Concomitancia de anticuerpos antimembrana basal y ANCA en el síndrome riñón-pulmón / Concomitance of glomerular basement membrane antibodies and ANCA in pulmonary - renal syndrome

La enfermedad antimembrana basal glomerular (anti-MBG) es una condición que se manifiesta clínicamente como glomerulonefritis rápidamente progresiva y hemorragia alveolar, también llamada Síndrome Riñón- Pulmón. Se asocia a la presencia de autoanticuerpos dirigidos contra el colágeno tipo IV de la membrana basal glomerular. Las vasculitis sistémicas asociadas a ANCA también pueden manifestarse como Síndrome Riñón-Pulmón, cuadro clínico a veces indistinguible de la enfermedad anti-MBG. La concomitancia de ANCA y anticuerpos anti-MBG en el Síndrome Riñón-Pulmón es del orden de un 30 por ciento, según distintos reportes de la literatura. El perfil clínico, el pronóstico y el rol fisiopatológico de cada anticuerpo en este grupo de pacientes todavía son materia de investigación. El mecanismo patogénico inicial parece ser el daño mediado por ANCA, que puede inducir la aparición de anticuerpos anti-MBG, los que perpetúan el daño en el glomérulo.

Anti-glomerular basement membrane (anti-MBG) disease is a condition that is manifested clinically as rapidly progressive glomerulonephritis and alveolar hemorrhage, also known as Pulmonary-Renal Syndrome. It is associated with the presence of autoantibodies directed against type IV collagen of the glomerular basement membrane. Systemic vasculitis associated with ANCA may also manifest as Pulmonary-Renal Syndrome, sometimes clinically indistinguishable from the anti-MBG disease.The concomitance of ANCA and anti-MBG antibodies in the Pulmonary-Renal Syndrome is about 30 percent, according to various reports in literature. The clinical profile, prognosis and physiopathologic roles of each antibody in this group of patients is still under investigation. The pathogenic mechanism appears to be the initial damage mediated by ANCA, which may induce the appearance of anti-MBG, those who perpetuate the glomerulus damage.

Síndrome POEMS: reporte de un caso clínico / POEMS syndrome: report of a clinical case

El síndrome POEMS se caracteriza por la presencia de Polineuropatía, Organomegalia, Endocrinopatía, Gammapatía Monoclonal y Cambios en la Piel. Muchas otras manifestaciones clínicas del síndrome no están incluidas en este acrónimo, como las lesiones óseas osteoescleróticas, edema de papila, sobrecarga de volumen extravascular, trombocitosis, policitemia, acropaquia, hipertensión pulmonar, baja de peso, fatiga, diarrea, compromiso renal y artralgias. Es una patología infrecuente, de mayor prevalencia en varones, con una relación 2:1, preferentemente entre la quinta y sexta década de la vida. Es una enfermedad de curso crónico y tiene sobrevida promedio de 13,8 años en algunas series. La patogenia es desconocida y no existe en la actualidad consenso respecto al tratamiento ni estudios randomizados y controlados que comparen los resultados de diferentes terapias en esta enfermedad.

POEMS syndrome is characterized by the presence of Polyneuropathy, Organomegaly, Endocrinopathies, Monoclonal Gammopathy and Skin changes. Many other clinical manifestations of the syndrome are not included in this acronym, as the osteosclerotic lesions, papillary edema, extravascular volume overload, thrombocytosis, polycythemia, acropaquia, pulmonary hypertension, weight loss, fatigue, diarrhea, arthralgia, and renal involvement. It is an infrequent disease, more prevalent in males with a 2:1 ratio, mostly between the fifth and sixth decades of life. It has a chronic course with an average survival of 13.8 years in some series. The pathogenesis is unknown and currently there is no consensus regarding the treatment or randomized controlled studies comparing the outcomes of different therapies in this disease.

Compromiso cardiovascular en el lupuseritematoso sistémico / Cardiovascular involvement in patient with systemic lupus erythematosus

Lupus erythematosus is a multisystemic disease that compromises principally women in fertile age. The principal affected organs are kidney, SNC, bone marrow and serous membranes. Cardiovascular affection includes pericardium, conduction system, myocardium, valves and coronary arteries. The most frequent valve disease is Libman-Sacks endocarditis, although valvulitis or valve dysfunction can exist as well. The mitral valve is the most affected, followed by the aortic valve. The most frequent valve abnormality is slight to moderate aortic insufficiency, while serious insufficiency or valve disruption is very rare. A physical examination has limited efficacy in the diagnosis of valve disease. A high degree of suspicion associated with echocardiography helps to establish the diagnosis. If surgery is not needed, antiplatelet therapy is recommended for asymptomatic patients, and oral anticoagulation treatment is advised for those with valve disease and evidence of thromboembolic phenomena. Recurrence of the disease has been observed in biological grafts, which makes the use of mechanical prostheses advisable when valve replacement indication exists. The following case shows the clinical evolution of a female patient with a rare but very serious lupus erythematosus complication.