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.

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.

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.

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.

Key Points: A simplified lung ultrasound (LUS)–guided management approach safely reduced pulmonary congestion in patients undergoing hemodialysis. LUS may be performed by nurses and paramedics, making it even easier to integrate it into routine practice. LUS may contribute to better BP control in patients undergoing hemodialysis. Background: Pulmonary congestion (PC) is frequent in hemodialysis patients and is associated with deleterious prognosis. Lung ultrasound (LUS) accurately quantifies PC; however, many methods exist to evaluate PC using LUS. Here, we validate a simplified LUS-guided protocol for managing PC in patients undergoing hemodialysis and explore its impact on BP control. Methods: We enrolled 100 hemodialysis patients from six dialysis units in three countries in a multicenter randomized controlled trial. All patients had LUS after their mid-week session on days 1, 15, 30, 45, and 60. LUS was performed using the eight-zone method to obtain a global B-line score (BLS). Doctors and nurses performed LUS at the bedside. Dry weight was adjusted according to the standard of care in the control group, while it was reduced by 500 g if BLS was above five on day 1 and by 500 g further on day 15 if BLS was still above five in the active group. BP home monitoring was obtained weekly. Results: The mean BLS on day 1 (13±9) decreased to 8±5 on day 60 ( P < 0.001) in the active group. By contrast, the control group showed no significant changes in BLS on day 60 compared with baseline. Home blood pressure monitoring on day 60 was similar to the baseline in both groups. Applying this protocol did not increase the intradialytic hypotension frequency. Conclusions: This simplified LUS-guided protocol for managing PC was effective and safe in hemodialysis patients and could be considered for inclusion in standard care practices. Nurses and advanced caregivers may perform LUS in hemodialysis units. LUS-guided management may have a positive impact on BP control. Clinical Trial registry name and registration number: ClinicalTrials.gov, NCT06296160.

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.