Atypical Hemolytic Uremic Syndrome in a Patient With Bothrops asper Envenomation.

Bothrops asper envenomation is common in Colombia and is characterized by local tissue injury and venom-induced consumption coagulopathy (VICC). Rarely, thrombotic microangiopathy is associated with envenomation by this species. The case of a 57-y-old man with B asper bite and envenomation on the left foot is presented. The patient was admitted 8 h after the event and progressively developed edema, hemorrhage at the site of the bite, and hemorrhagic blisters. His coagulation test results (prothrombin and partial thromboplastin times) were prolonged, and his fibrinogen levels were severely reduced. The diagnosis of VICC was made. Administration of Colombian polyvalent viper antivenom controlled the VICC within a few hours. Subsequently, the patient developed severe microangiopathic anemia, thrombocytopenia, and acute kidney injury. A diagnosis of thrombotic microangiopathy was made, and the patient met the criteria for hemolytic uremic syndrome. Management with hemodialysis in addition to therapeutic plasma exchange and replacement with fresh frozen plasma was indicated. The patient's condition resolved 14 d later. To the best of our knowledge, this is the first case of B asper envenomation in which the patient presented with hemolytic uremic syndrome after VICC. A proposal is made regarding the pathogenesis of this chain of events.

Rituximab for Inflammatory Myopathies in a Colombian Cohort.

INTRODUCTION/OBJECTIVES: Rituximab (RTX) is a treatment for refractory inflammatory myopathies, such as dermatomyositis (DM) and polymyositis (PM). This study describes the characteristics of patients receiving RTX for myositis in our institution to evaluate its efficacy. METHOD: We collected demographic data from all patients diagnosed with DM or PM who received RTX between 2011 and 2018. Clinical and serological variables (including creatine phosphokinase [CPK] levels) were analyzed. Remission of disease was defined as no evidence of disease activity (active myositis) for longer than a 6-month continuous period while undergoing myositis therapy or no medication. RESULTS: Eighteen patients who had received first-line immunosuppressants were included. Fifteen (83%) had DM, 2 (11%) had PM, 1 had juvenile dermatomyositis, and 14 (77%) were women. All patients received glucocorticoids. Three patients (16.6%) were treated with RTX as monotherapy, and 15 (83.3%) were treated with RTX combined with other immunosuppressants. On average, there were 2 RTX treatment cycles. Improved muscular weakness was found in 13 cases (72%), and improved serum CPK levels were found in 15 cases (83%). Twelve patients (66%) achieved remission. CONCLUSIONS: Most patients experienced an objective improvement, as reflected in their serum CPK values and degree of muscular weakness. This suggests that RTX could be helpful in treating refractory myositis.

Systemic Sclerosis at an Intensive Care Unit: A Case Series and Literature Review.

INTRODUCTION: Systemic sclerosis (SSc) is a multisystemic autoimmune disease. Few studies have focused on the outcomes of SSC patients who require intensive care unit (ICU) admission, largely due to the absence of protocols for the optimal management of this disease during an ICU stay. OBJECTIVES: This study aimed to describe the outcomes of a series of SSc patients admitted to the ICU at a single center in Cali, Colombia. METHODS: Case series of SSc patients admitted to the ICU were reviewed. The main outcome was ICU mortality. Statistical analysis was performed with measures of central tendency and proportions. RESULTS: All the patients (n = 14) were female and either middle-aged or elderly; 9 (64%) were diagnosed with diffuse cutaneous sclerosis, and the remaining 5 patients with limited cutaneous sclerosis. Some were readmitted; therefore, the total number of ICU admissions was 21. The principal causes of ICU admissions were non-SSc-related causes (n = 15 [71.4%]). The respiratory system was the most involved on ICU admissions. The ICU mortality rate was 43% (n = 6). CONCLUSIONS: The severity of the disease at ICU admission and comorbidity are independently associated with ICU-related mortality. Furthermore, the optimal management of SSc patients includes accurate detection of SSc-associated organ involvement. More studies involving this category of patients are needed to establish the best effective protocols.

Severe cerebral edema related to oral methadone: A case report and literature review.

Introduction: Opioids are widely used for pain management, and increased intracranial pressure (ICP) has been evidenced in some cases. We reported a patient with severe cerebral edema after initiating methadone and its complete resolution upon discontinuing the medication. Additionally, a review of the literature is made. Case report: A 53-year-old woman patient with a history of systemic lupus erythematosus developed mechanic chronic lower back pain, refractory to conventional treatments. She presented improvement with oxycodone. She withdrew this medication due to a lack of supplies in her country (Colombia) and showed withdrawal symptoms. She consulted the emergency department, where oral methadone was started and symptom control was achieved. Three days after admission, she presented intense headaches and emesis. A brain CT scan was performed in which severe cerebral edema was appreciated. Methadone was discontinued, and neurological symptoms quickly disappeared. A follow-up brain CT scan was performed later, finding full resolution of the edema. Conclusion: A case of severe cerebral edema associated with the initiation of oral methadone and its rapid resolution without neurological sequelae after its withdrawal is presented, clinicians must be attentive to this adverse event.

Viperidae snake envenomation from a highly complex hospital in southwestern Colombia.

Background: Snake envenomation is a medical condition with high morbidity and mortality in southwestern Colombia. Objectives: To describe the characteristics of the envenomation caused by Viperidae snakes view in a highly complex hospital in Southwestern Colombia. Methods: A cross-sectional study was carried out. Patients treated for Viperidae snake envenomation from 2001 to 2020 in a Hospital Fundación Valle del Lili, Cali, Colombia, were studied. Results: Twenty-eight patients were included. Envenomation was caused by the genera Bothrops, Bothriechis, Porthidium, and Bothrocophias. The median age was 37.7 (±20.6), and they were predominantly male (19, 68%). Bites occurred on the upper extremities in 16 (57%) patients. Pain (23, 81%) and edema (22, 78%) were the most common clinical symptoms. Thirteen (46%) patients presented coagulopathy. Prolonged prothrombin and activated partial thromboplastin times were common: (22, 78% and 15, 53%, respectively). Twenty (71%) patients were treated with polyvalent antivenom (median dose of 6 (2-15) vials). The median time between the accident and antivenom administration was 9 h (5.5-17). Door-to-needle time was 37.5 (0-62) min. Eighteen (64%) patients were admitted to the intensive care unit. Three (11%) patients had serum sickness. Seven (25%) developed infectious complications, four (14%) had surgery, one (3%) had compartment syndrome, one (3%) underwent amputation of the affected limb, and one (3%) patient died. Conclusions: Local manifestations and coagulopathy were common clinical features. Polyvalent antivenom was an effective treatment for disease control. Significant complications were associated with delays in seeking medical care.

Eosinophil-related diseases during treatment with glucagon-like peptide one receptor (GLP-1 RA): a case report and review of the literature.

Glucagon-like peptide one-receptor agonists (GLP-1 RA) are drugs that differ in their pharmacological composition and homology to human GLP-1 and are used most frequently for the treatment of type 2 diabetes and weight loss. There are isolated reports of eosinophilic adverse reactions associated with GLP-1 RA. We present the case of a 42-year-old female patient who, after starting weekly subcutaneous semaglutide, developed eosinophilic fasciitis with favorable clinical evolution after the discontinuation of semaglutide and the initiation of immunosuppression. A review of the eosinophilic adverse events that have been previously reported with GLP-1 RA is provided.

Improvement of the autoimmune phenomenon after treatment of primary hyperparathyroidism: Possible role of dynamics of parathyroid hormone-1-receptor in B-lymphocytes.

We report a case of 65-year-old male patient with primary hyperparathyroidism (PHPT) who was admitted to the hospital for autoimmune manifestations (including autoimmune hepatitis and autoantibody development) and exhibited subsequent clinical and paraclinical improvement after parathyroidectomy. By flow cytometry, the expression of PTH receptor 1 (PTHR1) on B lymphocytes of peripheral blood was documented to be higher than that in healthy controls. After parathyroidectomy, autoimmune manifestations improved, while PTH1R expression on B-lymphocytes increased. The possible role of the dynamics of B-lymphocyte PTHR1 in the development of this autoimmune phenomenon is discussed.

Association of Antiribosomal P Antibody with Neurological and Systemic Manifestations in Patients with Systemic Lupus Erythematosus in Southwestern Colombia.

BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by heterogeneous pathogenesis, various clinical manifestations, and a broad spectrum of autoantibodies which recognize different cellular components. This study examines the clinical significance and serological associations of serum antiribosomal P antibodies (anti-P) derived from SLE patients in a population from southwestern Colombia. METHODS: We performed a cross-sectional study of 66 SLE patients. Serum antiribosomal P0 autoantibodies were detected by line immunoassay using the ANA-LIA MAXX kit and processed on the automated HumaBlot 44FA system (Human Diagnostics, Germany). RESULTS: Of the 66 SLE patients included in the study, 17 patients (25.76%) showed anti-P positivity by line immunoassay (IA), 47 (71.21%) were negative, and results from 2 patients were indeterminate. We did not find an association with neuropsychiatric SLE (NPSLE), renal, or hepatic disorders (P > 0.05). Laboratory findings indicated that anti-P positivity was significantly associated to anti-Smith (P = 0.001), anti-Ro60/SSA (P = 0.046), and anti-dsDNA antibodies (P = 0.034), the latter being true only when performed using indirect immunofluorescence (IIF). CONCLUSION: The anti-P antibodies are not associated with clinical manifestations such as NPSLE, lupus nephritis, or hepatic involvement in the southwest Colombian SLE population. Moreover, we confirmed previously reported association between anti-P antibody, serum anti-dsDNA, and anti-Smith.

Human endogenous retroviruses (HERV) and non-HERV viruses incorporated into the human genome and their role in the development of autoimmune diseases.

Genomic incorporation of viruses as human endogenous retroviruses (HERVs) are components of our genome that possibly originated by incorporating ancestral of exogenous viruses. Their roles in the evolution of the human genome, gene expression, and the pathogenesis of autoimmune diseases (ADs) and neoplastic phenomena are the subject of intense research. This review analyzes the evolutionary and virological aspects of HERVs and other viruses that incorporate their genome into the human genome and have known role in the genesis of ADs. These insights are helpful to understand further the possible role in autoimmunity genesis of HERVs, other ancestral viruses no HERVs and modern viruses with the ability to incorporate into the human genome or interact with HERVs.

Validation and adaptation to Spanish of the EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI).

INTRODUCTION AND OBJECTIVES: Sjögren's Syndrome (SS) is an autoimmune disease with a wide spectrum of clinical manifestations that can have an important impact on the patient's quality of life. To make an objective evaluation of the components of the disease, clinimetric tools such as the ESSPRI have been designed. The objective of this study is to adapt this scale to the Spanish language. MATERIALS AND METHODS: This is a cross-sectional study to validate clinimetric scales, carried out in Cali, Colombia. A translation of the original English version of ESSPRI into Spanish was made and applied to patients with SS, as well as PROFAD and ESSDAI, as an activity marker. The reliability index of the questionnaire in Spanish with Cronbach's alpha coefficient and Spearman's correlation coefficient were calculated to compare the scales. Demographic, clinical and laboratory characteristics were also evaluated. RESULTS: ESSPRI, PROFAD and ESSDAI were applied to 42 patients with SS, 97.62% were women. The average result of the ESSPRI was 5.8 (± 4.6), with a reliability coefficient of .8034 and a correlation with PROFAD of .5800 (p=.0001), and of -.0848 (p=.593) with ESSDAI. DISCUSSION AND CONCLUSIONS: Reliability with the applied version of ESSPRI in Spanish was adequate. A discrepancy was found between this scale and ESSDAI, which highlights the importance of applying both tools to ensure objective monitoring of disease control and its impact on the quality of life of patients with SS.

Therapeutic Plasma Exchange as a Treatment for Autoimmune Neurological Disease.

INTRODUCTION: Therapeutic plasma exchange (TPE) is commonly used as treatment of certain autoimmune neurological diseases (ANDs), and its main objective is the removal of pathogenic autoantibodies. Our aim was to describe the clinical profile and the experience with the usage of TPE in patients with ANDs at our institution. METHODS: This is an observational retrospective study, including medical records of patients with diagnosis of ANDs who received TPE, between 2011 and 2018. Characteristics of TPE, such as number of cycles, type of replacement solution, and adverse effects, were evaluated. The modified Rankin Scale (mRS) was applied to measure the clinical response after the therapy. RESULTS: 187 patients were included with the following diagnoses: myasthenia gravis (MG), n = 70 (37%); Guillain-Barré syndrome (GBS), n = 53 (28.3%), neuromyelitis optica spectrum disorders (NMOSD), n = 35 (18.7%); chronic inflammatory demyelinating polyneuropathy (CIDP), n = 23 (12.2%); and autoimmune encephalitis (AE), n = 6 (3.2%). The most used types of replacement solution were albumin (n = 131, 70%) and succinylated gelatin (n = 45, 24%). All patients received a median of five cycles (IQR 5-5). Hypotension and hydroelectrolytic disorders were the main complications. After TPE, 99 patients (52.9%) showed improvement in the mRS scores and a statistical significance (p < 0.05) was seen between the admission score and after TPE for every diagnosis except for CIDP. CONCLUSION: TPE has an adequate safety profile, and improvement in functionality in treated patients reflects its effectiveness.

Mycobacterium malmoense: an unusual pathogen causing endocarditis, a case report and literature review.

Non-tuberculous mycobacterias (NTM) are important pathogens responsible for a broad spectrum of diseases in humans. Although exposure is widespread since they are distributed in the environment, the development of the disease is rare. It will depend on the specific species, their virulence (only 50 have been found to cause disease), and the host's immune response. M Mycobacterium Malmoense is a NTM first reported in 1977 at Malmö, Sweden, based on four cases of lung infections. After these, other infections have been reported mainly involving the respiratory tract. Extrapulmonary infections are limited to cervical adenitis, and rarely to tenosynovitis and disseminated disease. We are hence reporting, to our knowledge, the first case of M. malmoense as the cause of bacterial endocarditis in the world.

Prevalence of anti-DFS70 autoantibodies in a Latin American cohort of patients with systemic lupus erythematosus and without autoimmune diseases.

INTRODUCTION/OBJECTIVES: Anti-dense fine speckled 70 (DFS70) autoantibodies were reported to be more prevalent in healthy individuals than those with autoimmune diseases such as systemic lupus erythematosus (SLE). We determined anti-DFS70 autoantibody prevalence in a Latin American cohort of patients with SLE and healthy individuals. METHODS: This study included 127 individuals with anti-nuclear antibodies (ANAs; > 1:160) suggesting the presence of anti-DFS70, including 64 patients with SLE and 63 healthy controls. The anti-DFS70 autoantibodies were determined by immunoadsorption using NOVA Lite® HEp-2 Select kit with DAPI. Negative fluorescence after adsorption with the DFS70 antigen indicated anti-DFS70 autoantibody positivity. RESULTS: The presence of anti-DFS70 autoantibodies was confirmed by indirect immunofluorescence in 21 (33.3%) healthy controls and 8 (12.5%) patients with SLE (p = 0.005). Among the anti-DFS70-positive patients with SLE, the most frequent compromise was renal involvement in six cases (75%), 4 patients (37.5%) were positive for anti-Sm, which was the most frequently associated antibody, and one patient (12.5%) was positive for anti-DNA. CONCLUSIONS: Anti-DFS70 autoantibodies might be considered a biomarker to differentiate patients with SLE from ANA-positive individuals without autoimmune diseases. KEY POINTS: • In a Latin American cohort, the anti-DFS70 was higher in individuals without autoimmune diseases compared with that in patients with SLE.• The anti-DFS70 might have utility as a biomarker of exclusion in patients with non-specific clinical signs of AARDs.