Performance of the 2023 American College of Rheumatology/EULAR Classification Criteria for Antiphospholipid Syndrome in a Mexican Cohort.

OBJECTIVE: The objective is to assess the performance of the 2023 American College of Rheumatology/EULAR classification criteria (2023 AECC) for antiphospholipid syndrome (APS) in a Mexican cohort. METHODS: We enrolled patients with primary APS (PAPS) and secondary APS (SAPS) and a control group of nonautoimmune thrombophilia. We evaluated the fulfillment of the 2023 AECC and the 2006 revised Sapporo classification criteria (2006 RSCC) and their performance against the clinical diagnosis as the gold standard. The baseline Global APS Score (GAPSS) and the Damage Index for APS (DIAPS) at last follow-up were calculated. RESULTS: We included 85 patients with PAPS, 54 with SAPS, and 50 with thrombophilia. According to the 2023 AECC criteria, 69 patients (81.2%) with PAPS, 28 patients (51.9%) with SAPS, and none of the patients with thrombophilia met the criteria. When comparing true positive (TP) (n = 69) versus false negative (n = 16) cases within the PAPS group, TP cases exhibited a higher frequency of thrombotic manifestations and IgM anti-cardiolipin and IgG anti-ß2-glycoprotein-I positivity. For PAPS, there was a correlation between the 2023 AECC score and both GAPSS (rho = 0.621, P < 0.0001) and DIAPS scores (rho = 0.433, P < 0.0001). When comparing the 2023 AECC with the 2006 RSCC, a lower sensitivity (81.2% vs 88.2%) but a higher specificity (100.0% vs 92.0%) was observed for PAPS. Similar findings were observed in SAPS. CONCLUSION: In both PAPS and SAPS, the 2023 AECC have higher specificity than the 2006 RSCC. The main feature of patients with PAPS according to the 2023 AECC was thrombosis. These criteria might identify patients at higher risk of thrombosis and damage accrual.

Description of therapeutic strategies in severe systemic lupus erythematosus-associated immune thrombocytopenia: a retrospective cohort study of response and relapse.

OBJECTIVES: To describe the response and relapse of severe thrombocytopenia in patients with systemic lupus erythematosus (SLE) with different treatments. METHOD: We performed a retrospective cohort study, which included SLE patients who were hospitalized for thrombocytopenia of less than 30,000/µL platelets, from January 2012 to December 2021. Demographic and clinical information was obtained from clinical records. Kaplan-Meier and logrank test were performed. RESULTS: Forty-seven patients, mostly women (83%) with a median age of 31 years, were included in the study. Eight patients (17%) relapsed within a median period of 35.7 weeks. Initial acute treatment with prednisone at 1 mg/kg/day was as effective as glucocorticoid pulses. However, induction treatment with cyclophosphamide (CYC) had the lowest remission rate (43%, p = 0.034). There was no significant difference in relapse-free survival (RFS) among the acute glucocorticoid treatments. CYC induction was associated with lower RFS compared to rituximab (RTX) (CYC 43.6 weeks vs. RTX 51.8 weeks, p = 0.040) or azathioprine (AZA) (CYC 43.6 weeks vs. AZA 51.2 weeks, p = 0.024). Administration of antimalarials was associated with longer RFS (51.6 weeks vs. 45.0 weeks, p = 0.021). Factors such as antiphospholipid syndrome, IgG anti-ß2 glycoprotein I positivity, renal and additional hematologic SLE activity during follow-up significantly reduced RFS. CONCLUSIONS: Despite similar response of acute glucocorticoid regimens, induction therapy with AZA or RTX resulted in a longer RFS compared to CYC. Adding an antimalarial also improved RFS. Our study provides evidence that may help develop better treatment strategies for severe thrombocytopenia in SLE patients. Key Points • Induction therapy with azathioprine or rituximab provided longer relapse-free survival in SLE thrombocytopenia compared with cyclophosphamide. • Antimalarial administration was associated with longer relapse-free survival in SLE thrombocytopenia. • Antiphospholipid syndrome, IgG anti-ß2 glycoprotein I positivity, as well as renal and additional hematologic SLE activity during follow-up, decreased relapse-free survival.

Disability and cognitive impairment are interdependent in primary antiphospholipid syndrome.

BACKGROUND: Cognitive impairment (CI) occurs at a high frequency in primary antiphospholipid syndrome (PAPS). Its psychosocial-related factors are of interest. OBJECTIVE: We aimed to determine disability and perceived stress and their correlation with CI in PAPS. METHODS: First study phase: a longitudinal study including patients with PAPS and paired controls for cardiovascular risk factors, age, and sex, determining CI with Montreal Cognitive Assessment (MoCA) and then repeating the measurement 1 year later. Second study phase: a cross-sectional analytical study by quantification of disability with the World Health Organization Disability Assessment Schedule (WHODAS 2.0) and perceived stress with the Perceived Stress Scale (PSS-14). Descriptive statistics and Spearman correlation coefficient were used. RESULTS: Sixty-three patients with PAPS and 60 controls were studied. In PAPS, age (range, 48.0 ± 13.5 years), thrombotic artery events (TAE) (44.4%), and stroke/TIA (42.8%) were found. Disability was documented in the majority of WHODAS 2.0 domains and the total score for this was higher in participation and mobility, the stress level was normal, and 65.1% had CI. PAPS exhibited greater deterioration in the WHODAS 2.0 total score (p .017) and the MoCA test (p < .0001). Personal domains and the total WHODAS 2.0 score correlated inversely with MoCA. Life activities (rho = -0.419) and self-care (rho = -0.407) were those that correlated to the greatest degree. Stroke conferred risk for CI. CONCLUSIONS: Disability in PAPS and CI are interdependent. New treatment options and neurocognitive stimulation strategies are necessary to maintain functionality and prevent further cognitive dysfunction in PAPS patients.

Risk Factors and Outcomes for COVID-19 in Autoimmune Inflammatory Diseases during the SARS-CoV-2 Pandemic: A Comparative Study.

BACKGROUND: Patients with autoimmune disease (AID) and coronavirus disease 2019 (COVID-19) could have higher mortality due to the co-morbidity and the use of immunosuppressive therapy. OBJECTIVES: To analyze the risk factors and outcomes of patients with AID and COVID-19 versus a control group. METHODS: A prospective cohort study included patients with and without AID and COVID-19. Patients were paired by age and sex. Clinical, biochemical, immunological treatments, and outcomes (days of hospital stay, invasive mechanical ventilation [IMV], oxygen at discharge, and death) were collected. RESULTS: We included 226 COVID-19 patients: 113 with AID (51.15 ± 14.3 years) and 113 controls (53.45 ± 13.3 years). The most frequent AIDs were Rheumatoid arthritis (26.5%), systemic lupus erythematosus (21%), and systemic sclerosis (14%). AID patients had lower lactate dehydrogenas, C-reactive protein, fibrinogen, IMV (P = 0.027), and oxygen levels at discharge (P ≤ 0.0001) and lower death rates (P ≤ 0.0001). Oxygen saturation (SaO2) ≤ 88% at hospitalization provided risk for IMV (RR [relative risk] 3.83, 95% confidence interval [95%CI] 1.1-13.6, P = 0.038). Higher creatinine and LDH levels were associated with death in the AID group. SaO2 ≤ 88% and CO-RADS ≥ 4 were risk factors for in-hospital mortality (RR 4.90, 95%CI 1.8-13.0, P = 0.001 and RR 7.60, 95%CI 1.4-39.7, P = 0.016, respectively). Anticoagulant therapy was protective (RR 0.36, 95%CI 0.1-0.9, P = 0.041). CONCLUSIONS: Patients with AID had better outcomes with COVID-19 than controls. Anticoagulation was associated with a lower death in patients with AID.

Primary meningococcal septic arthritis associated with joint calcium oxalate crystals: A case report and review of the literature.

Primary meningococcal septic arthritis (PMSA) is an extremely rare local infection by Neisseria meningitidis in the absence of meningitis or meningococcaemia syndrome. A 30-year-old healthy, immunocompetent man presented with arthralgia, fever, chest rash, and significant swelling of the right knee. On admission, a disseminated maculopapular and purpuric rash, oligoarthritis, neutrophilia, and elevated acute phase reactants were documented. Following arthrocentesis of the right knee, isolation of N. meningitidis and the presence of calcium oxalate crystals in the synovial fluid were reported. The diagnosis of PMSA was made. Histological analysis of the skin lesion showed leucocytoclastic vasculitis. He was treated with intravenous ceftriaxone plus open surgical drainage and ambulatory cefixime with adequate response. After 1 month, he presented resolution of the pathological process. We performed an extensive review of the literature, finding that the key elements supporting the diagnosis of PMSA are prodromal upper respiratory tract symptoms and skin involvement prior to or synchronous with the arthritis. Also, the most frequently involved joint is the knee. This report is the first case of a patient presenting with PMSA associated with calcium oxalate crystals in the synovial fluid. Herein, we discuss the most frequent clinical manifestations, the unusual histological features, the recommended treatment, and the reported prognosis of this rare entity.

Transformation of a myelodysplastic syndrome to acute myeloid leukemia and concurrent necrotizing sweet syndrome.

The Sweet's syndrome, is an inflammatory skin disorder characterized by extensive infiltration of neutrophils in the dermis with extension to the subcutis, known as acute febrile neutrophilic dermatosis. It may occur as a paraneoplastic syndrome. To our knowledge, there are currently few reports about transformation of a myelodysplastic syndrome to acute myeloid leukemia and concurrent necrotizing Sweet syndrome in the literature. Herein we describe an unusual case in a young patient with these characteristics that evolved to a fatal outcome.

Kikuchi-Fujimoto Disease: Report of a Case with Progression to Lupus Nephritis.

BACKGROUND Kikuchi-Fujimoto disease (KFD) is an enigmatic disease, with a distinctive histopathology and a benign and self-limited course. It is more frequent in young Asian women. Autoimmune diseases are identified as one of its triggers; primarily SLE, which may precede, be concomitant with, or develop after the diagnosis of KFD. Patients with KFD should receive periodic follow-up for several years to detect possible evolution of SLE. The main feature of KFD is lymphadenopathy, and cervical lymph nodes are involved in 50% to 98% of cases. Other symptoms such as fever, fatigue, weight loss, and arthralgias are also reported. Differential diagnosis between KFD and SLE is a challenge. When KFD and SLE coexist, a lymph node biopsy may be diagnostic. Treatment should be symptomatic with analgesics and anti-inflammatories, with complete resolution in 3 to 4 months. Corticosteroids and immunosuppressive therapy are justified only in cases concomitant with SLE. CASE REPORT We report a case of KFD in a 28-year-old woman who was initially negative for anti-nuclear antibodies (ANA) and anti-double-stranded deoxyribonucleic acid antibodies (anti-dsDNA), but who became antibody-positive and presented with lupus nephritis 2 months later. CONCLUSIONS We present a case of a patient with KFD who developed SLE 2 months later; highlighting the importance of recognizing its association and its possible progression to monitor for future development of SLE and provide timely treatment to avoid complications. We also compared the clinical, laboratory, and histological similarities between the 2 entities.

Damage index for antiphospholipid syndrome during long term follow-up: Correlation between organ damage accrual and quality of life.

BACKGROUND: Consequences of organ damage in primary antiphospholipid syndrome (PAPS) are diverse, our aim was to determine organ damage over time and the correlation of organ damage accrual with health-related quality of life (HRQoL) in PAPS. METHODS: First phase: retrospective cohort applying Damage Index for Antiphospholipid Syndrome (DIAPS) at 1, 5, 10, 20 years, or longer since diagnosis. Second phase: cross-sectional study, assessing HRQoL by the Medical Outcomes Study Short Form 36 (SF-36), and organ damage accrual. Descriptive statistics and Spearman correlation coefficient were used. RESULTS: Sixty-seven patients were included, mean follow-up:15 years. Deep vein thrombosis prevailed (71.6%), pulmonary embolism (35.8%) and stroke (32.8%). Organ damage was found in 98.5%, with a cumulative DIAPS value of 3, with greater involvement in the neuropsychiatric and peripheral vascular domains. Regarding HRQoL, deterioration in the physical component summary (PCS) was found in 89.6%. Organ damage accrual correlated inversely and significantly with all the SF-36 domains, mainly with the total score and PCS. Body pain and PCS correlated the most (rho = -0.503, rho = -0.475). CONCLUSIONS: Organ damage accrual impaired HRQoL in PAPS. Secondary thromboprophylxis through adequate systemic management and control of cardiovascular risk factors are necessary to prevent further impairment.