Retroperitoneal fibrosis (RPF) is a rare disease and has a high risk of developing chronic kidney disease (CKD). This retrospective study was carried out with the objective to study the epidemiological, clinical and therapeutic characteristics of RPF and identify the risk factors associated with its progression to CKD. All 30 cases (24 males and five females) of RPF admitted from January 1985 to December 2013 in the Military Hospital of Tunis were included in this study. The mean age was 50.5 years. Presentation was with lower back pain, acute renal failure and inflammatory syndrome in 93%, 56% and 43% of the cases, respectively. Sixteen patients (54%) had a creatinine clearance <60 mL/min at the time of diagnosis. Erythrocyte sedimentation rate and C-reactive protein of >30 mm/h and 10 mg/L were observed in 56% and 53% of cases, respectively. The abdominal computed tomography scan showed ureterohydronephrosis in 63% of the cases. Classes I, II and III according to Scheel's radiological classification were found in, respectively, 16%, 13% and 70% of cases. Biopsy of RPF was performed in 20% of the cases, and all showed an inflammatory infiltrate without signs of vasculitis. RPF was idiopathic in 85% of the cases. Oral corticosteroid therapy was started for all patients. After a mean follow-up time of 53.2 months, an initial favorable response was noted in 76% of the cases. Fifty-three percent of the patients have presented one or more relapses during follow-up and 20% progressed to CKD. Most relapses were successfully treated by corticosteroids; only five patients had required additional immunosuppressive therapy. Two patients died. Elevated creatinine at diagnosis, high urea, clearance of creatinine lower than 60 mL/min and the use of ureteral stents were identified as risk factors for development of CKD.
Tissue calcification is a common complication in patients on continuous hemodialysis (HD) for chronic renal failure; however, severe calcification is unusual. Three distinct clinical types of extraosseous calcifications are found in uremic patients: vascular calcification, periarticular (tumoral) calcification, and visceral calcification (heart, lung, and kidney). We report a case of a young chronic HD patient who presented with extensive metastatic calcifi cations both vascular, visceral specially localized in the lungs, and periarticular with progressively increasing multiple subcutaneous swellings. This evolution was secondary to noncompliance of the patient to the treatment of a malignant hyperparathyroidism with a marked elevation of phosphocalcium product.
Thromboembolic disease is an important and frequent complication in patients with the nephrotic syndrome (NS), and the consequences are often severe. Usually, the venous system is affected. Arterial thrombosis has rarely been reported and occurs mainly in children. We report the case of a 27-year-old man with a history of NS due to focal and segmental glomerulosclerosis resistant to steroids and cyclosporine, admitted for bilateral pain in the calves. Aortogram revealed a suspended thrombus in the abdominal aorta just below the origin of the renal arteries with embolism into the left tibioperoneal trunk and the right anterior tibial artery. Endarterectomy was performed followed by systemic heparinization with a good outcome. Arterial thrombosis is rare and must be prevented.
Background
Lupus nephritis (LN) is one of the most serious complications of systemic lupus erythematosus since it is the major predictor of poor prognosis. Corticosteroids and immunosuppressive treatment has transformed the prognosis of this pathology despite the frequent infectious complications. The Eurolupus protocol is a new regimen using lower doses and shorter treatment durations of intravenous cyclophosphamide that have been advanced to reduce toxicity without sacrificing efficacy of therapy.
Objectives
Our study aims to evaluate this protocol used in patients followed in the internal medicine department of the military hospital of Tunis.
Results
Among 120 patients with SLE, 34 (34,16 %) had LN. Eleven (9,17%) patients were treated according to the Eurolupus protocol (6 fortnightly pulses of 500 mg; cumulative dose 3 gm, followed by azathioprine or mycophenolate monfetil “MMF”). The average age was 38 years, with 2 males and 9 females. Renal biopsy performed in 10 cases showed a LN class III, IV and IV+V in respectively 1, 7 and 2 cases. Histological signs of activity were noted in all cases with signs of chronicity in 2 cases. Maintenance therapy was azathioprine in 5 cases and MMF in 5 cases. The evolution was marked by a complete remission in 7 cases (58%), partial remission in 3 cases (27%) and deterioration of renal function in one case (9%). The average delay of remission was 5 months (1-12months). Renal relapse was noted in 4 cases (38,5%) related to treatment withdrawal, infection and/or pregnancy respectively in three, two and one cases. Infectious complications were observed in 2 cases but no cases of death have been noted.
Conclusions
The Eurolupus protocol leads to similar results as the old regimen of cyclophosphamide with a cumulative dose much smaller and less frequent infectious complications.
References
Updates on the Treatment of Lupus Nephritis. Andrew S. Bomback and Gerald B. Appel. JASN December 1, 2010vol. 21 no. 12 2028-2035
Immunosuppressive Therapy in Lupus Nephritis. The Euro-Lupus Nephritis Trial, a Randomized Trial of Low-Dose Versus High-Dose Intravenous Cyclophosphamide. Frédéric A. Houssiaud al* ARTHRITIS & RHEUMATISM, Vol. 46, No. 8, August 2002, pp 2121–2131
Disclosure of Interest
None Declared
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