José Félix Restrepo

Lupus arthropathy: a case series of patients with rhupus

Among the clinical manifestations of systemic lupus erythematosus (SLE) is an arthropathy, which is usually nonerosive. In many cases the joint involvement is mild. A subset of patients have deforming, nonerosive Jaccoud’s arthropathy, and a minority have an arthropathy with clinical findings similar to rheumatoid arthritis (RA) that has been called “rhupus.” We report our series of eight patients (seven female, one male) with rhupus arthropathy. Patients were between the ages of 17 and 38 years (average: 30.3 years) at disease onset. All had deforming or Jaccoud∝s arthropathy, and three had erosive disease. The arthritis was typically the first disease manifestation. Other symptoms of lupus including vasculitis and glomerulonephritis appeared after an average of 2.8 years. All had positive antinuclear antibody and rheumatoid factor. Rhupus arthritis is not a combination of RA and SLE, but should be regarded as a variant of the arthropathy of lupus.

Lupus arthropathy: historical evolution from deforming arthritis to rhupus

Systemic lupus erythematosus is an autoimmune and inflammatory disease with multiple clinical manifestations, including arthropathy. The clinical presentation of articular involvement is variable, ranging from arthralgia without erosions or deformity to an erosive arthropathy and severe functional disability. A subset of patients with this articular involvement have Jaccoud’s arthropathy, and others have an arthropathy with clinical findings similar to rheumatoid arthritis that has been called “rhupus.” In this paper we review the historical evolution of concepts of lupus arthropathy, from deforming arthritis to rhupus, and conclude that rhupus is not a combination of rheumatoid arthritis and lupus. Instead, rhupus arthropathy should be regarded as a variant of the arthropathy of systemic lupus erythematosus.

Takayasu arteritis in Colombia.

Takayasu arteritis has been recognized in Colombia just recently, and so far we do not have any report concerning its presentation here. In this first report, some issues related to the presentation of the disease are indicated and compared with those found in the medical literature. No differences were found in age and sex. Most of the cases were diagnosed during an inactive phase of the disease with advanced manifestations due to vascular lesion which suggests the existence of some genetic factor influencing such a presentation, or may be the consequence of a delay in diagnosing the disease during initial and active stages due to not suspecting it. Comparing the vessels which are affected among other races and countries, we can find both differences and similarities. With the purpose of discovering the demographic, clinical, angiographic and laboratorial characteristics of Takayasu arteritis in Colombia, the present study was carried out by studying 35 clinical cases in different medical centers of the country.

A Brief History of Stoll-Brodie-Fiessinger-Leroy Syndrome (Reiters Syndrome) and Reactive Arthritis with a Translation of Reiters Original 1916 Article into English

While not the only form of reactive arthrits, Reiters syndrome is the eponym given to a form of reactive arthritis associated with with the classice triad of conjuntivitis, urethritis, and inflammatory arthritis. Although in popular use, the term Reiters syndrome has become clouded, not only because of the variable pathophysiology of reactive arthritis, but also because of Reiters own past as an early member of the Nazi party and his prominent role in the German health system during the Thrid Reich, including involvement with involuntary medical procedures and experiments in Nazi concentration camps. As is often the case, the eponym attached to the syndrome does not honor the original describers of the disease, although doubtless Reiters account remains the classic description. We offer a brief historical review of the disease, and complete it with a translation into English of Reiters original publication.

Defectos en la síntesis de cadena ζ en el linfocito T, generando señales intracelulares equivocadas, responsables de lupus eritematoso sistémico

Resumen La expresion anormal de moleculas claves en senalizacion y la funcion defectuosa de los linfocitos T cumplen un papel significativo en la patogenesis de la enfermedad autoinmune. Las celulas T muestran numerosas anormalidades en la senalizacion del complejo TCRζ 1 , estas aberraciones resultan en la alteracion de la expresion de citoquinas. Mientras algunas de estas anormalidades explican el aumento de la actividad de celulas B por celulas T con incremento de los anticuerpos, la disminucion en la produccion de IL-2 resulta en un aumento en la susceptibilidad a las infecciones, reduccion en la activacion de las celulas T, induccion de la muerte celular y prolongada sobrevida de las celulas T autorreactivas 2 .

Artropatía de Jaccoud y fibrosis pulmonar en síndrome CREST

Resumen Se describe el caso de una paciente de 48 anos de edad con diagnostico de sindrome CREST incompleto (variante de esclerosis sistemica limitada), en quien se documenta la presencia de artropatia de Jaccoud de las manos y compromiso pulmonar intersticial por fibrosis pulmonar tipo neumonia intersticial usual, con positividad para factor reumatoide y anticuerpos contra el peptido citrulinado.

Epidemiología de las vasculitis primarias en Colombia y su relación con lo informado para Latinoamérica

Resumen Propuesta las vasculitis primarias se expresan de forma variable entre pacientes y entre regiones, con frecuencias variables a traves del mundo. Su incidencia promedio esta calculada en 0,3 a 20 casos por millon de habitantes. Nosotros describimos la frecuencia de las vasculitis primarias en Colombia y la comparamos con lo informado en otros paises de Latinoamerica (LA). Metodos se reviso lo publicado en la literatura de vasculitis primaria en Colombia y en LA desde 1945 hasta 2007 en OLD Medline, Pub Med, BIREME, SciELO Colombia, LILACS. FEPAFEN, incluyendo publicaciones en ingles, espanol y portugues. La literatura incluye la informacion publicada del Hospital San Juan de Dios, Bogota, Colombia y casos sin publicacion enviados directamente por los autores. Nosotros calculamos el porcentaje para todos los casos que fueron informados para Colombia. Resultados se identificaron 857 casos de vasculitis primaria en Colombia. La arteritis de Takayasu fue la vasculitis mas frecuente en un 13,3% (114 casos) seguida de la enfermedad de Buerger en 11,2% (96 casos), las vasculitis cutaneas primarias y la poliarteritis nodosa en un 10% (86 casos) cada una. En ninos, la vasculitis mas frecuente fue la purpura de Henoch Schonlein en un 24% (206 casos). En Latinoamerica se publicaron 177 articulos con 1605 casos informados. Se evidencio mayor presencia de arteritis de Takayasu en Mexico y Brasil, y de poliangeitis microscopica en Chile y Peru. Conclusion la mayoria de publicaciones sobre vasculitis primarias provienen de Europa, Norteamerica, Japon, Kuwait y Nueva Zelanda. Existen una serie de publicaciones y experiencia con estas patologias en LA. Un numero considerable de publicaciones y casos con vasculitis primarias se han informado en Colombia en los recientes anos, incluyendo la reciente descripcion de la variante nodular de la vasculitis cutanea. La arteritis de Takayasu fue la variante mas reportada del promedio de vasculitis, al igual que en Brasil y Mexico. En contraposicion a los hallazgos realizados en Brasil, Colombia y Mexico, las vasculitis asociadas a ANCA fueron la forma mas informada en paises como Chile y Peru. La mayoria de casos informados en LA provienen de Mexico, siendo Colombia el segundo pais en frecuencia. Es posible que exista mas informacion pero no la conocemos, por no estar publicada. No existen estudios de incidencia y prevalencia. A escala mundial solo existen estudios de incidencia de las vasculitis primarias, y solo se han realizado estudios de incidencia y prevalencia en la granulomatosis de Wegener.

Osteomesopicnosis asociada a litiasis renal, informe de un caso. Diagnóstico diferencial de las enfermedades osteoesclerosantes axiales

La osteomesopicnosis, junto a la picnodisostosis, osteodistrofia renal, hyperostosis con osteoesclerosis con aumento de la fosfatasa alcalina y osteopetrosis, forman parte de un grupo de enfermedades poco frecuentes, que se caracterizan por la osteoesclerosis, especialmente del esqueleto axial, con lesiones tipicamente en forma de parches, comprometiendo ademas la pelvis, a nivel de los acetabulos, los bordes superiores e inferiores de los cuerpos vertebrales de la region lumbar y la region proximal de femur y del humero. Es de caracter benigno y heredada en forma autosomica dominante. La primera descripcion de esta entidad la realizaron Simon, Cazalis, Dryll, et al en 1979. El nombre de la enfermedad fue propuesto por Maroteaux en 1980. En este articulo presentamos una breve descripcion de las enfermedades oseas con osteoesclerosis, presentamos nuestra casuistica de enfermedades con alteraciones morforradiologicas, describimos un caso de osteomesopicnosis asociado a litiasis renal y proponemos una clasificacion para enfermedades osteoesclerosantes del esqueleto axial junto a un enfoque practico para el diagnostico diferencial de estas enfermedades.

Arthropathy in Patients with CREST Variant Scleroderma

Background: Systemic sclerosis is a disease characterized by sclerosis of the skin, internal organs and vasculopathy. Articular manifestations are common, and include arthralgias, arthritis, and morning stiffness, which in some cases can be confused with rheumatoid arthritis (RA). Patients develop functional impairment and thickening of the skin including around the joints, with inflammation and fibrosis of tendon sheaths. However, articular inflammation is rare clinically, as is the development of articular erosions. Objective: To define the presence and characteristics of arthropathy in patients with scleroderma and distinguish it from RA. Methods: Case series of 106 Colombian patients with scleroderma evaluated between January 1998 and December 2004, of whom 5 had significant articular involvement and are the subject of this report. Results: All patients had the CREST variant of scleroderma. Their average age was 48.6 years (range 35-56). All had arthropathy affecting mainly the hands and feet. In the majority of cases the clinical picture resembled that of RA, with inflammation and subluxation of the metacarpophalangeal joints, and involvement of the feet with subluxation of the toes. Radiographs revealed joint space narrowing, subluxation, juxtaarticular osteopenia, carpal ankylosis and erosions. Rheumatoid factor was negative and antinuclear antibody (ANA) was positive with anticentromere antibodies in all patients. Conclusions: We conclude that there is a unique arthropathy of scleroderma which is due to inflammation and mechanical factors related to skin and periarticular involvement from the underlying disease. It is not due to coexisting RA. All of our cases had CREST variant scleroderma with erosive arthritis, negative rheumatoid factor, and positive ANA with centromere antibody. None fulfilled classification criteria for RA. This is a distinct subtype of scleroderma. Patients should be identified and treated promptly to avoid development of serious articular disease. The tendency to develop severe articular disease is likely immunogenetically linked.

Renal vasculitis in Colombia

A retrospective review of publications about primary vasculitis cases was performed from the Colombian medical literature, the clinical and pathological features are described. The Renal vasculitis occurred in 33.9% of cases of primary vasculitis reviewed and was more frequent in women. The main clinical, hematological, immunological variables and nephropathology and immunosuppressive treatments used findings are described.