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Common variable immunodeficiency (CVID) is a primary disorder characterized by impaired B cell differentiation and defective immunoglobulin production. This condition often presents with a wide range of clinical manifestations, including increased frequency and severity of infections, autoimmune diseases, and inflammatory disorders, which can lead to delays in diagnosis. Granulomatous involvement of the brain is an extremely rare but severe manifestation of CVID. We present a case of a woman in her 30s with a history of Evans syndrome and lymphocytic alveolitis who was admitted with persistent headache without neurological symptoms. Imaging revealed multiple infiltrative brain lesions. Despite the absence of recurrent infections, the patient's history of autoimmune manifestations and immunoglobulin deficiencies led to the diagnosis of CVID without the need for a brain biopsy. Treatment with intravenous immunoglobulin and immunosuppressive therapy resulted in significant clinical improvement and resolution of brain lesions. This case highlights the importance of considering CVID in patients with autoimmune manifestations and the effectiveness of prompt immunoglobulin replacement and immunosuppression in managing severe presentations of this condition.
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Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by arterial, venous, or microvascular thrombosis, pregnancy morbidity, or non-thrombotic manifestations in patients with persistent antiphospholipid antibodies (aPL). Catastrophic APS is a rare and severe form of APS that is defined by the presence of multiple vascular occlusive events. When a patent foramen ovale (PFO) is present, paradoxical embolization can occur, simultaneously leading to arterial and venous thrombosis. We present a complex clinical case of a patient who presented with multiple arterial and venous thrombotic events with positive aPL. The suspicion of catastrophic APS was removed when a PFO was found in a transesophageal echocardiogram, justifying paradoxical embolization. This emphasizes the importance of searching for PFO in patients with APS presenting with simultaneous venous and arterial thrombosis for management and prognosis purposes.
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BACKGROUND: Patients with heart failure (HF) often have multiple cardiovascular risk factors (CVRF) and comorbidities (CMB). We evaluated the impact of additive CMB and CVRF in HF prognosis. METHODS: We retrospectively analyzed ambulatory patients with systolic dysfunction between January 2012 and May 2018. Follow-up: until January 2021. Endpoint: all-cause death. CVRF analyzed: Arterial hypertension, Diabetes mellitus and smoking. CMB evaluated: coronary artery disease, non-coronary atherosclerotic disease, respiratory disease, dementia, anemia, chronic kidney disease, inflammatory/autoimmune disease, active cancer and atrial fibrillation. Classification according to the number of CVRF and/or CMB: < 2 and ≥ 2. The independent prognostic impact of CVRF/CMB burden was assessed with multivariate Cox-regression. RESULTS: Most patients had ≥ 2 CMB (67.9%). Regarding CVRF, 14.9% presented none, 40.2% had one and 32.1% had two. During a median 49-month follow-up, 419 (49.1%) patients died. Mortality was higher among patients with ≥2 CVRF (56.1 vs 43.4% in those with <2) and in those with ≥2 CMB (57.7 vs 31.0%). While patients with one CMB had similar mortality than those with none. Patients with ≥2 CMB had higher long-term mortality risk: HR=2.47 (95% CI: 1.95-3.14). In patients with ≥2CVRF: HR of dying = 1.39 (1.14- 1.70). When taken together there was a clear survival disadvantage for patients with ≥ 2 CVRF/CMB - adjusted HR: 2.20 (1.45-3.34). CONCLUSION: The presence of only 2 CVRF/CMB more than doubles the patients´ risk of dying. CVRF and CMB should be assessed as part of routine patient management.
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INTRODUCTION: The prognostic implications of using benzodiazepines (BZD) in heart failure (HF) patients are still unknown. OBJECTIVES: This study aimed to assess the association of BZD use with allcause death in ambulatory, chronic HF patients. PATIENTS AND METHODS: We investigated a retrospective cohort of ambulatory HF patients with left ventricular systolic dysfunction (LVSD). The patients were followed up from their first medical appointment until January 2021 and allcause mortality was the primary end point. The Cox regression analysis was used to assess the association between BZD use and allcause mortality. Subgroup analyses were performed considering age, sex, body mass index (BMI), respiratory disease, chronic kidney disease (CKD), and New York Heart Association (NYHA) class. Multivariable models were built to account for confounders. RESULTS: We studied 854 patients (69% men), of mean (SD) age 71 (13) years, of whom 51% had severe LSVD, and 242 (28.3%) regularly used BZD. During a median followup of 46 months, 443 patients (51.9%) died. BZD use predicted no crude survival disadvantage in the entire investigated group and in the subgroup analysis according to sex, respiratory disease, BMI, and NYHA class. BZD use was not mortalityassociated among patients aged 75 years and younger. However, in those older than 75 years the hazard ratio (HR) of allcause death was 1.3 (95% CI, 0.99-1.69; P = 0.06). BZD use seemed safe in the patients without CKD, but in those with CKD it was associated with worse survival (HR, 1.33; 95% CI, 1.02-1.73). In a multivariableadjusted analysis, the use of BZD was independently associated with increased death risk (HR, 1.36; 95% CI, 1.06-1.75). CONCLUSIONS: The patients medicated with BZD presented a 36% higher risk of dying. BZD should probably be used with caution, particularly in older HF patients and in those with CKD.