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Abstract Background Takayasu arteritis is a rare and chronic granulomatous vasculitis that affects the large vessels. Takayasu arteritis targets the aorta and its branches and is still of unknown etiology. It often affects female patients under 50 years of age. A relationship between Takayasu arteritis and tuberculosis has been suggested for a long time. Case presentation We report a severe case of Takayasu arteritis in a 10-year-old Tunisian child revealed by renovascular hypertension with concomitant pulmonary tuberculosis. Conclusions Our patient is among only a few cases of Takayasu arteritis published worldwide affecting young infants and adolescents, which underlines the strong relationship between Takayasu arteritis and tuberculosis.

Abstract Background Lupus nephritis and lupus erythematosus tumidus (LET) are uncommon manifestations of systemic lupus erythematosus (SLE), and their coexistence as the initial presentation of SLE is exceedingly rare. Here, we report such a case, emphasizing the diagnostic challenges and therapeutic implications of this unusual association. Case report A 38-year-old North African woman presented in Nephrology department with a history of lower extremity edema, fatigue, and weight loss of 3 kg in 4 weeks. Physical examination revealed LET lesions on the chest and the Neck. Laboratory investigations showed lymphopenia, low C3 and C4 complement levels, positive antinuclear antibodies, anti-dsDNA antibodies, and anti-SSA/Ro antibodies. Renal function tests showed normal serum creatinine and nephrotic proteinuria. Renal biopsy revealed Class V lupus nephritis. Skin biopsy confirmed the diagnosis of LET, with the presence of lymphohistiocytic infiltrates and dermal mucin. The patient was diagnosed with SLE based on the 2019 EULAR/ACR criteria and treated with prednisone (1 mg/kg/day) and hydroxychloroquine. She showed significant improvement in her cutaneous and renal symptoms at 6 and 12 months follow-up. Conclusion The rarity of the coexistence of LET and lupus nephritis as the initial manifestation of SLE, especially in the North African population, underscores the need for further research to elucidate the immunopathogenic mechanisms and prognostic factors associated with this association.

Introduction. Longer survival in dialysis led to a higher incidence of vascular access complications and failure. With the limited access to kidney transplantation programs and peritoneal dialysis, exhaustion of vascular access for hemodialysis is an increasingly common situation. Among the available options, atrial tunneled dialysis catheter (ATDC) has been reported as an effective vascular access in this population. Methodology. We report the experiences of two nephrology centers in Tunis with ATDC as an ultimate vascular access for dialysis. Case Reports. Two patients with exhausted vasculature underwent ATDC insertion in 2020 and 2022, respectively, as a vascular access of last resort. Both patients underwent CRBI, which resolved with favorable outcomes. One case was complicated by post-operative thrombosis and was successfully treated with thrombolysis. Both patients are currently on dialysis via their ATDC with a catheter patency of 29 months. Conclusion. ATDC is a life-saving and safe vascular access in cases of depleted vasculature. Little more than 50 cases have been reported in the literature during the last 30 years. As the frequency of vasculature exhaustion is expected to increase, preservation of veinous access in patients at risk of chronic kidney disease have never been more crucial.

Background. Urinary lithiasis constitutes a recurrent pathology affecting a relatively young population. The risk of progression to chronic renal failure and the cost of treatment are the most important issues. Primary hyperparathyroidism (PHPT) is responsible for urolithiasis and nephrocalcinosis in 7% of patients, and it represents the 7th cause of urolithiasis in Tunisia. Unfortunately, it remains an underdiagnosed pathology although it is curable. We aim to determine the clinical, biological, therapeutic, and evolutionary particularities of urinary lithiasis associated with PHPT in a nephrology setting. Methods. This is a monocentric, retrospective, descriptive study which took place in our nephrology department during the period from January 2010 to January 2023. Ten patients were included. All of them underwent blood and urine tests and a morphoconstitutional study of the urinary stones if possible. Results. The median age at diagnosis of PHPT was 42 years (34–54). The median time from the onset of kidney stones to the diagnosis of PHPT was 6.2 years (1–17). The male/female gender ratio was 0.66. Five patients had hypertension, two patients had obesity, one patient had diabetes, and three patients had urinary tract infections. Kidney stones were bilateral in eight cases and unilateral in two cases. Nine patients underwent urological intervention: surgery in 5 cases associated with nephrectomy in one case, extracorporeal lithotripsy in 4 cases, and percutaneous nephrolithotomy in two cases. The diagnosis of PHPT was retained with high or uncontrolled PTH associated with hypercalcemia in 8 cases and normocalcemic PHPT was found in 2 patients. Two patients had parathyroid adenoma and one patient had mediastinal adenoma. Radiology exploration was normal for the others patients. Surgery was performed in 7 patients and histology revealed an adenoma in 5 cases and hyperplasia in one case. The predominant urinary risk factors in our study were hypercalciuria in 6 cases and insufficient diuresis in 4 cases. Conclusion. This study underlines the role of the nephrologist in the exploration of urinary lithiasis and the prevention of recurrences, especially as PHPT is a curable aetiology of urolithiasis and affects a relatively young population. The determination of the epidemiological profile of patients with stones associated with primary PHPT and lithogenic risk factors allows the primary and secondary prevention of stone formation.

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a disease of multi-organ dysfunction due to excessive immune activation causing widespread inflammation and tissue destruction. It is a severe condition associated with high morbidity and mortality. Early identification is crucial for prompt treatment. The objective of this case series is to underscore the intricacy of managing HLH in individuals with renal dysfunction. Methods: This is a retrospective study of patients diagnosed with HLH in a nephrology department over a period of 30 years. We retrospectively reviewed the medical files by applying the Revised HLH-2004 criteria. Results: Among the 14 female patients included, the mean age was 45.2 years (range 23-78). Nine patients presented with sudden onset of fever and chills. Physical examination revealed purpura in 3 cases, hepatomegaly and splenomegaly in 6 and 5 cases respectively, and peripheral lymphadenopathy in 1 case. Hemorrhagic complications were observed in 5 cases, hypertriglyceridemia in 9 cases, and hyperferritinemia in all cases. Hypothyroidism was observed in all cases, and impaired renal function was detected in 11 of them, with 5 experiencing it as a result of lupus nephritis, and 1 case attributed to pre-eclampsia. Hemophagocytosis was confirmed through sternal puncture in 11 cases. Treatment involved etiological therapy with corticosteroids and immunosuppressants and/or anti-infectives. Intravenous immunoglobulins were administered in 6 cases, while 2 cases required coagulation factor transfusions. Unfortunately, 9 patients did not survive. Conclusion: The study highlights the need for increased awareness and prompt recognition of HLH, particularly in patients with associated renal complications.

Abstract Catheter-related infections (CRI) are a major cause of morbidity and mortality in chronic hemodialysis (HD) patients. In this paper, we share our experience with CRI in HD patients. We recorded 49 cases of CRI among 167 patients during a period of 40 months (January 2018–April 2021). The incidence of CRI was 3.7 per 1000 catheter-days. The revealing symptoms were dominated by fever or chills (90%). Inflammatory signs were observed in 74% of cases with respectively concurrent exit-site (51%) and tunnel infection (6%). The biological inflammatory syndrome was found in 74% of patients (average CRP level = 198.9 mg/l). Blood cultures were performed in all cases and were positive in 65% of cases. Thirteen patients have been diagnosed with Infection complications, which were respectively infective endocarditis in 7 cases, septic arthritis in 3 cases, infective myositis in one case, cerebral thrombophlebitis in 1 case and mediastinitis in 1 case. The death occurred in eleven patients, it was due to septic shock in 9 cases, pulmonary embolism in one case and neurologic alterations related to cerebral thrombophlebitis. The mean seniority in HD was 16.5 months in the group with CRI and 3.7 months in the group without CRI (p

Dent’s disease is a rare genetic kidney disorder characterized by proximal tubular dysfunction, nephrocalcinosis, recurrent nephrolithiasis, and chronic kidney disease. Hypercalcemia is a rare finding in this disease. In this report, we present a case of possible Dent’s disease in a young adult male with hypercalcemia and chronic kidney disease. The diagnosis was evoked based on the presence of low-molecular-weight proteinuria, kidney stones, and renal failure. This case underscores the significance of considering Dent’s disease as a potential diagnosis, even in patients with chronic renal disease who exhibit hypercalcemia. It also emphasizes the importance of regular monitoring and management of patients with this condition to prevent further complications.

Abstract Fulminant lupus pneumonitis is a rare complication of SLE. We report a case of 75 years‐old male patient with SLE who developed pneumonia and severe respiratory failure requiring mechanical ventilation. Refractory respiratory distress complicating noninfectious fulminant lupus pneumonitis did not respond to methylprednisolone and intravenous immunoglobulin treatment.

Introduction. Primary hyperoxaluria type 1 (PH1) is a rare and inherited condition of urolithiasis. The aim of our study was to analyze clinical, paraclinical, and evolutionary aspects of PH1 in adult patients in our Nephrology department. Methods. We conducted a retrospective single-center study between 1990 and 2021. We collected patients followed for PH1 confirmed by genetic study and/or histopathological features of renal biopsy and morphoconstitutional analysis of the calculi. Results. There were 25 patients with a gender ratio of 1.78. The median age at onset of symptoms was 18 years. A delay in diagnosis more than 10 years was noted in 13 cases. The genetic study found the I244T mutation in 17 cases and 33-34 InsC in 4 cases. A kidney biopsy was performed in 5 cases, on a native kidney in 4 cases and on a graft biopsy in one case. The analysis of calculi was done in 10 cases showing type Ic in 2 cases. After a median follow-up of 13 years (1 year–42 years), 14 patients progressed to end-stage chronic renal failure (ESRD). The univariate study demonstrated a remarkable association with progression to ESRD in our population (44% vs. 56%) RR = 13.32 (adjusted ORs (95% CI): 2.82–62.79) (p

Chronic kidney disease (CKD) treated by hemodialysis (HD) is a worldwide major public health problem. Its incidence is getting higher and higher, leading to an alarming social and economic impact. The survival of these patients is significantly low, especially during the first year of treatment. The purpose of our study was to identify the epidemiological and clinico-biological characteristics of patients at the HD initiation and to reveal the predictive factors of mortality at three months and one year of HD. This is a prospective, analytical, and descriptive study dealing with 229 patients with an end-stage renal disease (ESRD), followed up in the Nephrology Department of Charles Nicolle Hospital and La Rabta Hospital in Tunisia, that was started HD between January and June 2017. A multivariate logistic regression analysis allowed us to identify the independent predictors of mortality at three months and one year. The average age was 60.2 ± 15.3 years, with a gender ratio of 1.41. Seventy-eight percent of patients had more than two comorbidities, 59% had diabetes, and 88% had hypertension. Diabetic nephropathy was the leading etiology of kidney disease (48.9%), while 11% of nephropathies were of unknown etiology. Only 58% were early referred to a nephrologist. The average glomerular filtration rate at HD initiation was 6.06 ± 2.33 mL/min/1.73 m2. Hypocalcemia and hyperphosphatemia were noted, respectively, in 60.8% and 84.9% of cases. Anemia was objectified in 98.6% of cases. HD was started in an emergency in 56.8% of cases. One of the most urgent indications was acute pulmonary edema (APE) for 43.8% of patients. Only 10.5% of patients had functional arteriovenous fistula at the dialysis initiation. Patients were hemodialyzed one, two, or three sessions per week, respectively, in 23.2%, 26.6%, and 50.2% of cases. The crude mortality rate was 25% and 13% in, respectively, one year and three months of HD. On multivariate analysis, we identified heart failure and insufficient dialysis dose per week as predictive factors of mortality at the 1st year of HD. C-reactive protein more than 21 mg/L, insufficient dialysis per week, modified Charlson Comorbidity Index less than 6, and APE at the dialysis initiation were identified as predictive factors of three-month mortality. Despite the short period of study, this work revealed the alarming conditions of patients at HD initiation. This critical situation is due to the delay in CKD diagnosis, the late nephrologist referral, and the lack of preparation before HD initiation.

Abstract Among salt‐wasting tubulopathies' complications, venous thrombosis is one of the rarest. We report a case of a young woman with Gitelman syndrome (GS). She presented a deep venous thrombosis in her leg and was treated with heparin with favorable outcomes. We retained hypomagnesemia as the cause of the thrombosis.

Abstract Acute pancreatitis may be the first manifestation in systemic lupus erythematosus or occur during evolution. It is a rare complication, which is often associated with other visceral manifestations. Outcome is usually favorable but can be serious. We report a case of a 17‐year‐old girl with a past history of systemic lupus erythematosus who developed acute pancreatitis revealed by abdominal pain. Elevated serum amylase and lipase levels and pancreatic enlargement on tomography confirmed the diagnosis. Although high‐dose corticosteroid was prescribed, the patient died from a refractory diabetic ketoacidosis.

Background. Renal amyloidosis is one of the main differential diagnoses of nephrotic proteinuria in adults and the elderly. The aim of this study with the most important series in our country is to contribute to the epidemiological, clinical, and etiological study of the renal amyloidosis. Methods. In a retrospective study carried out between 1975 and 2019, 310 cases of histologically proven and typed renal amyloidosis were selected for this study. Results. There were 209 men and 101 women with a mean age of 53.8 ± 15.4 years (range, 17–84 years). Of the 310 cases, 255 (82.3%) were diagnosed with AA renal amyloidosis and 55 (17.7%) with non-AA amyloidosis. Infections were the main cause of AA amyloidosis, and tuberculosis was the most frequent etiology. The period from the onset of the underlying disease to diagnosis of the renal amyloidosis was an average of 177 months. The most frequent manifestations at the time of diagnosis were nephrotic syndrome (84%), chronic renal failure (30.3%), and end-stage renal disease (37.8%). After a medium follow-up of 16 months (range, 0–68 months), mortality occurred in 60 cases. Conclusions. Given the high frequency of AA amyloidosis in our country, awareness of the proper management of infectious and chronic inflammatory diseases remains a priority in reducing the occurrence of this serious disease.

Angiotensin-converting enzyme inhibitors (ACEI) are commonly prescribed drugs for blood pressure (BP) control and renal protection. The use of ACEI is not associated with an increased risk of acute pancreatitis and ACEI-induced angioedema is rare. A 36-year-old woman presented with vomiting, headache, and aphasia. Her BP was 220/100 mm Hg. urine analysis revealed proteinuria (2+), hematuria (3+). Serum creatinine level was at 1125 μmol/L. She had anemia with 6.1 g/dL of hemoglobin and thrombocytopenia (61,000/mm3). Renal histology revealed lesions of thrombotic microangiopathy. The diagnosis of atypical hemolytic uremic syndrome was made by the complement factor I deficiency. Plasma exchanges could not be done. She was placed on peritoneal dialysis for renal insufficiency. We introduced an ACE (captopril) for the treatment of high BP. Twelve-hours after taking the first dose, she experienced severe epigastric pain and two episodes of vomiting. Serum lipase was 560 IU/L, and abdominal computed tomography showed Stage B pancreatitis. Twenty-four hours later, the patient developed marked edema of the neck region without dyspnea or dysphonia. Cervical ultrasound revealed the infiltration of the subcutaneous tissues. Captopril was stopped with the progressive disappearance of the edema. Serum lipase was 350 IU/L and then normalized at the end of the 4th day. Clinicians should be careful about widely used drugs and their side effects. ACEI can cause potentially life-threatening complications such as angioedema and acute pancreatitis. Possibly, there could be a common mechanism for the onset of pancreatitis and angioedema under ACEI.

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.

Thalidomide, which is an angiogenesis inhibitor and immunomodulator that reduces tumor necrosis factor-alpha, has regained value in the treatment of multiple myeloma. Serious pulmonary complications due to thalidomide use remain relatively uncommon. We describe a case of bronchiolitis obliterans organizing pneumonia (BOOP) due to thalidomide. A 51-year-old man with IgG lambda myeloma was treated with thalidomide and dexamethasone. Seven days after the beginning of chemotherapy, the patient presented a fever and a persistent cough. Auscultation revealed crackles in both pulmonary bases. The chest X-ray showed a diffuse bilateral alveolar–interstitial syndrome. Computed tomography scan revealed bilateral pulmonary involvement, with bilateral interstitial alveolar infiltration and ground-glass pattern consolidations. Pulmonary infection, malignant tumor, and lung involvement of multiple myeloma were excluded through various tests. Thalidomide-induced BOOP was suspected, and the drug was withdrawn and replaced by Melphalan. The patient had complete resolution of his symptoms and radiologic pulmonary involvement on discontinuation of the drug. In the absence of other etiologies, physicians should be cognizant of this potential complication in patients receiving thalidomide who present with respiratory symptoms.

BackgroundPrimary membranous nephritis (PMN) is an autoimmune disease induced by the deposit of antibodies (Ab) to the phospholipase receptor A2 receptor (PLA2R) on podocytes. In this context, we aimed to assess the relationships between anti-PLA2R Ab, PLA2R rs4664308 SNP, PLA2R mRNA levels and PMN susceptibility and outcome.MethodsSixty-eight PMN patients, 30 systemic lupus erythematosus (SLE) patients with secondary MN and 30 healthy control subjects served for anti-PLA2R Ab measurement by ELISA and PLA2R rs4664308 SNP genotyping by a commercial real-time PCR. Twenty patients with tubulo-interstitial nephritis (TIN) were used as controls for renal PLA2R mRNA quantification in PMN patients from kidney biopsies. PLA2R mRNA quantification was carried-out by real-time PCR after RNA extraction.ResultsForty-three (63.2%) PMN patients received initial therapy consisting of alternating monthly cycles of corticosteroids and cyclophosphamide. Twelve (17.6%) patients had resistant PMN to initial therapy and were consecutively treated by cyclosporine or tacrolimus. Anti-PLA2R Ab were positive in 54 (79.4%) PMN patients, while all SLE patients and controls were negative, pConclusionAnti-PLA2R Ab and renal PLA2R mRNA could be useful markers for PMN outcome predicting. The PLA2R rs6446308 SNP is associated with PMN susceptibility in Tunisians.

The arteriovenous fistula (AVF) is the vascular access of the first choice for hemodialysis (HD). Studies on patency of AVF and its affecting factors reveal a high risk for access failure. The aim of this study was to assess the primary and secondary AVF patency and their determinant factors. It was a retrospective, descriptive study conducted in the HD facility of the Nephrology Department in Rabta University Hospital. We included AVF created before December 2009 in end-stage renal disease (ESRD) patients. The end of the follow-up was fixed in December 2013. We included 126 AVFs created in 111 patients; 22.5% were aged >65 years, 39.6% were diabetic, 68.5% were hypertensive, and 26.1% had peripheral vascular disease. The primary patency rates were 78% at one year and 42% at five years. The secondary patency rates were 80% at one year and 69% at five years. Multivariate analysis revealed that the factors affecting the primary patency of AVF were: the use of jugular catheter for longer than three months (odds ratio (OR):1.91, P = 0.044) and a C-reactive protein >5 mg/L (OR: 1.7, P = 0.049). Aging (>65 years) (OR: 2.46, P = 0.042), referral time to a nephrologist

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.

Autosomal recessive polycystic kidney disease (ARPKD) is the most common childhood-onset ciliopathy. Intracranial aneurysms (ICA) are a serious complication of autosomal dominant polycystic kidney disease (ADPKD). However, there are only three reports about ICA in an adult patient with ARPKD. We describe a rare case of a 29-year-old man with ARPKD presenting with subarachnoid hemorrhage secondary to a ruptured intracranial aneurysm. The diagnosis of ARPKD was at the age of eight years based on typical ultrasonography findings with polycystic kidneys and liver disease. Magnetic resonance cholangiography showed a nonobstructive dilatation of intrahepatic bile ducts. Liver biopsy showed hepatic fibrosis. None of the family members was affected. At the age of 15 years, he had progressed to end-stage kidney disease, and hemodialysis was started. The patient had always a severe arterial hypertension. At the age of 29 years, he complained of headaches with an uncontrolled hypertension and disturbance of consciousness, computed tomography angiography showed subarachnoid hemorrhage and multiple cerebral aneurysms. Early neurologic screening of intracranial aneurysm should be recommended in ARPKD like in ADPKD patients.

Diabetic nephropathy (DN) is one of the major complications of type 2 diabetes mellitus (T2DM). The diagnosis of DN is mostly clinical. Kidney biopsy is indicated only if nondiabetic renal disease (NDRD) is suspected. This study is aimed to assess the prevalence of NDRD and to determine predictor and prognostic factors of DN, NDRD. It was a retrospective analytic study including T2DM patients in whom renal biopsies were performed at our department from 1988 to 2014. Seventy-five patients were included. Mean age was 52.7 years with sex ratio at 1.56. Renal biopsy findings were isolated NDRD in 33 cases, NDRD superimposed on DN in 24 cases, and isolated DN in 18 cases. Most common NDRD found were focal segmental glomerulosclerosis (21%) and membranous nephropathy (19%). Multivariate analysis showed that the absence of ischemic heart disease [odds ratio (OR) = 0.178, 95% confidence interval (CI) = 0.041–0.762], absence of peripheral vascular disease (OR = 0.173, 95% CI = 0.045–0.669), and presence of hematuria (OR = 7.200, 95%CI = 0.886–58.531) were independent predictors of NDRD. 24 patients reached end-stage renal disease 55% in DN group, 16% in DN associated to NDRD group, and 30% in NDRD group. The prevalence of NDRD found in our study confirmed usefulness of renal biopsy in patients with T2DM, especially in those without degenerative complications, hypertension, and insulin therapy.

Mortality in systemic lupus erythematosus (SLE) has decreased with the advent of immunosuppressive therapy and the development of hemodialysis. This study aims to evaluate the survival rate, factors of poor prognosis, and causes of death in SLE in a Tunisian series. The records of all SLE patients followed up in a single center during 1974–2014 were reviewed. The causes of death were identified. Prognostic factors of survival were analyzed by multivariate analysis using the comparison of the survival rates by the log-rank test. Two hundred ninety-nine patients with SLE were included (274 women, 25 men) aged meanly of 27.52 years at diagnosis. The death occurred in 50 cases (16.7%). The mean age at death was 28.46 years (14–69 years). The patient survival rates at 5, 10, and 20 years were 83.8%, 78.6%, and 56.7%, respectively. The leading causes of death were active SLE (50%) and infectious complications (36%). Independent factors of poor prognosis identified by multivariate analysis were myocarditis (P = 0.029), splenomegaly (P = 0.0015), and worsening of renal function (P = 0.004). Remission was identified as a protective factor (P = 0.047). Our study shows that renal disease remains the primary cause and the main predictor of death in SLE, which is consistent with the literature data.

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.

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

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.

A phosphate binder combining calcium and magnesium offers an interesting therapeutic option to control hyperphosphatemia in dialysis patients. We investigated the effectiveness and tolerance of calcium acetate-magnesium carbonate (Ca-Mg). This is a 16-week prospective study including 16 dialysis patients. After an initial two-week washout period, serum phosphorus (sPho) ≥1.8 mmol/L, serum calcium (sCa) ≤2.6 mmol/L, and serum magnesium ≤1.5 mmol/L were the main inclusion criteria. The initial dose of Ca-Mg depended on sPho level and was titrated for every two weeks to have a sPho ≤ 1.8 mmol/L. A second two-week washout period followed the 12 weeks of treatment. Ca-Mg significantly reduced the mean sPho levels from 2.14 to 1.75 mmol/L by the end of the 12-week treatment period (P

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.

La maladie céliaque (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.

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.

Malgré les progrès réalisés dans le traitement de l'nsuffisance 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.

Urinary tract infection (UTI) is the most frequent infectious complication among renal transplant recipients and a frequent cause of bacteremia, sepsis and acute graft failure. To evaluate the incidence, risk factors, type of pathogens and long-term effect of UTIs on graft and patient survivals in our center, we performed a retrospective cohort study reviewing the medical records of patients who received a renal transplant at our center from June 1986 to December 2009, excluding patients who lost their grafts in the first month due to arterial or veins thrombosis and acute antibody-mediated rejection. We studied 393 kidney-transplanted recipients; at least one UTI occurred in 221 (53.69%) patients during the follow-up period. The most frequent pathogens isolated in urine culture were Escherichia coli (n = 39, 18.4%) and Klebsiella pneumonia (n = 31, 14.6%). When patients with UTIs were compared with those without UTIs, female gender and use of mycophenolate mofetil or azathioprine seemed to be risk factors for UTIs on univariate analysis. However, female gender was the only independent risk factor on multivariate analysis RR = 1.964 (1.202-3.207), P = 0.007. This study confirmed that UTIs remain a major problem in renal transplant recipients, and female gender was the only independent risk factor.

Kidney transplantation remains the best treatment option of end-stage renal disease. Kidney donations are of particular interest with the currently increasing practice of living-donor transplantation. The purpose of this study was to analyze retrospectively the general health status as well as renal and cardiovascular consequences of living-related kidney donation. A total of 549 living-related kidney donors had donated their kidneys between 1986 and 2007. We attempted to contact all donors to determine short- and long-term outcome following kidney donation. All kidney donors who responded underwent detailed clinical and biochemical evaluation. The data were compared with age-matched health tables of the Tunisian general population. In all, 284 donors (52%) had a complete evaluation. They included 117 men and 167 women with a mean age of 42 ± 12 years. The major peri-operative complications that occurred in these donors included four cases of pneumothorax, six cases of surgical site infection, one case of phlebitis and one case of pulmonary embolism. None of the study cases died. The median length of hospital stay after donor nephrectomy was 6.5 days (range: 3-28 days). The median follow-up period was eight years. The mean creatinine clearance after donation was 90.4 ± 25 mL/min in men and 81.5 ± 27.2 mL/min in women. Proteinuria was >300 mg/24 h in 17 cases (5.9%). Fifty-eight (20.4%) donors became hypertensive and 19.6% of the men and 37.2% of the women became obese. Diabetes mellitus developed in 24 (8.4%), and was more common in patients who had significant weight gain. Our study suggests that kidney donors have minimal adverse effects on overall health status. Regular follow-up identifies at-risk populations and potentially modifiable factors. Creation of a national registry of living donors and their monitoring are an absolute necessity.

Cette étude a pour objectif d'évaluer la fréquence des facteurs de risque cardiovasculaires au cours du lupus et de préciser leur prévalence. Etude rétrospective portant sur 250 patients ayant un lupus, diagnostiqué selon les critères de l'ACR, hospitalisés entre 1970 et 2013. Les données cliniques et para cliniques ont été recueillies à partir des observations médicales. Il s'agit de 228 femmes et 22 hommes d'âge moyen au diagnostic du lupus de 30, 32 ans (extrêmes: 16-69). La durée moyenne du suivi des patients était de 64 mois (extrêmes: 7 jours- 382mois). Quatre vingt dix patients (36%) étaient hypertendus, 74% avaient une hypercholestérolémie et 22% étaient diabétiques. Pour les autres facteurs de risque cardiovasculaire traditionnels, un âge = 50 ans a été retrouvé dans 40% des cas, le sexe masculin dans 8% des cas, l'obésité dans 76% des cas et le tabagisme dans 11% des cas. Les facteurs de risque surajoutés sont représentés par la présence des anticorps antiphospholipides (47% des cas), la néphropathie lupique (49% des cas), l'insuffisance rénale (42% des cas), la corticothérapie au long cours (74% des cas) et la chronicité de la maladie dans 35% des cas. Les complications cardiovasculaires retrouvées dans notre série étaient: les accidents vasculaires cérébraux (2%) et l'insuffisance coronarienne (5,6%). Devant l'importance du risque cardiovasculaire au cours du lupus, une surveillance rapprochée des facteurs de risque cardio-vasculaires semble primordiale chez les lupiques.

Hereditary complete C4 deficiency has until now been reported in 30 cases only. A disturbed clearance of immune- complexes probably predisposes these individuals to systemic lupus erythematosus, other immune- complex diseases and recurrent microbial infections. We present here a 20- year- old female with hereditary complete C4 deficiency. Renal biopsy demonstrated renal AA amyloidosis. This unique case further substantiates that deficiency of classical pathway components predisposes to the development of recurrent microbial infections and that the patients may develop AA amyloidosis. Furthermore, in clinical practice, the nephrotic syndrome occurring in a patient with hereditary complete complement C4 deficiency should lead to the suspicion of renal AA amyloidosis.

Microscopic polyangiitis is a systemic necrotizing vasculitis of the small vessels. Its typical clinical manifestations are rapidly progressive glomerulonephritis and alveolar hemorrhage. We describe a 30-year-old woman with rapidly progressive glomerulonephritis. Seven years later, she presented with partial loss of motor and sensory function in both lower limbs with sphincter dysfunction. This is the first reported case of epidural inflammation ascribed to microscopic poly-angiitis. Possible mechanisms include auto-immune disease.

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 mem-branous glomerulonephritis associated with a focal segmental glomerulosclerosis. She was started on corticosteroid treatment with good outcome.