RESUMEN
ABSTRACT: The Janus kinase (JAK)-signal transducer and activator of transcription (STAT) pathway plays a critical role in the pathogenesis of many immune-mediated inflammatory diseases (IMIDs). Although Janus kinase inhibitors (JAKi) are an effective treatment for several IMIDs, they have come under scrutiny as a class due to a potential risk of venous thromboembolism (VTE) and cardiovascular (CV) events, specifically noted with the oral JAKi, tofacitinib, as reported in the ORAL Surveillance Trial of a high CV risk rheumatoid arthritis population. This trial resulted in a black box warning from the Food and Drug Administration and European Medicines Agency regarding risk of VTE and CV events that was extended across several types of JAKi (including topical ruxolitinib) when treating IMIDs, leading to considerable controversy. Included is an up-to-date review of the current and rapidly evolving literature on CV risk in patients with IMIDs on JAKi therapy, including identification of potential risk factors for future VTE and CV events on JAKi therapy. We suggest a comprehensive, multimodal, and systematic approach for evaluation of CV risk in patients considering taking JAKi and emphasize that cardiologists play an important role in risk stratification and mitigation for patients with high CV risk factors or on long-term JAKi therapies.
Asunto(s)
Fibrilación Atrial , Enfermedades Cardiovasculares , Dermatomiositis , Infarto del Miocardio , Salud Poblacional , Accidente Cerebrovascular , Humanos , Estudios Transversales , Dermatomiositis/epidemiología , Dermatomiositis/complicaciones , Comorbilidad , Infarto del Miocardio/epidemiología , Fibrilación Atrial/complicaciones , Factores de Riesgo , Accidente Cerebrovascular/epidemiología , Enfermedades Cardiovasculares/epidemiología , Enfermedades Cardiovasculares/complicacionesRESUMEN
The pathophysiology of generalized essential telangiectasia is not well understood. Generalized essential telangiectasia is an uncommon disorder in which widespread telangiectasias of unknown cause develop without associated systemic or antecedent dermatologic disease. We report a case of generalized essential telangiectasia in an otherwise healthy 49-year-old man.
Asunto(s)
Dermatosis del Pie/patología , Dermatosis de la Pierna/patología , Telangiectasia/patología , Dermatosis del Pie/diagnóstico , Humanos , Dermatosis de la Pierna/diagnóstico , Masculino , Persona de Mediana Edad , Telangiectasia/diagnósticoRESUMEN
Kaposi sarcoma (KS) is a vascular neoplasm that is one of the most common human immunodeficiency virus (HIV)-related malignancies. We present the case of a 42-year-old man with a new diagnosis of HIV and acquired immune deficiency syndrome (AIDS)-related epidemic KS.
Asunto(s)
Infecciones Oportunistas Relacionadas con el SIDA/patología , Sarcoma de Kaposi/patología , Infecciones Oportunistas Relacionadas con el SIDA/diagnóstico , Infecciones Oportunistas Relacionadas con el SIDA/tratamiento farmacológico , Infecciones Oportunistas Relacionadas con el SIDA/epidemiología , Adulto , Terapia Antirretroviral Altamente Activa , Epidemias , Herpesvirus Humano 8 , Humanos , Masculino , Sarcoma de Kaposi/diagnóstico , Sarcoma de Kaposi/tratamiento farmacológico , Sarcoma de Kaposi/epidemiologíaRESUMEN
PURPOSE OF REVIEW: First recognized in 1974, eosinophilic fasciitis (EF) is a fibrosing disorder of the fascia with characteristic cutaneous and hematologic manifestations. This review discusses recent trends in the diagnosis and treatment of EF. RECENT FINDINGS: Although fascial biopsy has classically been considered the gold standard for making a diagnosis of EF, radiologic imaging, particularly magnetic resonance imaging, has been increasingly used for both diagnosis and monitoring of treatment response. Systemic corticosteroids remain the first-line treatment for EF; however, their often prolonged use in the treatment of EF has prompted a search for adjunctive therapies. Methotrexate has emerged as the leading corticosteroid-sparing agent for EF. Since EF was initially described over 40 years ago, important diagnostic and therapeutic progress has been made. Future efforts should be directed at the pursuit of prospective studies including clinical trials and evidence-based guidelines.
Asunto(s)
Corticoesteroides/uso terapéutico , Eosinofilia/diagnóstico , Eosinofilia/tratamiento farmacológico , Fascitis/diagnóstico , Fascitis/tratamiento farmacológico , Metotrexato/uso terapéutico , Eosinofilia/diagnóstico por imagen , Fascitis/diagnóstico por imagen , Humanos , Imagen por Resonancia Magnética , Resultado del TratamientoRESUMEN
Chronic arsenic exposure is known to inducepunctate keratoses with an increased risk ofprimary squamous-cell carcinoma. Drinking wateris currently the major source of arsenic exposureworldwide and is considered one of the mostsubstantial environmental carcinogens. We describethe case of a 61-year-old Hungarian woman withscattered, acral, hyperkeratotic papules and a historyof five palmoplantar squamous-cell carcinomasas well as two other extremity non-melanomaskin cancers. Prior to immigration, she had livedin a county of Southern Hungary that is known tohave elevated concentrations of inorganic arsenicin the drinking water above the World HealthOrganization's current maximum threshold forsafety. To date, this report is the first to describethe phenomenon of palmoplantar squamouscellcarcinomas in a patient from this region andunderscores the importance of vigilant screening inthose individuals who have spent substantial time inhigh-risk regions internationally and domestically.
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Carcinoma de Células Escamosas/diagnóstico , Queratosis/diagnóstico , Neoplasias Cutáneas/diagnóstico , Intoxicación por Arsénico/complicaciones , Carcinoma de Células Escamosas/etiología , Carcinoma de Células Escamosas/patología , Agua Potable , Femenino , Pie , Dermatosis del Pie/diagnóstico , Dermatosis del Pie/etiología , Dermatosis del Pie/patología , Dermatosis de la Mano/diagnóstico , Dermatosis de la Mano/etiología , Dermatosis de la Mano/patología , Humanos , Hungría/etnología , Queratosis/etiología , Queratosis/patología , Persona de Mediana Edad , Neoplasias Cutáneas/etiología , Neoplasias Cutáneas/patologíaAsunto(s)
Pitiriasis Rubra Pilaris/complicaciones , Calidad de Vida , Anciano , Alopecia/etiología , Artralgia/etiología , Femenino , Humanos , Queratodermia Palmoplantar/etiología , Masculino , Persona de Mediana Edad , Pitiriasis Rubra Pilaris/psicología , Grupos de Autoayuda , Encuestas y CuestionariosAsunto(s)
Traumatismos por Radiación/tratamiento farmacológico , Esclerodermia Localizada/tratamiento farmacológico , Adulto , Anciano , Femenino , Humanos , Persona de Mediana Edad , Terapia PUVA , Traumatismos por Radiación/etiología , Traumatismos por Radiación/terapia , Radioterapia/efectos adversos , Estudios Retrospectivos , Esclerodermia Localizada/etiología , Esclerodermia Localizada/terapiaAsunto(s)
Hepatitis B Crónica/complicaciones , Piel/patología , Vasculitis Leucocitoclástica Cutánea/patología , Adulto , Sedimentación Sanguínea , Proteína C-Reactiva/análisis , Femenino , Humanos , Pierna/patología , Púrpura/patología , Vasculitis Leucocitoclástica Cutánea/sangre , Vasculitis Leucocitoclástica Cutánea/etiologíaAsunto(s)
Eosinofilia , Extremidades , Fascitis , Diagnóstico Diferencial , Errores Diagnósticos/prevención & control , Edema/diagnóstico , Eosinofilia/sangre , Eosinofilia/diagnóstico , Eosinofilia/fisiopatología , Extremidades/diagnóstico por imagen , Extremidades/patología , Fascitis/sangre , Fascitis/diagnóstico , Fascitis/fisiopatología , Femenino , Humanos , Persona de Mediana EdadRESUMEN
Precision medicine, which recognizes and upholds the uniqueness of each individual patient and the importance of discerning these inter-individual differences on a molecular scale in order to provide truly personalized medical care, is a revolutionary approach that relies on the discovery of clinically-relevant biomarkers derived from the massive amounts of data generated by epigenomic, genomic, transcriptomic, proteomic, microbiomic, and metabolomic studies, collectively known as multi-omics. If harnessed and mined appropriately with the help of ever-evolving computational and analytic methods, the collective data from omics studies has the potential to accelerate delivery of targeted medical treatment that maximizes benefit, minimizes harm, and eliminates the "fortune-telling" inextricably linked to the prevailing trial-and-error approach. For a disease such as dermatomyositis (DM), which is characterized by remarkable phenotypic heterogeneity and varying degrees of multi-organ involvement, an individualized approach that incorporates big data derived from multi-omics studies with the results of currently available serologic, histopathologic, radiologic, and electrophysiologic tests, and, most importantly, with clinical findings obtained from a thorough history and physical examination, has immense diagnostic, therapeutic, and prognostic value. In this review, we discuss omics-based research studies in DM and describe their practical applications and promising roles in guiding clinical decisions and optimizing patient outcomes.
RESUMEN
Dermatomyositis (DM) is a strikingly heterogenous disease characterized by a broad and ever-evolving spectrum of cutaneous manifestations that transcend the classic "hallmarks" defined by Peter and Bohan in 1975. Despite the increasing preponderance and ubiquity of autoantibody, radiologic, and electrophysiologic testing, the diagnosis of DM still hinges largely on prompt detection of cutaneous manifestations of this condition. While pathognomonic cutaneous features of DM are more readily recognizable, many patients present with subtle and/or atypical skin manifestations, and diagnosis of DM may require clinician identification of these cutaneous clues. In this review, we highlight several of the lesser-known skin manifestations of DM, specifically, panniculitis, diffuse subcutaneous edema, erythroderma, calcinosis, ulceration, flagellate erythema, Wong-type DM, gingival telangiectasias, and the ovoid palatal patch. We describe the clinical and histopathologic presentation of these cutaneous findings. While manifesting less frequently than the heliotrope rash, Gottron's papules, and Gottron's sign, these cutaneous clues are equally important for clinicians to recognize in order to facilitate timely diagnosis and early intervention.
Asunto(s)
Enfermedades del Tejido Conjuntivo/diagnóstico , Eritromelalgia/diagnóstico , Dermatosis Facial/diagnóstico , Biopsia , Enfermedades del Tejido Conjuntivo/patología , Diagnóstico Diferencial , Eritromelalgia/patología , Dermatosis Facial/patología , Humanos , Masculino , Persona de Mediana EdadRESUMEN
This cohort study describes the clinical features, patient characteristics, and treatment of anti-melanoma differentiationassociated gene 5 (MDA5) dermatomyositis.
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Continuidad de la Atención al Paciente , Dermatomiositis , Hospitalización , Helicasa Inducida por Interferón IFIH1 , Humanos , Dermatomiositis/inmunología , Dermatomiositis/tratamiento farmacológico , Dermatomiositis/terapia , Helicasa Inducida por Interferón IFIH1/inmunología , Hospitalización/estadística & datos numéricos , Femenino , Continuidad de la Atención al Paciente/organización & administración , Masculino , Persona de Mediana Edad , Adulto , Autoanticuerpos/sangre , Autoanticuerpos/inmunologíaRESUMEN
This cohort study examines the association between methotrexate use and interstitial lung disease in patients with dermatomyositis.