Uncovering the Spectrum of Hemophagocytic Lymphohistiocytosis: A Nephrology Department's Analysis of 14 Cases.

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.

Multiple Myeloma in a Young Adult with Renal Involvement.

Multiple myeloma (MM) results from malignant plasma cell disorder. It represents approximately 10% of hematological malignancies and it is typically diagnosed in the elderly with a median age of 70 years and has a steep increase in incidence with advancing age. N Engl J Med. 2004, 1860; Clin Interv Aging. 2020, 619. The incidence of MM has been increasing over time, mostly due to population aging. Mayo Clin Proc. 2010, 225 However, certain MMs are diagnosed at young age even under 40 years old (2%). Leuk Lymphoma. 1998, 493; Blood. 2010, 5501. We report a case of a MM in a thirty-four-year-old woman whose circumstance of discovery was acute kidney failure.

Incidence and challenges in management of hemodialysis catheter-related infections.

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 < 0.04). We did not notice significant difference in mortality between tunnelled and non-tunnelled catheters. CRI does not seem to be more severe in patients with diabetes. Duration of use of the HD catheter (p < 0.007) and ferritin level (p < 0.0001) were independent factors that predispose to CRI in our population.

Peritoneal dialysis in the era of COVID-19: experience of a Tunisian center.

The COVID-19 pandemic has transformed the health landscape by hampering the management of patients with chronic diseases. Providing optimal healthcare has become a critical issue, especially for patients with end-stage renal disease (ESRD) receiving in-center dialysis. Peritoneal Dialysis (PD) has the advantage of being a home-based therapy. Several papers about COVID-19 in the chronic kidney disease (CKD) population have been published, but few studies focused on the PD population, with limited case series. In this paper, we share our strategy for managing PD patients during the pandemic and describe the characteristics of 24 episodes of COVID-19 that occurred in our PD patients. Also, we report the impact of the pandemic on different outcomes and discuss the challenges of renal replacement therapy (RRT) in the time of COVID-19 and the advantages of PD. During the period from December 2019 to September 2021, 127 patients received PD in our center. Among them, we recorded 24 episodes of COVID-19 that occurred in 20 patients, corresponding to an incidence of 8.4 per 1000 patient-months. None of the 20 patients with COVID-19 were vaccinated and there was a significant male gender predominance in the COVID-19 group compared to the non-COVID-19 group. The prevalence of diabetic nephropathy and primary glomerulonephritis were also significantly higher in the COVID-19 group. The revealing symptoms were asthenia, dry cough, and the deterioration of general conditions in 100%, 75%, and 63% of the patients, respectively. A biological inflammatory syndrome was found in 30% of the patients. Chest computed tomography (CT) scan, performed in 5 patients, showed features of COVID pneumonia with an average extent of damage of 55%. The rate of patients starting PD during the study period was comparable to that before the pandemic. Furthermore, we did not find a significant difference between the infected and the non-infected groups regarding the incidence of peritonitis, PD technique failure, and mortality (6.1 [0-1.46] vs 3.9 [0.15-0.64] deaths per 1000 patient-months. COVID-19 does not seem to have influenced the outcomes of our patients treated with PD even before the launch of mass immunization in our country. Thus, PD can be a great option for RRT in the era of the COVID-19 pandemic since many issues could be managed remotely to avoid regular hospital visits and contribute to maintaining social distancing, which is the cornerstone of breaking the chain of transmission of the novel virus.

Thirty years of experience with anti-neutrophil cytoplasmic antibody glomerulonephritis in Charles Nicolle Hospital-Tunisia: a retrospective cohort study.

Introduction: antineutrophil cytoplasmic antibodies (ANCA) associated Glomerulonephritis (GN) is rare but a life-threatening disease especially, particularly in patients with advanced renal failure at presentation. This study aims to evaluate the epidemiological, clinical and histopathological features of renal involvement and investigate factors associated with ESRD. Methods: patients with renal biopsy-proven ANCA associated glomerulonephritis were included retrospectively over a thirty years period. The renal survival, defined as time to reach ESRD, was evaluated based on clinical parameters, histopathological classification as well as the renal risk score. Results: a total of 65 patients with crescentic GN were included in the study. The mean age was 47.9 years ± 22.4 years (range: 18-78) with an M/F sex ratio at 1.13. Hematuria, proteinuria and oliguria were found in respectively 100%, 81.5% and 56.2% of cases. Sixty patients (92.3%) had renal failure at presentation, and 30 patients (46%) required initial hemodialysis (HD) therapy. The pattern of glomerular injury was categorized as mixed in 43.7% of cases, sclerotic in 34.3%, crescentic in 16.6%, and focal class in 6%. Regarding renal risk score, patients were classified in the category low risk, intermediate risk and high risk with respectively 16.9%, 44.6% and 38.4%. All patients received corticosteroids and immunosuppressive treatment. Complete, partial remission and relapses were noted in respectively 15.3%, 18% and 72% of cases. Factors associated with ESRD were serum creatinine level >500 µmol/l (P=0,0016), CRP level >60 mg/l (P = 0,0013), interstitial fibrosis (P=0,0009) and glomerulosclerosis> 10% of total glomeruli (P=0,001). The survival rate was 89%, 60.9% and 32.8% at respectively 1, 5 and 10 years. Death occurred in 10 cases (15%) caused mostly by infections (40%). Initial serum creatinine level>140 µmol/l (P=0,02), alveolar hemorrhage (P=0.001) and infections (P=0,0001) were associated with mortality. Conclusion: in our cohort of ANCA GN, confirms the data showing improved patient survival but constantly high relapse risk. In addition, we observed that ANCA GN classification was predictive, as the risk of progressing to ESRD increased with the ascending category of focal, crescentic, mixed and sclerotic GN.

Renal Amyloidosis: Epidemiological, Clinical, and Laboratory Profile in Adults from One Nephrology Center.

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.

[Renal involvement in sarcoidosis: Prognostic and predictive factors]. / Atteinte rénale au cours de la sarcoïdose : facteurs pronostiques et prédictifs.

INTRODUCTION: Sarcoidosis is a systemic granulomatous disease that primarily affects the respiratory system and lymphatic vessels. Renal involvement is rare, poorly studied and found in less than 10% of cases. The objective of our study was to identify factors of poor renal prognosis and predictive factors of renal involvement during sarcoidosis. METHODS: It's a retrospective study including patients hospitalized in our department for sarcoidosis with renal involvement over a period of 40 years. To study renal survival, we identified two groups of patients with renal manifestations of sarcoidosis by following their evolution: group A (n=26) represents those with renal remission or deterioration of renal function but without progression to end-stage renal disease and group B (n=8) those with progression to end-stage renal disease. To detect the predictive factors of end-stage renal disease in patients with sarcoidosis, we compared the clinical and paraclinical characteristics of our patients (group 1) to those of 44 patients with sarcoidosis without renal impairment followed in our department during the same period (group 2). RESULTS: Renal involvement was observed in 34 patients hospitalized for sarcoidosis (43.6%). There were 28 women and 6 men with a sex ratio of 0,21. The mean age at diagnosis of sarcoidosis was 47.1 years. The median time from sarcoidosis diagnosis to renal disease was 2 months (range 1-72). Tubulointerstitial nephropathy was the most frequent renal manifestation observed in 24 patients (70.6%). Hypercalcemia and hypercalciuria were found in 52.9% and 46.4% respectively. Renal failure was noted in 25 patients (73.5%). Corticosteroid therapy was initiated in 33 patients (97%) associated with immunosuppressive therapy in 3 cases. Predictive factors of end-stage renal disease were advanced age at diagnosis of nephropathy (P=0.007), comorbidities (P=0.002), multi-organ involvement (P=0.041), initial renal failure (P=0.013), interstitial fibrosis (P=0.006) and renal granulomas (P=0.007). Predictive factors of renal impairment during sarcoidosis were multi-organ involvement, inflammatory syndrome and hypercalcemia. CONCLUSION: Renal envolvement, although rare during sarcoidosis, can influence the prognosis hence the great interest of its early detection to prevent progression to end-stage renal failure.

Nephrotic syndrome in elderly: Etiologies, management, and prognosis.

The elderly population has significantly increased in the world. Nephrotic syndrome (NS) is one manifestation of glomerular nephropathy in the elderly. The objective of our study is to determine NS particularities in the elderly and to identify the factors predicting progression to chronic end-stage renal disease (ESRD). This is a retrospective and descriptive study, carried out between January 1, 1975 and December 31, 2017. It includes participants aged 65 years old or over hospitalized for NS. A multivariate study was carried out and the dependent variable was the evolution to ESRD. We studied 115 patients with an average age of 71 ±5 years (65-83) and a sex ratio (male/female) of 1.7. Twenty-three percent of patients were diabetic. The median proteinuria was 4.7 g/L (3-19.5). NS was impure in 89.5% of patients. Renal biopsy was performed in 45 patients (39.13%). NS was secondary in 65.2% of cases mainly to amyloidosis (35.6%). Idiopathic nephropathy was dominated by membranous nephropathy (9.5%). Treatment was etiopathogenic in only 18 patients (15.6%). At the end of follow-up, 15.8% of patients achieved complete or partial remission and 56.6% progressed to ESRD. The multivariate study found as independent risk factors of progression to ESRD: uremia >17 mmol/L [adjusted odds ratio (aOR) = 33.2 (1.3-837.7); P <0.05],, phosphoremia ≥1.6 mmol/L [aOR = 22.1 (1.8-266.5); P <0.05], serum potassium ≥4.3 mmol/L (aOR = 24.7 (2.4, 251.5); P <0.01], extra-renal signs [aOR = 38.9 (2.4-634.3); P = 0.01], secondary nephropathy [aOR = 74 (3.1-1788.2); P <0.01] and membranoproliferative glomerulonephritis [aOR = 48 (1.4-1675.5); P <0.05]. The protective factors were hemoglobinemia ≥9.3 g/dL [aOR = 0.007 (0-0.2); P <0.01], kidneys well differentiated [0.032 (0.003-0.4); P<0.01] and treatment with two diuretics [aOR = 0.03 (0.00-30.4); P <0.01]. It is important to recognize the spectrum of kidney diseases in the elderly to improve the progression factors to ESRD.

End-stage renal disease at dialysis initiation: Epidemiology and mortality risks during the first year of hemodialysis.

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.

National Epidemiological Study about Hepatitis C Virus Infection among Dialysis Patients.

The World Health Organization estimates that 3% of the general population is infected with this virus. Hepatitis C remains the main viral infection in dialysis patients, and the severity of this infection is the risk of developing cirrhosis or hepatocarcinoma. We aim to determine the prevalence of hepatitis C in dialysis patients, to calculate the rate of hepatitis C virus (HCV) seroconversion, and to identify the risk factors for seroconversion. This is a nationwide multicenter observational study including all dialysis patients regardless of age and gender. Those with acute renal failure and vacationers were excluded from the study. We included 185 centers including 176 hemodialysis (HD) centers and nine peritoneal dialysis (PD) centers with a total number of patients at 11,238, a number of HCV-positive patients at 402, and a number of functional machines at 3139. The mean age of a patient was 55.6 years (range: 18-65), and sex ratio was 0.9. The prevalence of HCV-positive patients is 3.6%; it is higher in private centers with an average of 2.7 compared to 1.18 in public centers with a significant difference (P = 0.009). The prevalence of HCV-positive patients was significantly higher in HD centers compared to PD centers, in centers where the number of generators was >15 and when the number of patients per center is >70. One hundred and fifty-six patients seroconverted after dialysis, i.e., a prevalence of seroconversion at 1.3% with a mean delay of 6.052 ± 5.3 years. Our study shows a lower prevalence of HCV seroconversion than that reported in the literature; this requires a national survey to be carried out by homogenizing virological diagnostic kits and treating infected patients to eradicate this infection in dialysis patients.

Clinical study on autosomal dominant polycystic kidney disease among North Tunisians.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disease, which usually manifests in adulthood. It is characterized by the development of multiple cysts in the kidneys and many other extrarenal manifestations. We aimed to determine the factors that contribute to the progression of ADPKD to end-stage renal disease (ESRD). In a retrospective multicentric study, we reviewed the records of 569 patients with ADPKD, hospitalized at a nephrology department or followed up at the outpatient department of university and regional hospitals, covering the north and center of the country, during the period 1969-2016. The mean age of the study patients was 48.54 ± 13.68 years and 14% were young adults (<40 years). There were 272 female and 297 male patients (sex ratio: male/female = 1.09). A family history of ADPKD was found in 43.7% of cases. Renal symptoms were dominated by loin pain, renal failure, hypertension, and hematuria, seen in, respectively, 51.9%, 48.2%, 29.1%, and 24.6% of the patients. The median serum creatinine level was 459 µmol/L (range: 47-2454), and hypertension had preceded the onset of ADPKD in 28.8% of cases. Extrarenal manifestations consisted of urologic complications (54.6%), liver cysts (43.5%), cardiac involvement (31.9%), cerebral aneurysms (12.9%), and gastrointestinal involvement (9.4%). ESRD occurred in 43.1% after a mean follow-up of 47 months (range: 0-384). Risk factors for poor renal prognosis were age >40 years (P = 0.009), hematuria (P = 0.034), hemoglobin >14 g/dL (P = 0.0013), high uric acid level (P = 0.001), and leukocyturia (P = 0.02). Death occurred in 59 cases (10.3%), mostly caused by infections (44.1%). In our study, ADPKD was lately diagnosed in most cases. Family screening is important, which will enable early detection and management of the complications associated with ADPKD.

Tubulointerstitial nephritis due to sarcoidosis: Clinical, laboratory, and histological features and outcome in a cohort of 24 patients.

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.

Management of acute pyelonephritis.

INTRODUCTION: Acute pyelonephritis (APN) results from bacterial invasion of the renal parenchyma. The aim of this study was to investigate the epidemiological, clinico-biological, therapeutic and evolutionary APN and to identify the risk factors of this renal disease. METHODS: We conducted a retrospective and descriptive study, over a period of 37 years (1977-2014), including 43 patients with APN, hospitalized in our Department. RESULTS: There were 43 patients with mean age of 36.98 years. The sex ratio M/W was 0.39. The history of our patients was diabetes (16.28%), recurrent urinary tract infection (25.58%), chronic kidney failure (CKF) (46.51%), Renal lithiasis (9.3%), malformative uropathy (9.3%) and immunosuppressive therapy (23.3%). Typical clinical triad (fever, back pain and urinary functional signs) was present in 25 patients (58.14%). The Gram-negative bacilli represented 97.67% of bacteria isolated in urine culture, dominated by Escherchia coli in 83.72% of cases. APN was simple in 19 cases (44.18%) and at risk of complications in 24 cases (55.81%). Thirty-nine patients (90.69%) had non-severe APN. Four patients (9.3%) had severe APN. Complications were found in 30.23% of cases: acute renal failure (ARF) (30.23%), renal abscess (2.33%), septic shock (4.65%). Risk factors for developing ARF were the higher age group to 60 years (p = 0.02) and CKF (p = 0.001). A significant correlation was found between vesicoureteral reflux and recurrent infections (p = 0.01). Five years renal survival was 69%. CONCLUSION: APN induced ARF is a serious risk for progression to CKF.

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.

[Renal prognosis of IgA vasculitis nephritis in adult patients: a monocentric study of 25 cases]. / 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.

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.

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.

Predictive factors of mortality in a tunisian cohort with systemic lupus erythematosus.

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.

Outcome of Renal Transplant in Recipients With Vasculitis.

OBJECTIVES: End-stage renal disease develops in a high percentage of patients with vasculitis, in whom kidney transplant has become a therapeutic option. However, limited data are available on the prognosis and outcomes after kidney transplant in these patients. We aimed to compare the long-term graft survival and graft function in 8 renal transplant recipients with vasculitis (granulomatosis with polyangiitis, microscopic polyangiitis, Goodpasture syndrome, and Henoch-Schonlein purpura) with the other kidney recipients at a single center. MATERIALS AND METHODS: We conducted a retrospective study of patients followed for chronic renal failure associated with vasculitis before renal transplant. We excluded patients with no biopsy-proven nephropathy. RESULTS: There was no difference in the occurrence of metabolic and cardiovascular complications in our case group compared with the other graft recipients. Infections were frequent and included cytomegalovirus and urinary tract infection. The rates of bacterial and viral infection were equivalent in our population. The incidence of allograft loss was estimated at 1.8%, less than that seen in our entire transplant population. The presence of vasculitis was not significantly related to renal failure (P = .07). Extrarenal relapse occurred in 1 patient with microscopic polyangiitis. Antineutrophil cytoplasmic antibody levels in patients with granulomatosis with polyangiitis and microscopic polyangiitis did not seem to influence the renal outcome (P = .08). Circulating antineutrophil cytoplasmic antibodies were associated with the development of vascular lesions in the graft but were not significantly correlated with graft survival (P = .07). CONCLUSIONS: This study supports the theory that renal transplant is an effective treatment option for patients with end-stage renal disease secondary to vasculitis. These patients fare similarly to, if not better than, other patients.

Outcome of Kidney Transplant in Patients with Polycystic Kidney Disease: A Single-Center Study in Tunisia.

OBJECTIVES: Autosomal dominant polycystic kidney disease is a common cause of end-stage renal disease and a common indication for renal transplant. This study was undertaken to evaluate the demographics, outcomes, and complications of renal transplant in patients with autosomal dominant polycystic kidney disease compared with other nephropathies. MATERIALS AND METHODS: In a retrospective case-control design, we reviewed the records of 7 patients with autosomal dominant polycystic kidney disease from a total of 701 renal transplant patients over a 30-year period (1986-2016). For each patient, a matched control was selected based on sex, age, year of transplant, and type of kidney donor. We excluded patients who underwent kidney transplant abroad and those with a follow-up period of less than 2 years. RESULTS: The number of patients with autosomal dominant polycystic kidney disease requiring transplant at our center was estimated at 0.23 per year, and the condition represented 1.57% of initial nephropathy causes. The mean patient age at transplant was 50.8 ± 8.05 years. There were 5 male and 2 female patients in the case group, with a male-to-female ratio of 2.5. The source of the graft was predominantly a living related donor (5/7). Four patients had extrarenal manifestations, the most common of which were liver cysts (3 patients). Rejection occurred in a single study patient (14.2%) and in 4 control patients (57.1%; P = .51). Two patients did not develop any complications. Complications noted after transplant included infection (3/7 cases vs 2/7 controls; P= .67) and cerebrovascular accidents (2/7 cases vs 0/7 controls). CONCLUSIONS: Further studies with longer follow-up and greater numbers of patients are needed to compare more precisely the complications and results of transplant between patients with autosomal dominant polycystic kidney disease and other kidney transplant recipients.