Reporte de caso: vasculitis leucocitoclastica como forma de presentación de hepatitis b crónica / Case report: leukocytoclastic vasculitis as presenting manifestation of chronic hepatitis b virus infection

La vasculitis leucocitoclástica, también denominada angeitis cutánea leucocitoclástica, es la forma más común de vasculitis. Si bien la mayoría de los casos son idiopáticos, entre los agentes etiológicos que podemos nombrar se encuentran los agentes infecciosos, las enfermedades del tejido conectivos, las reacciones de hipersensibilidad a medicamentos y las neoplasias solidas o hematológicas. Si bien los procesos infecciosos son una causa conocida de vasculitis leucocitoclástica, la infección por virus de Virus de hepatitis B (VHB) es muy infrecuente. Presentamos una mujer de 47 años, sin antecedentes patológicos previos, que consultó por artralgias en rodillas y tobillos, mialgias en gemelos y rash purpúrico con leve prurito en ambos miembros inferiores, de un mes de evolución. La biopsia cutánea de las lesiones de miembros inferiores fue compatible con vasculitis leucocitoclástica. La serología de hepatitis B fue positiva por lo que inició tratamiento antiviral con Tenofovir y Prednisona con buena evolución de sus lesiones cutáneas

Leukocytoclastic vasculitis, also called leukocytoclastic cutaneous angiitis, is the most common form of vasculitis. Although most cases are idiopathic, etiologic agents include infectious agents, connective tissue diseases, drug hypersensitivity reactions, and solid or hematologic malignancies. Although infectious processes are a known cause of leukocytoclastic vasculitis, hepatitis B virus (HBV) infection is very rare. We present a 47-year-old woman, with no previous pathologic history, who consulted for arthralgias in the knees and ankles, myalgia's and purpuric rash with mild pruritus in both lower limbs, of one month evolution. Skin biopsy of lower extremity lesions was compatible with leukocytoclastic vasculitis. Hepatitis B serology was positive, so she started antiviral treatment with tenofovir and prednisone with good evolution of her skin lesions

Efficacy of Rituximab for Refractory Pyoderma Gangrenosum-Like Ulcers in Granulomatosis With Polyangiitis Associated to Antiphospholipid Antibodies.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis that most often presents with painful ulcerations of violaceous borders in lower limbs and/or trunk. PG treatment varies according to the severity of the lesion and may either respond to local therapies or require immunosuppressive agents. In this article, we present the case of an antiphospholipid antibody-positive 59-year-old female patient diagnosed with granulomatosis with polyangiitis who developed severe PG-like skin involvement that was responsive to rituximab therapy.

Sudden Sensorineural Hearing Loss in Systemic Lupus Erythematosus and Antiphospholipid Syndrome: A Clinical Review.

BACKGROUND: Sudden sensorineural hearing loss (SSNHL) is defined as a sudden loss of hearing, usually unilateral, of more than 30 dB in 3 contiguous frequencies of the tonal audiometry. SSNHL estimates an incidence ranging from 5 to 20 per 100.000 people per year. In approximately 75% of cases, a cause cannot be identified. However, it could be a clinical manifestation of Systemic lupus erythematosus (SLE) and Antiphospholipid Syndrome (APS). OBJECTIVE: This review will focus on the clinical presentation, diagnosis, and management of the SLE and APS associated SSNHL. METHODS: We searched in PubMed, Scopus, Lilacs, and Cochrane reviewing reports of Sudden sensorineural hearing loss in SLE and/or APS. Articles written in English and Spanish, and were available in full text, were included. RESULTS: In patients with SLE, bilateral involvement was frequent. Antiphospholipid antibodies were positive in the majority of the patients. Corticosteroids were the mainstay of the treatment. The auditory prognosis was poor with total hearing loss recovery reached in only 22% of patients. On the other hand, most of the patients with SSNHL and APS were males and presented associated symptoms such as vertigo, tinnitus and/or headache, 75% had bilateral disease. Lupus anticoagulant and aCL were found in equal proportions, all patients were anticoagulated, and aspirin was associated in 25% of the cases. Complete resolution or improvement of symptoms was observed in 25% of the patients. CONCLUSION: Sudden sensorineural hearing loss, can be a clinical feature of SLE and APS. Treating physicians should be aware of this devastating complication, especially when bilateral involvement occurs.

Borramiento de huellas dactilares debido a fenómeno de Raynaud / Loss of fingerprints due to Raynaud's phenomenon

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Clinical Relevance of Galectin-1 and Galectin-3 in Rheumatoid Arthritis Patients: Differential Regulation and Correlation With Disease Activity.

Galectins, a family of animal lectins, play central roles in immune system regulation, shaping both innate and adaptive responses in physiological and pathological processes. These include rheumatoid arthritis (RA), a chronic multifactorial autoimmune disease characterized by inflammatory responses that affects both articular and extra-articular tissues. Galectins have been reported to play central roles in RA and its experimental animal models. In this perspective article we present new data highlighting the regulated expression of galectin-1 (Gal-1) and galectin-3 (Gal-3) in sera from RA patients under disease-modifying anti-rheumatic drugs (DMARDs) and/or corticoid treatment in the context of a more comprehensive discussion that summarizes the roles of galectins in joint inflammation. We found that Gal-1 levels markedly increase in sera from RA patients and positively correlate with erythrocyte sedimentation rate (ERS) and disease activity score 28 (DAS-28) parameters. On the other hand, Gal-3 is downregulated in RA patients, but positively correlates with health assessment questionnaire parameter (HAQ). Finally, by generating receiver-operator characteristic (ROC) curves, we found that Gal-1 and Gal-3 serum levels constitute good parameters to discriminate patients with RA from healthy individuals. Our findings uncover a differential regulation of Gal-1 and Gal-3 which might contribute to the anti-inflammatory effects elicited by DMARDs and corticoid treatment in RA patients.

Effects of Amerindian Genetic Ancestry on Clinical Variables and Therapy in Patients with Rheumatoid Arthritis.

OBJECTIVE: To define whether Amerindian genetic ancestry correlates with clinical and therapeutic variables in admixed individuals with rheumatoid arthritis (RA) from Latin America. METHODS: Patients with RA (n = 1347) and healthy controls (n = 1012) from Argentina, Mexico, Chile, and Peru were included. Samples were genotyped for the Immunochip v1 using the Illumina platform. Clinical data were obtained through interviews or the clinical history. RESULTS: Percentage of Amerindian ancestry was comparable between cases and controls. Morning stiffness (p < 0.0001, OR 0.05), rheumatoid factor (RF; p < 0.0001, OR 0.22), radiographic changes (p < 0.0001, OR 0.05), and higher number of criteria were associated with lower Amerindian ancestry after Bonferroni correction. Higher Amerindian ancestry correlated only with weight loss (pBonferroni < 0.0001, OR 2.85). Increased Amerindian ancestry correlated with higher doses of azathioprine (p < 0.0001, OR 163.6) and sulfasalazine (p < 0.0001, OR 48.6), and inversely with methotrexate (p = 0.001, OR 0.35), leflunomide (p = 0.001, OR 0.16), and nonsteroidal antiinflammatory drugs (pBonferroni = 0.001, OR 0.37). Only the presence of RF and weight loss were modified after confounders adjustment. CONCLUSION: Amerindian ancestry protects against most major clinical criteria of RA, but regarding the association of RF with increased European ancestry, age, sex, and smoking are modifiers. Ancestry also correlates with the therapeutic profiles.

Antiphospholipid and antioangiogenic activity in females with recurrent miscarriage and antiphospholipid syndrome.

Background Antiphospholipid syndrome is an autoimmune disease characterized by thrombosis, fetal losses and thrombocytopenia associated to antiphospholipid antibodies. They are directed to phospholipids, such as cardiolipins (anticardiolipin) and lupus anticoagulant or to complexes formed by phospholipids and protein cofactors, such as ß2 glycoprotein 1 (a-ß2GP1) and annexin V (a-annexin V). These auto-antibodies may be considered as a family of antibodies involved in thrombotic events and antiphospholipid activity. On the other hand, some proangiogenic factors are involved in the normal development of placental vasculature, such as the vascular endothelial growth factor. Overexpression of vascular endothelial growth factor receptor in its soluble form (sVEGFR-1) has been associated to a higher antiangiogenic activity. Our aim was to analyse the association between anticardiolipin, lupus anticoagulant, a-ß2GP1, a-annexin V and sVEGFR-1 with recurrent miscarriage before week 10 of gestation in females with antiphospholipid syndrome. Methods We studied 24 females (primary or secondary antiphospholipid syndrome), who were divided into two groups: females with recurrent miscarriage before week 10 of gestation (M; n = 12) and females with no history of fetal loss (NM; n = 12). Anticardiolipin, a-ß2GP1, a-annexin V and sVEGF-R1 concentrations were assessed by ELISA, while lupus anticoagulant was assessed by screening and confirmatory tests. Results A significant association was observed between the number of positive biomarkers and the belonging group ( P < 0.05). Besides, a positive result for lupus anticoagulant and a-ß2GP1 was found to be significantly associated to the M group ( P < 0.05). Conclusions Lupus anticoagulant and a-ß2GP1 may be implicated in pregnancies complicated by recurrent miscarriage in females with antiphospholipid syndrome.

Infecciones graves en pacientes con enfermedades autoinmunes tratados con ciclofosfamida / Severe infections in patients with autoimmune diseases treated with cyclophosphamide

Introducción Las infecciones son una de las principales causas de morbimortalidad en los pacientes con enfermedades autoinmunes sistémicas. Los corticoides y los inmunosupresores como la ciclofosfamida (CF) son algunos de los factores que se asocian a esta complicación. El objetivo de este estudio fue determinar el porcentaje de pacientes que presentaron infecciones graves durante el tratamiento con CF y en los 3 meses posteriores. Pacientes y métodos. Se realizó un análisis retrospectivo de 60 pacientes con diferentes enfermedades autoinmunes que recibieron tratamiento con CF. Se valoró el porcentaje de infecciones graves. Resultados. La patología mñas común fue el lupus eritematoso sistémico y la indicación más común fue la glomerulonefritis proliferativa difusa. El 15% de los pacientes presentó un episodio infeccioso. Las infecciones más frecuentes fueron neumonía adquirida en la comunidad en 3 casos y herpes zóster en 2 casos. La dosis acumulada de corticoides fue el único parámetro que se asoció a infecciones 32,8 ± 16,7 vs. 20,1 ± 15,3 p = 0,007. Conclusión. Un seguimiento estricto, la utilización de corticoides en la menor dosis posible y el tratamiento enérgico de los procesos infecciosos permiten un perfil de seguridad aceptable en aquellos pacientes tratados con CF (AU)

Introduction. Infectious diseases are a significant cause of morbidity and mortality in patients with connective tissue diseases. Corticosteroids and immunosuppressive drugs, such as cyclophosphamide (CYC), increases the risk of infections. The objective of this study was to estimate the incidence rates of severe infections in patients who received treatment with CYC. Patients and methods. The records of 60 patients with systemic autoimmune diseases who received treatment with CYC were retrospectively reviewed. We evaluated the rate of severe infections that occurred during CYC therapy and the 3 subsequent months. Results. Systemic lupus erythematosus was the most common disease, and diffuse proliferative glomerulonephritis the most frequent indication. Severe infection occurred in 9 patients (15%). Community acquired pneumonia was the most frequent infection with 3 cases (33%) followed by Herpes Zoster with 2 reports (22%). The cumulative dose of corticosteroid was the only significant risk factor for infection 32.8±16.7 vs. 20.1±15.3 P=.007. Conclusion. The use of lower doses of corticosteroids and an aggressive management of infectious complications, allows for an acceptable safety profile in patients treated with CYC (AU)

Severe infections in patients with autoimmune diseases treated with cyclophosphamide.

INTRODUCTION: Infectious diseases are a significant cause of morbidity and mortality in patients with connective tissue diseases. Corticosteroids and immunosuppressive drugs, such as cyclophosphamide (CYC), increases the risk of infections. The objective of this study was to estimate the incidence rates of severe infections in patients who received treatment with CYC. PATIENTS AND METHODS: The records of 60 patients with systemic autoimmune diseases who received treatment with CYC were retrospectively reviewed. We evaluated the rate of severe infections that occurred during CYC therapy and the 3 subsequent months. RESULTS: Systemic lupus erythematosus was the most common disease, and diffuse proliferative glomerulonephritis the most frequent indication. Severe infection occurred in 9 patients (15%). Community acquired pneumonia was the most frequent infection with 3 cases (33%) followed by Herpes Zoster with 2 reports (22%). The cumulative dose of corticosteroid was the only significant risk factor for infection 32.8±16.7 vs. 20.1±15.3 P=.007. CONCLUSION: The use of lower doses of corticosteroids and an aggressive management of infectious complications, allows for an acceptable safety profile in patients treated with CYC.

Rheumatoid arthritis in Latin Americans enriched for Amerindian ancestry is associated with loci in chromosomes 1, 12, and 13, and the HLA class II region.

OBJECTIVE: To identify susceptibility loci for rheumatoid arthritis (RA) in Latin American individuals with admixed European and Amerindian genetic ancestry. METHODS: Genotyping was performed in 1,475 patients with RA and 1,213 control subjects, using a customized BeadArray containing 196,524 markers covering loci previously associated with various autoimmune diseases. Principal components analysis (EigenSoft package) and Structure software were used to identify outliers and define the population substructure. REAP software was used to define cryptic relatedness and duplicates, and genetic association analyses were conducted using Plink statistical software. RESULTS: A strong genetic association between RA and the major histocompatibility complex region was observed, localized within BTNL2/DRA-DQB1- DQA2 (P = 7.6 × 10(-10) ), with 3 independent effects. We identified an association in the PLCH2-HES5-TNFRSF14-MMEL1 region of chromosome 1 (P = 9.77 × 10(-6) ), which was previously reported in Europeans, Asians, and Native Canadians. We identified one novel putative association in ENOX1 on chromosome 13 (P = 3.24 × 10(-7) ). Previously reported associations were observed in the current study, including PTPN22, SPRED2, STAT4, IRF5, CCL21, and IL2RA, although the significance was relatively moderate. Adjustment for Amerindian ancestry improved the association of a novel locus in chromosome 12 at C12orf30 (NAA25) (P = 3.9 × 10(-6) ). Associations with the HLA region, SPRED2, and PTPN22 improved in individuals positive for anti-cyclic citrullinated peptide antibodies. CONCLUSION: Our data define, for the first time, the contribution of Amerindian ancestry to the genetic architecture of RA in an admixed Latin American population by confirming the role of the HLA region and supporting the association with a locus in chromosome 1. In addition, we provide data for novel putative loci in chromosomes 12 and 13.

Elastofibroma dorsi: revisión de 4 casos / Elastofibroma dorsi: review of 4 cases

El elastofibroma dorsi es un tumor benigno, poco frecuente, de tejido fibroelástico, más común en mujeres luego de la quinta década de vida. Se suele localizar en la región subescapular, pudiendo en ocasiones ser bilateral. Presentamos 4 pacientes, entre 53 y 73 años de edad, con esta patología. Frecuentemente es una lesión asintomática, aunque puede observarse dolor leve con resalto de la escápula al realizar movimientos. Todas nuestras pacientes presentaron dolor y una de ellas además resalto de la escápula. El diagnóstico se basa en los hallazgos clínicos y los estudios por imágenes, especialmente la ecografía, la tomografía computarizada y la resonancia magnética nuclear. La biopsia se reserva para los casos sospechosos que no presentan en los estudios por imágenes el patrón característico. En nuestra serie, la extirpación de la lesión fue necesaria en una de las enfermas, ya que en las demás la clínica y los estudios por imágenes permitieron arribar a un diagnóstico definitivo (AU)

Elastofibroma dorsi is a benign, uncommon fibroelastic tissue condition, more common in women after the fifth decade of life. It is usually located in the subscapular region, and can sometimes be bilateral. We present 4 patients, between 53 and 73 years of age, with this disease. It is often an asymptomatic lesion that can manifest, even at its apex, with mild pain when moving the scapula. All our patients had pain.The diagnosis is based on clinical findings and imaging studies, especially ultrasound, computed tomography, and nuclear magnetic resonance. The biopsy is reserved for patients who have no characteristic signs on imaging. In our series, surgical excision was necessary in one of the patients, and in the others, clinical and imaging studies allowed us to arrive at a definitive diagnosis (AU)

Hiperparatiroidismo, una causa olvidada de dolor musculoesquelético difuso / Hyperparathyroidism, a forgotten cause of musculoskeletal pain

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Impact of genetic ancestry and sociodemographic status on the clinical expression of systemic lupus erythematosus in American Indian-European populations.

OBJECTIVE: American Indian-Europeans, Asians, and African Americans have an excess morbidity from systemic lupus erythematosus (SLE) and a higher prevalence of lupus nephritis than do Caucasians. The aim of this study was to analyze the relationship between genetic ancestry and sociodemographic characteristics and clinical features in a large cohort of American Indian-European SLE patients. METHODS: A total of 2,116 SLE patients of American Indian-European origin and 4,001 SLE patients of European descent for whom we had clinical data were included in the study. Genotyping of 253 continental ancestry-informative markers was performed on the Illumina platform. Structure and Admixture software were used to determine genetic ancestry proportions of each individual. Logistic regression was used to test the association between genetic ancestry and sociodemographic and clinical characteristics. Odds ratios (ORs) were calculated with 95% confidence intervals (95% CIs). RESULTS: The average American Indian genetic ancestry of 2,116 SLE patients was 40.7%. American Indian genetic ancestry conferred increased risks of renal involvement (P < 0.0001, OR 3.50 [95% CI 2.63- 4.63]) and early age at onset (P < 0.0001). American Indian ancestry protected against photosensitivity (P < 0.0001, OR 0.58 [95% CI 0.44-0.76]), oral ulcers (P < 0.0001, OR 0.55 [95% CI 0.42-0.72]), and serositis (P < 0.0001, OR 0.56 [95% CI 0.41-0.75]) after adjustment for age, sex, and age at onset. However, age and sex had stronger effects than genetic ancestry on malar rash, discoid rash, arthritis, and neurologic involvement. CONCLUSION: In general, American Indian genetic ancestry correlates with lower sociodemographic status and increases the risk of developing renal involvement and SLE at an earlier age.

Elastofibroma dorsi: review of 4 cases.

Elastofibroma dorsi is a benign, uncommon fibroelastic tissue condition, more common in women after the fifth decade of life. It is usually located in the subscapular region, and can sometimes be bilateral. We present 4 patients, between 53 and 73 years of age, with this disease. It is often an asymptomatic lesion that can manifest, even at its apex, with mild pain when moving the scapula. All our patients had pain.The diagnosis is based on clinical findings and imaging studies, especially ultrasound, computed tomography, and nuclear magnetic resonance. The biopsy is reserved for patients who have no characteristic signs on imaging. In our series, surgical excision was necessary in one of the patients, and in the others, clinical and imaging studies allowed us to arrive at a definitive diagnosis.

Anticuerpos anti-anexina V y otros marcadores de actividad antifosfolip¿¬dica en mujeres abortadoras recurrentes con enfermedades autoinmunes / Anti-annexi V antibodies and other markers of antiphospholipid activity in women with recurrent miscarriage with autoinmune diseases

Los anticuerpos (Ac) antifosfolip¿¬dicos componen una familia de auto Ac involucrada en eventos tromb¿«ticos que participar¿¬an de la actividad antifosfolip¿¬dica (AAF). La probabilidad de aborto en una paciente con estos Ac es del 91%. Se ha sugerido la existencia de un nuevo cofactor: la anexina V, altamente expresada en el sinciciotrofoblasto placentario, originando Ac que podr¿¬an estar implicados en las p¿ªrdidas fetales recurrentes. Nuestro objetivo fue analizar la asociaci¿«n entre los Ac anti-anexina V y otros indicadores de AAF [anticardiolipina(ACA), anti-ªÂ2 glicoprote¿¬na 1 (a-ªÂ2GP1) o anticoagulante l¿²pico (AL)] en mujeres con enfermedades autoinmunes y repetidas p¿ªrdidas fetales. Se incluyeron 25 mujeres abortadoras recurrentes con lupus eritematoso sist¿ªmico y/o s¿¬ndrome antifosfolip¿¬dico (A) y un grupo control de 33 mujeres con las patolog¿¬as mencionadas anteriormente, no abortadoras (NA). Se determinaron los niveles de anti-anexina V, ACA y de a-ªÂ2GP1 por ELISA. El AL se evidenci¿« con pruebas de screening y confirmatorias. El 96% del grupo A present¿« AAF positiva y el 4% niveles elevados de Ac a-anexina V. El grupo NA mostr¿« AAF en el 70% de los casos y niveles elevados de Ac a-anexina V en un 3%. Se puede concluir que no existe asociaci¿«n entre Ac anti-anexina V y los indicadores de AAF.(AU)