicentric data on adult patients with aHUS will enable better characterization of the spectrum of adult aHUS in our country and the evaluation of current treatments and different outcomes.

Clinicopathological findings and outcome of lupus nephritis in Tunisian children: a review of 43 patients

We report clinical and renal histological data, treatment modalities and outcome of 43 Tunisian children with biopsy-proven lupus nephritis seen over a 23-year period. There were 39 girls and 4 boys with a mean age of 12.5 years at diagnosis of lupus nephritis and followed for a mean period of 77 months. Renal symptoms included urinary abnormalities in all patients, hypertension in 40% of cases, nephrotic syndrome in 60% of cases and renal failure in 25% of cases. Class IV and class III nephritis were observed in 48.8 % and 30.2 % respectively. Corticosteroids were used in all cases, associated to immunosuppressive therapy in 23%. Overall survival was 86% at 5 years and 74% at 10 and 15 years. Renal survival was 83% at 5 and 10 years and 63% at 15 years. Initial renal failure and tubulointerstitial fibrosis were significantly increased risk for the development of end-stage renal disease in our study group. Renal histological findings provide the basis for treatment recommendations. Timely performed renal biopsy is greatly needed to accurately determine the prognosis and to guide treatment in children lupus nephritis.

Factors associated with relapse of lupus nephritis: A single center study of 249 cases

This is a retrospective cohort study over 20 years (1990-2013) that included all patients with biopsy-proven lupus nephritis (LN) followed up at our nephrology department. We aimed to determine the clinicobiologic predictors of flare-up of LN. Flare was defined as an increase in systemic lupus erythematosus (SLE) disease activity index (SLEDAI) score of at least four points. Clinical manifestations and laboratory parameters were assessed and the SLEDAI score was determined for each patient. We included patients with SLE who fulfilled at least four of the American College of Rheumatology criteria for the classification of SLE. A total of 249 patients including 227 females and 22 males with a median age at diagnosis of 34.32 years (range 16-69) were studied. The mean follow-up duration was 122.4 ± 27 months. Renal symptoms included hypertension in 40%, nephrotic syndrome in 30%, and renal failure in 69.4% of the cases. Class IV and class III nephritis (ISN/RPS) were observed in 44.9% and 24% of the patients, respectively. On univariate analysis, flare predictors were age <30 years (P = 0.02), lymphocytopenia (P = 0.002), the presence of diffuse proliferative LN (P = 0.009), and discontinuation of immunosuppressive therapy (P = 0.004). Our study suggests that these markers should be monitored routinely as prognostic parameters in SLE to characterize patients who are at risk and who should be followed more closely.

Disseminated cryptococcosis as a complication of lupus nephritis

Cryptococcus neoformans is an opportunistic fungal infection affects predominately the central nervous system in HIV patients and patients with other immunocompromised states. It has rarely been described in immunocompetent patients. It is a serious infection with a high of mortality rate. We describe a case of a 48-year-old patient diagnosed with lupus nephritis treated with corticosteroids and mycophenolate mofetil who developed central nervous cryptococcosis complicated by septicemia. She died despite the use of antifungals. Cryptococcal infection is an uncommon, but often a fatal complication of systemic lupus erythematosus. Timely diagnosis and effective antifungal therapy could improve its prognosis.

A rare cardiac manifestation in autosomal-dominant polycystic kidney disease

Autosomal-dominant polycystic kidney disease (ADPKD) is a systemic disorder associated with various extrarenal complications. There is little information regarding the occurrence and distribution of cardiovascular abnormalities during the course of ADPKD. The major cardiovascular complications of ADPKD include valvulopathies and vascular ectasia. Aneurysm of the atrial septum (ASA) is a very rare manifestation in ADPKD. A 37-year-old woman who was diagnosed with ADPKD was admitted to our hospital for advanced renal failure. Pelvic computed tomography revealed multiple variable-sized cysts in both kidneys. Trans-thoracic echocardiography showed ASA while the patient was completely asymptomatic.

Acute renal cortical necrosis in pregnancy: Clinical course and changing prognosis

Obstetric cortical renal necrosis is a serious complication that can lead to chronic renal failure and the need for chronic dialysis. The aim of renal cortical necrosis therapy is to restore hemodynamic stability, institute early dialytic therapy, and treat the underlying cause of the disease. Most cases of renal cortical necrosis do not recover a normal renal function despite intensive care. We describe the course of a patient who was diagnosed with acute renal cortical necrosis in pregnancy treated with hemodialysis for three years but then she recovered her renal function.

Epuisement du capital vasculaire en hémodialyse: quelle issue?

Malgré les progrès réalisés dans le traitement de l’insuffisance rénale chronique, l’accès vasculaire reste le maillon faible dans la thérapie de suppléance extrarénale et la principale source de morbidité chez les patients hémodialysés. Nous rapportons l’observation d’une jeune patiente ayant une insuffisance rénale chronique secondaire à une néphropathie vasculaire en hémodialyse périodique, confrontée précocement à un épuisement de son capital vasculaire, en raison de thromboses itératives des fistules artério-veineuses et l’échec de la dialyse péritonéale. Un déficit en protéine C a été objectivé. Elle a bénéficié de la mise en place d’un cathéter tunnelisé au niveau de l’oreillette droite par thoracotomie antéro-latérale droite à travers la veine cave inférieure non fonctionnel au bout de trois mois de son utilisation. Elle est depuis dialysée par ponction des veines jugulaires externes.Despite advances in the treatment of chronic renal failure, vascular access remains the weakest link in renal replacement therapy (RRT) and the leading cause of morbidity in patients on hemodialysis We report the case of a young female patient with chronic renal insufficiency secondary to vascular nephropathy on periodic hemodialysis and whose vascular capital was early exhausted due to iterative thromboses in arteriovenous fistulas and failure in peritoneal dialysis. Protein C deficiency was objectified. The patient underwent tunneled hemodialysis catheter insertion at the level of the right atrium via a right anterolateral thoracotomy with cannulation of the inferior vena cava, with poor functional outcome after three months of use. Since then she has been dialyzed using puncture of the external jugular veins.