Multisystemic Beryllium Disease: An Exceptional Case Revealed by a Urinary Tract Granulomatosis.

Chronic beryllium disease (CBD), or berylliosis, is an interstitial lung disease caused by the chronic inhalation of finely particulate beryllium, frequently mistaken for sarcoidosis. It is rarely associated with skin nodular lesions, asymptomatic granulomatous hepatitis or calcium nephrolithiasis. To date, it has never been reported as a diffused multi-organ granulomatous disease. A 60-year-old Pakistani man, a former excavation worker with ancient history of suspected sarcoidosis, underwent a left nephroureterectomy for suspected papillary kidney carcinoma. The histopathological analysis showed a benign non-necrotic granulomatous infiltration of the renal pelvis and ureter. Six months later, he suffered from two consecutive episodes of acute kidney failure. Bladder biopsies found similar noncaseous granulomatosis and kidney biopsies showed interstitial nephritis. Known for suspected asthma, sleep apnea, and usual interstitial pneumonia, the patient would regularly consult for episodes of pyrexia, chills, nocturnal coughing, and wheezing. As kidney function gradually worsened, he ultimately started hemodialysis and was transferred to our facility. A positive blood beryllium lymphocyte proliferation test confirmed the diagnosis of CBD. This original report is the first description of multi-organ berylliosis with diffused urothelial granulomatosis and pseudo-tumor. The patient's pulmonary disease is minimal compared with renal and urinary tract involvement, eventually responsible for end-stage kidney disease. Berylliosis usually responds to glucocorticoids. This case report highlights the importance of evoking the diagnosis of CBD in the presence of any granulomatosis, even extra-thoracic, especially if associated with pulmonary symptoms, however atypical.

Diagnosis and Management of Catastrophic Antiphospholipid Syndrome and the Potential Impact of the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria.

Catastrophic antiphospholipid syndrome (CAPS) is a rare and life-threatening condition characterized by the persistence of antiphospholipid antibodies and occurrence of multiple vascular occlusive events. CAPS currently remains a diagnostic challenge and requires urgent treatment. The diagnosis of CAPS is made difficult by classification criteria used as diagnostic criteria in clinical practice, knowledge derived from retrospective data and case reports, confounding clinical and biological features, and its rapid onset and mortality. The absence of prospective studies of CAPS limits the strength of evidence for guideline treatment protocols. This comprehensive review summarizes the current understanding of the disease, and discusses how the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria impact the definition and therapeutic management of CAPS, which is considered the most severe form of APS. The correct integration of 2023 ACR/EULAR APS classification criteria is poised to facilitate CAPS diagnosis, particularly in critical situations, offering a promising avenue for improved outcomes.

Antiphospholipid Antibodies Associated with Native Arteriovenous Fistula Complications in Hemodialysis Patients: A Comprehensive Review of the Literature.

Antiphospholipid antibody (aPL)-persistent positivity is frequent in hemodialysis (HD) patients. Native arteriovenous fistula (AVF) complications such as stenosis and thrombosis are among the most important causes of morbidity and mortality in hemodialysis patients. The association between aPL positivity and AVF thrombosis seems to now be well established. However, whether aPL positivity is associated with other AVF complications, such as maturation failure or stenosis, is not well known. Given the significant impact of AVF failure on patient's prognosis, it is of interest to further investigate this particular point in order to improve prevention, surveillance and treatment, and, ultimately, the patient's outcome. This literature review aims to report the recent literature on aPL-associated native AVF complications.

Efforts to Better Characterize "Antiphospholipid Antibody Nephropathy" for the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria: Renal Pathology Subcommittee Report.

OBJECTIVE: Antiphospholipid antibody (aPL) nephropathy (-N) can be challenging to recognize due to a lack of established classification or diagnostic criteria. As part of efforts to develop new antiphospholipid syndrome (APS) classification criteria (CC), the APS CC Renal Pathology Subcommittee aimed to better characterize the entity of aPL-N. METHODS: We used a 4-pronged approach that included (1) administering Delphi surveys to worldwide APS physicians to generate aPL-N terminology; (2) conducting a literature review to demonstrate the association of nephropathy with aPL and identify published aPL-N histopathological terminology and descriptions; (3) evaluating aPL-N terminology used in renal biopsy reports from an international patient registry; and (4) evaluating proposed kidney pathologic features for aPL-N by assessment of international Renal Pathology Society (RPS) members. RESULTS: After completing our metaanalysis demonstrating an association between nephropathy and aPL, we used Delphi surveys, a literature review, and international renal biopsy reports to develop a preliminary definition of aPL-N. The preliminary definition included include specific terms associated with acute (ie, thrombotic microangiopathy in glomeruli or arterioles/arteries) and chronic (ie, organized arterial or arteriolar microthrombi with or without recanalization, organized glomerular thrombi, fibrous and fibrocellular [arterial or arteriolar] occlusions, focal cortical atrophy with or without thyroidization, and fibrous intimal hyperplasia) lesions. Most RPS survey respondents agreed with this terminology and the importance of knowing aPL results for histopathological diagnosis. CONCLUSION: Our results support the inclusion of aPL-N in the 2023 American College of Rheumatology/European Alliance of Associations for Rheumatology APS CC, and provide the most widely accepted terminology to date for both acute and chronic pathologic lesions of aPL-N.

Multiple Complications of Crohn's Disease and the Need for Early and Continuous Multidisciplinary Undertaking.

Crohn's disease is an inflammatory disease that typically affects the bowels but can also have many different extraintestinal manifestations. One of those complications is immunoglobulin A nephropathy (IgAN), which is one of the most encountered renal lesions in the setting of Crohn's disease. Another point of focus for Crohn's patients is the risk of cancer, with a higher risk of colorectal cancer but also extraintestinal neoplasia such as hepatobiliary, hematological, and urinary tract neoplasia. We present the case of a young patient suffering from long-term Crohn's disease and subsequent IgAN leading to end-stage kidney disease and hemodialysis. The patient was diagnosed young and had undergone multiple surgeries and different treatments in various countries. He then presented in our center already with advanced chronic renal failure from IgAN that was unknown due to poor multidisciplinary follow-up. Shortly after starting hemodialysis, he developed a large abdominal mass, first thought to result from Crohn's-related fistula. This mass turned out to be a urachal adenocarcinoma, a rare type of bladder cancer with an especially poor prognosis. It is not known whether this type of cancer is associated with either Crohn's disease or IgAN, and no such association has been previously described. The treatment of urachal cancer usually relies on surgery, with the addition of chemotherapy in some cases. Unfortunately for our patient, his case was already so advanced at the moment of diagnosis that he was excluded from curative treatment and quickly passed away thereafter. This case illustrates many important aspects of the rigorous follow-up that is needed for Crohn's patients, with regular check-ups, screening investigations, and the need for multidisciplinary evaluation. Furthermore, it describes the development of a rare type of cancer in the setting of Crohn's disease and IgAN, with no prior established link between these different pathologies.

Evaluation of Transplant Suitability in a Patient with Previous Colorectal Cancer and Subsequent Radiation Cystitis: Insights from a Complicated Case.

Assessing transplant suitability can be a meticulous process, involving multiple investigations and various specialties. This process is well described in the latest KDIGO guidelines. We recently asked ourselves if those guidelines are still relevant to current clinical practice given the rapid evolution of modern medicine, especially in the field of oncology. We present the complicated case of a 60-year-old woman with ESKD (end-stage kidney disease) and a prior history of cancer, with secondary urological complications, to illustrate different interesting considerations for KT (kidney transplant). Our patient was diagnosed with rectal cancer at the age of 46, for which she was treated with surgery and radiotherapy before developing chronic radiation cystitis. This was followed by repeated urinary tract infections and secondary nephrolithiasis, ultimately leading to severe bilateral hydronephrosis and obstructive ESKD. We know that the type of cancer and its characteristics should be evaluated in detail, and we should offer patient-tailored recommendations after a multidisciplinary evaluation. In our case, the prior rectal cancer is not to be feared because curative treatment has been achieved and the patient has been cancer-free for 14 years, knowing that this type of cancer is not at high risk of recurrence after transplantation. The frail urological anatomy, however, represents a bigger challenge. Not only does it complicate the technical feasibility of KT but it also increases the risk of complications and graft failure. It is difficult to clearly determine KT possibility when considering it in such patients. What is clear on the other hand is that such a decision should be taken considering the choice of the patient and the involved physicians. We should also consider the potential benefits and risks of KT in order to make an informed decision.

Scleroderma renal crisis triggered by ibuprofen: Insights on complement-directed therapy.

Scleroderma renal crisis is a severe complication of systemic sclerosis with a poor prognosis. Therefore, identifying precipitating factors is essential. Among known risk factors, only few are reversible. On the contrary, anti-C5 therapy appears effective, at least in some cases. We describe a 59-year-old man with diffuse cutaneous systemic sclerosis who developed life-threatening scleroderma renal crisis following ibuprofen administration. Despite aggressive management, he did not improve. Renal biopsy have displayed features of thrombotic microangiopathy but no complement deposition. We then discuss the pathomechanism of scleroderma renal crisis that could drive eculizumab treatment since some renal biopsies exhibit complement deposits and others do not.

Native Mitral Valve Endocarditis Caused by a Non-HACEK Gram-Negative Pathogen in a Hemodialysis Patient.

Infective endocarditis (IE) due to non-HACEK (species other than Hemophilus, Aggregatibacter, Cardiobacterium, Eikenella, Kingella) bacteremia accounts for less than 2% of all IE cases but is proven to be associated with higher mortality, even more so in hemodialysis (HD) patients. Few data are available in the literature concerning non-HACEK Gram-negative (GN) IE in this immunocompromised population with multiple comorbidities. We report the atypical clinical presentation of an elderly HD patient diagnosed with a non-HACEK GN IE, namely E. coli, successfully treated with intravenous (IV) antibiotics. The objective of this case study and related literature was to highlight the limited applicability of the modified Duke criteria in the HD population, as well as the frailty of HD patients that increases their susceptibility to IE due to unexpected microorganisms that could have fatal consequences. The need for a multidisciplinary approach of an IE in HD patients is therefore imperative.

Jejunal perforation secondary to blind insertion of peritoneal dialysis catheter: a case report and review of literature.

BACKGROUND: Peritoneal dialysis (PD) depends upon a functioning and durable access to the peritoneal cavity. Many techniques exist to insert a peritoneal catheter, showing similar outcomes and benefits. Blind percutaneous insertion represents a bedside intervention predominantly performed by nephrologists requiring only local anesthesia, sedation and minimal transcutaneous access. Although current guidelines recommend insertion techniques allowing visualization of the peritoneal cavity, the blind percutaneous approach is still widely used and has been proven safe and effective to bring durable peritoneal dialysis access. Herein, we described a rare case of jejunal perforation secondary to blind PD catheter placement, and conduct a review of the current medical literature describing early bowel perforations secondary to PD catheter placement, gathering descriptions of symptomatology and outcomes and their relations to the insertion technique. CLINICAL PRESENTATION: We herein describe the case of a 48 year-old patient with a history of appendectomy who suffered from triple jejunal perforation after blind percutaneous insertion and subsequent embedment of his peritoneal catheter. Accurate diagnosis was made 1 month after insertion due to atypical clinical presentation and because physicians had no access to the peritoneal cavity after catheter embedment. After surgical repair and broad-spectrum antibiotics, the patient was switched to HD. CONCLUSION: Early catheter-related visceral injury is a rare, yet threatening condition that is almost always causing a switch to hemodialysis or death. Our review highlights that laparoscopic catheter placement might bring better outcomes if perforation occurs, as it allows immediate diagnosis and treatment. On the contrary, catheter embedment may delay clinical diagnosis and therefore bring worse outcomes.

Undetected Iatrogenic Drug-Induced Complications in a Hemodialyzed Anuric Patient: A Case Report and Review of the Literature.

Anuric hemodialyzed end-stage renal disease patients are prone to multiple complications and comorbidities and are therefore often treated with various medications. Adverse drug reactions and risk factors leading to them can be difficult to discern in such polymedicated patients. Most problems regarding low phosphate levels are frequently underdiagnosed in clinical practice and sometimes overlooked in these regularly hyperphosphatemic patients. Hemodialysis vascular accesses are frequently subject to infections and therefore require adapted antibiotic treatments. We report a case of an occult severe multifactorial hypophosphatemia in an anuric hemodialyzed patient with multiple comorbidities who required two hospitalizations for encephalopathy, seizures, and cardiac failure. Retrospective analysis of the medical record revealed several underlying causes of hypophosphatemia, as well as undetected risk factors for adverse drug reactions related to cephalosporins. A global approach to these concerns in routine clinical practice would raise awareness of often disregarded issues related to hypophosphatemia and drug prescription in these patients.

[Negative impact of COVID-19 pandemic on peritonitis rate in peritoneal dialysis patients: Pleading for a continuous educational training]. / Conséquences de la pandémie de COVID-19 sur le taux de péritonites de dialyse péritonéale : plaidoyer pour une formation continue ininterrompue.

BACKGROUND: Peritonitis is a common complication of chronic peritoneal dialysis treatment contributing to both technique failure and/or death. Little is effectively known about the actual benefits of a continuous training program on peritonitis rates. In the present study, we measured the impact of our patients' training protocol on peritonitis rates. We further studied which consequences the COVID-related disruption of our follow-up program had on peritonitis rates. METHODS: We present our yearly peritonitis rates since our patients' training and retraining program was implemented in 2010. We then focused our study on three consecutive years: 2019, 2020 (emergence of COVID-19), and 2021, collecting microbiological data from each peritonitis episode. Statistical analysis were used to corroborate our findings. RESULTS: Since 2010, peritonitis rates declined linearly (R2=0,6556; df=8; P<0.01) until its nadir in 2019 with 4 peritonitis episodes. The majority of infections were then treated in the outpatient Clinic. In 2020, our continuous technique evaluation decreased by 51% and 28 peritonitis episodes occurred, 47% secondary to strict cutaneous bacteria's, and 31% gastro-intestinal, irrespective of patients' experience or peritoneal dialysis modality. The hospitalization rate reached 71%. Having restored our protocol, we decreased peritonitis rates by 50% in 2021. CONCLUSIONS: Risk factors for peritonitis are identifiable and modifiable and require sustained intervention, continuous visual monitoring and training. These interventions significantly reduce peritonitis rates. Any brief interruption to patients' technique evaluation may elevate peritonitis rates significantly.

Iatrogenic Infective Endocarditis in Hemodialysis Patients: A Case Report and Review of the Literature.

Foreign bodies such as implanted cardiac devices are susceptible to infections and may be involved in infective endocarditis. Exposure to pathogens, by frequent use of intravascular accesses for hemodialysis (i.e., catheters or fistulas), combined with high rates of degenerative heart valve diseases in hemodialysis patients, both favor the development of infective endocarditis in this population. The mitral and aortic valves are predominantly implicated in endocardial infections. The involvement of both mitral and tricuspid valves is rare in the general population but can occur in hemodialysis patients with implanted cardiac devices. Infective endocarditis is associated with high morbidity and mortality rates among hemodialysis patients, mostly because of the complications of septic emboli. Prevention, prophylaxis, and early diagnosis of endocarditis can be lifesaving in this fragile population. We report a case of right and left heart methicillin-sensitive Staphylococcus aureus endocarditis with cerebral septic emboli in an elderly hemodialysis patient carrier of an arteriovenous fistula and an ipsilateral nonleadless pacemaker.

Clinical management of a granulomatous tubulointerstitial nephritis associated with a bilateral granulomatous anterior uveitis: A challenging case report and review of the literature.

Acute granulomatous tubulointerstitial nephritis (GTIN) is a rare finding in renal biopsy. Differential diagnosis is facilitated when GTIN is associated with granulomatous bilateral anterior uveitis (GBAU). Nevertheless, differentiation between a rare form of granulomatous tubulointerstitial nephritis and uveitis syndrome (TINU) and sarcoidosis can be challenging. We report a case of biopsy-proven GTIN with concomitant GBAU, leading to a dead-end diagnosis. We discuss workup and propose a diagnostic algorithm based on a literature review. We also report a successful treatment of ophthalmologic and renal relapse using mycophenolate mofetil.

Invasive non-typhoidal Salmonella infection complicated by metastatic infections: Report of three cases.

Non-typhoidal Salmonella (NTS) disease is usually a self-limiting infection presenting with digestive symptoms. However, disseminated presentation with involvement of secondary infectious sites is observed. We report diagnostic specificities and challenges related to the management of three patients with invasive NTS (iNTS) and secondary infectious locations. Among the seven patients (age range 46 - 83 years), four (two with extra-digestive infectious sites) had at least one immune debilitating condition. Two patients were incidentally discovered with iNTS and deceased after developing a septic shock despite antimicrobial treatment. Two individuals recovered under medical treatment without complications. Three other patients presented with secondary infectious sites. Case 1 suffered from urinary tract infection and dorsolumbar spondylodiscitis that responded well to antimicrobials and surgery. Abdominal prosthetic aortic aneurysm was diagnosed in case 2 and medical treatment only was applied. After four years of follow-up, he remains under antimicrobial treatment. Case 3 presented with conjoint thoracic aortic aneurysm and cutaneous abscesses managed with antimicrobials and surgery. Atherosclerosis and previous vascular intervention were the predisposing events for vascular involvement. iNTS is a serious disease carrying a high risk of mortality or secondary locations. Secondary locations can be managed by long duration antimicrobial therapy combined with surgery. Spine and aortitis are the most frequent secondary locations. Multi-drug resistant NTS represent an additional risk of mortality. Public health measures should be implemented to limit the spread of NTS to humans and the emergence of drug resistance.

Hemolysis in a Patient during Hemodialysis.

We report a case of hemolysis during a hemodialysis (HD) session in a 71-year-old man. His end-stage kidney disease is secondary to light-chain amyloidosis with renal involvement. Despite immunosuppressive treatment, his renal function continued to decline, and dialysis had to be initiated. Peritoneal dialysis (PD) was started but that had to be converted to HD because of pleural effusion due to PD fluid leakage. On the event day, the patient presented a respiratory distress 2 h after the initiation of HD. He developed a sudden onset of dyspnea with hypoxemia, associated with abdominal pain, nausea, and vomiting. He also presented chest pain with arterial hypertension. The pre-pump arterial and post-pump pressures were, respectively, 40 and 100 mm Hg, with no machine alarm. The blood color in the circuit changed and became darker, so HD was stopped immediately without blood restitution, and then a blood workup was obtained, and the patient was treated with oxygen therapy, IV methylprednisolone 40 mg, and IV furosemide 100 mg. Tubing checkup performed after the incident showed a kinked arterial tube which led to the suspicion of acute hemolysis. Blood transfusion was therefore urgently ordered, and the patient was immediately transferred to the intensive care unit (ICU). Artificial ventilation was required for 4 days, with initial massive blood transfusion. A 24-h treatment with extracorporeal cytokine adsorber CytoSorb® was also performed, followed by the regular HD sessions thrice weekly. Evolution was favorable, and the patient was discharged from the ICU 18 days later.