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 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.

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.

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.

Background. Froin’s syndrome (FS) is a rare entity with uncertain prevalence and prognosis, defined by a pathognomonic triad: cerebrospinal fluid (CSF) xanthochromia, elevated protein levels in the CSF, and hypercoagulated CSF, usually obtained through lumbar puncturing below the level of a partial or complete spinal block. Methods. We conducted a comprehensive review of the literature on FS from its first description in 1903 to December 2023, utilizing PubMed and Google Scholar, and included two new cases from our clinical practice. Results. We describe two patients who suffered from Froin’s syndrome secondary to spinal abscesses. According to our review, FS is caused by neoplasia in 33% of cases, non-malignant mechanical causes in 27%, infections in 27%, non-infectious inflammatory processes in 6%, and vascular in 6%. The most prevalent symptoms are paraplegia/paraparesis (64%), back pain (38%), altered mental state and/or confusion (23%), sciatica (17%), headaches (17%), leg sensory defects (17%), and urinary retention (14%), and are thought to be linked with the underlying causes rather than the CSF characteristics. FS holds a poor prognosis: only 22% recuperate fully after treatment, 22% die due to the cause leading to FS, and 14% retain sequelae. Conclusions. Xanthochromia and proteinorachia >500 mg/dL are not specific to any single pathological condition, but indicate defective CSF recirculation and spinal block, causing diffusive and/or inflammatory processes resulting in the hyperproteinosis and coagulation of the CSF. We reviewed the pathophysiology, etiologies, symptoms, outcomes, and workups of Froin’s syndrome according to the existing medical literature.

La péritonite est une complication fréquente du traitement par dialyse péritonéale chronique, contribuant à l’échec de la technique et/ou au décès. Les avantages réels d’un programme de formation continue sur les taux de péritonites sont peu connus. Dans cette étude, nous avons mesuré l’influence du protocole de formation continue de nos patients sur le taux de péritonites. Nous avons également étudié les conséquences de la perturbation de notre protocole liée au Covid sur les taux de péritonites. Nous présentons nos taux annuels de péritonites depuis la mise en place de notre programme de formation continue des patients en 2010. Nous avons ensuite concentré notre étude sur trois années consécutives : 2019, 2020 (émergence de la COVID-19) et 2021, en recueillant les données microbiologiques de chaque épisode de péritonite. Des analyses statistiques ont été utilisées pour corroborer nos résultats. Depuis 2010, le taux de péritonites a diminué linéairement (R2 = 0,6556 ; df = 8 ; p < 0,01) jusqu’à son nadir en 2019 avec 4 épisodes de péritonites. La majorité des infections ont alors été traitées en ambulatoire. En 2020, notre évaluation continue des procédures de dialyse au domicile des patients a diminué de 51 % et 28 péritonites sont survenues, 47 % secondaires à des bactéries cutanées strictes, et 31 % gastro-intestinales, indépendamment de l’expérience des patients ou de la modalité de dialyse péritonéale. Le taux d’hospitalisations a atteint 71 %. Après avoir rétabli notre protocole, nous avons diminué le taux de péritonites de 50 % en 2021. Les facteurs de risque de développer une péritonite sont identifiables et modifiables, et nécessitent des interventions soutenues, une surveillance visuelle ainsi qu’une formation continue. Ces interventions réduisent de manière significative les taux de péritonites. Toute brève interruption de l’évaluation de la technique des patients peut augmenter de manière significative les taux de péritonites.