Male systemic lupus erythematosus in a Latin-American inception cohort of 1214 patients.

The objective of the study was to evaluate the influence of the male gender in the clinical presentation and outcome of systemic lupus erythematosus in a prospective inception cohort of Latin-American patients. Of the 1214 SLE patients included in the GLADEL cohort, 123 were male. Demographic characteristics as well as clinical manifestations, laboratory profile, activity and damage scores were evaluated at onset and during the course of the disease and compared with female patients. The median age at onset of the male patients was 27 and that at diagnosis 29.2 years. Delay to diagnosis was shorter in males (134 versus 185 days, P = 0.01). At onset, men more frequently showed fever (42.3 versus 27.0%, P = 0.001) and weight loss (23.6 versus 11.8%, P = 0.001). During disease course the incident of symptoms was: fever, 67.8 versus 55.6%, P = 0.012; weight loss, 47.2 versus 24.3%, P = 0.001; arterial hypertension, 37.4 versus 25.8%, P = 0.007; renal disease (persistent proteinuria and/or cellular casts), 58.5 versus 44.6%, P = 0.004); and hemolytic anemia, 19.5 versus 10.9%, P = 0.008. The laboratory results showed that: men more frequently had IgG anticardiolipin antibodies (68.2 versus 49%, P = 0.02) and low C3 (61.3 versus 48.1%, P = 0.03); 5/123 men died (4%) compared with 29/1091 women (2.7%). In conclusion, 10% of GLADEL's cohort patients were male. They showed a distinctive profile with shorter delay to diagnosis, higher incidence of fever, weight loss, arterial hypertension, renal disease, hemolytic anemia, IgG anticardiolipin antibodies and low C3. Although not statistically significant, mortality was higher in men.

Analysis of molecular heterogeneity of prolactin in human systemic lupus erythematosus.

Hyperprolactinemia without clinical manifestations has been reported in some patients with systemic lupus erythematosus (SLE) because an increase of prolactin (PRL) is produced due to the BIG/BIG molecular variant (molecular variant < 150 kD). This research project aimed to determine levels of PRL: its bioactive form, the little nonglycosylated form (NGPRL) and variants with decreased bioactivity such as the BIG/BIG and the little glycosylated (GPRL), in 29 women and five men with SLE. PRL was assayed by IRMA with a kit from Immunotech Laboratory, the BIG/BIG form by precipitation with polyethyleneglycol 6000, and the NGPRL and GPRL by chromatography on Concanavalin-A- Sepharose. Increased PRL was detected in seven patients (20.6%) of whom three had increased BIG/BIG, six had increased GPRL and only four had increased NGPRL. The three cases with increased BIG/BIG were contrasted by chromatography on Sephadex G-100. No increased PRL or any of the other variants assayed were found in men. Results were similar when PRL was evaluated in the same blood samples by a different IRMA (DPC Laboratory). The etiology of the hyperprolactinemia in some of these patients is unknown, but their lack of symptoms (galactorrhea or amenorrhea) could be due to the BIG/BIG forms and basically to the glycosylation of the hormone. As for the relation between PRL and SLE activity, we found that hyperprolactinemic patients were younger, had a shorter history of illness, although it was not statistically significant, and a higher SLEDAI score. This would indicate a relation between hyperprolactinemia and lupus activity. The patients with increased BIG/BIG form also had a very active illness at the time of the study.

Prevalencia de subtipos de HLA B 27 en espondiloartropatías en Argentina / Prevalence of subtypes of HLB B27 in spondyloarthropathies in Argentina

Objetivo: analizar la distribución de los diferentes alelos B 27 (B 27.01-B 27.11) del complejo mayor de histocompatibilidad (CMH) en la población sana y en un grupo de pacientes con Espondiloartropatías Seronegativas (EASN) HLA B27 +. Materiales y métodos: s estudiaron 1.000 muestras de sangre provenientes de individuos normales donantes de sangre de la población general y se los comparó con 55 pacientes con diagnóstico de EASN: 33 pacientes (60 por ciento) con Espondilitis Anquilastante (EA), 12 (21.8 por ciento) con Síndrome de Reiter (SR) y 10 (18.1 por ciento) con Artropatía Psoriásica (APs). Los pacientes con EA y SR estudiados con anterioridad pertenecían a un grupo B 27 +. Se utilizaron técnicas de biología molecular PCR-SSO y por medio de sondas específicas PAN-B 27 se analizó la frecuencia de los alelos B 27.01 al B 27.11. No fue evaluada la presencia del alelo B 27.12. Resultados: de los 1000 donantes de sangre de la población general, 4.2 por ciento (42 personas) fueron B 27 + (en relación con estudios serológicos previos que mostraban un 3.5 por ciento)... (AU)

Prevalencia de subtipos de HLA B 27 en espondiloartropatías en Argentina / Prevalence of subtypes of HLB B27 in spondyloarthropathies in Argentina

Objetivo: analizar la distribución de los diferentes alelos B 27 (B 27.01-B 27.11) del complejo mayor de histocompatibilidad (CMH) en la población sana y en un grupo de pacientes con Espondiloartropatías Seronegativas (EASN) HLA B27 +. Materiales y métodos: s estudiaron 1.000 muestras de sangre provenientes de individuos normales donantes de sangre de la población general y se los comparó con 55 pacientes con diagnóstico de EASN: 33 pacientes (60 por ciento) con Espondilitis Anquilastante (EA), 12 (21.8 por ciento) con Síndrome de Reiter (SR) y 10 (18.1 por ciento) con Artropatía Psoriásica (APs). Los pacientes con EA y SR estudiados con anterioridad pertenecían a un grupo B 27 +. Se utilizaron técnicas de biología molecular PCR-SSO y por medio de sondas específicas PAN-B 27 se analizó la frecuencia de los alelos B 27.01 al B 27.11. No fue evaluada la presencia del alelo B 27.12. Resultados: de los 1000 donantes de sangre de la población general, 4.2 por ciento (42 personas) fueron B 27 + (en relación con estudios serológicos previos que mostraban un 3.5 por ciento)...

Clinic and functional evaluation of total hip and knee prostheses in patients with juvenile chronic arthritis: a 20-year experience / Avaliaçäo clínico-funcional de próteses de quadril e joelho em pacientes com artrite crônica juvenil: uma experiência de 20 anos

Foram revistos os resultados de implante de prótese total de quadril e de joelho em pacientes com artrite crônica juvenil e acompanhados em nosso Serviço nos últimos 20 anos. Os 40 casos avaliados (34 com artrite reumatóide juvenil e seis com espondilite anquilosante juvenil) receberam 71 próteses (61 de quadril e dez de joelho): quatro dos 34 casos de artrite reumatóide juvenil receberam três ou mais próteses articulares. No grupo de 61 próteses de quadril (23 bilaterais), a idade média à cirurgia foi de 24 anos, com 85 por cento dos pacientes abaixo de 31 anos. O acompanhamento pós-operatório médio foi de 60 meses. Os resultados foram excelentes em 46 próteses de quadril (75 por cento) e bons ou pobres em 15 (25 por cento). Das 61 próteses de quadril observou-se infecçäo em dois casos (3 por cento), falência mecânica devida a soltura em sete casos (11 por cento) e ossificaçäo heterotópica em 25 casos (45 por cento). Houve necessidade de revisÝo cirúrgica em sete próteses. Em um período pós-operatório médio de 47 meses, todos os seis casos de artrite reumatóide juvenil recebendo prótese total de joelho (bilaterais) apresentaram-se sem complicaçöes clínicas, com resultados bons a excelentes em 80 por cento dos casos. Pacientes que receberam múltiplas próteses devido a grave incapacitaçäo funcional (grau IV) obtiveram melhora significante. Conclui-se que artroplastia total de quadril e joelho é uma valiosa modalidade terapêutica para reabilitaçäo de casos de artrite crônica juvenil

Artroplastias de caderas y rodillas en artritis cronica juvenil / Hip and knee arthroplasties in juvenile chronic arthitis: our twenty-year experience

Se revisaron los resultados clinico-funcionales de las protesis totales de caderas y rodillas realizadas en pacientes con Artritis Cronica Juvenil (ACJ) seguidos en nuestro servicio durante 20 años. Se evaluaron 40 pacientes que recibieron un total de 71 protesis (61 caderas y 10 rodillas); 4 de 34 pacientes con ARJ, recibieron 3 o mas reemplazos articulares. Se realizaron 61 artroplastias totales de caderas (23 bilateral); la edad promedio a la cirugia fue de 24 años; el 85 por ciento de los pacientes era menor de 35 años. El tiempo promedio de seguimiento postoperatorio fue de 60 meses. Los resultados fueron a excelentes en 46 protesis (75 por ciento) y regular a pobre en 15 (25 por ciento). De estas 61 protesis se observo infeccion en 2 casos (3 por ciento), fallo mecanico debido a aflojamiento en 7 (11 por ciento) y osificacion heterotopica en 25 (41 por ciento). Siete protesis debieron ser revisadas quirurgicamente. Los 6 pacientes con ARJ que recibieron un total de 10 protesis de rodilla (4 bilaterales), permanecieron libres de complicaciones clinicas, alcanzando resultados bueno a excelente en el 80 por ciento de los casos. Los cuatro pacientes que fueron sometidos a reemplazos multiples por severa discapacidad funcional (grado IV ACR) mejoraron significativamente posterior a la cirugia funcional no clinica. Las atropatias totales de caderas y rodillas constituyen un recurso terapeutico a tener en cuenta en la rehabilitacion de pacientes con ADJ con severo compromiso funcional

Artroplastias de caderas y rodillas en artritis cronica juvenil / Hip and knee arthroplasties in juvenile chronic arthitis: our twenty-year experience

Se revisaron los resultados clinico-funcionales de las protesis totales de caderas y rodillas realizadas en pacientes con Artritis Cronica Juvenil (ACJ) seguidos en nuestro servicio durante 20 años. Se evaluaron 40 pacientes que recibieron un total de 71 protesis (61 caderas y 10 rodillas); 4 de 34 pacientes con ARJ, recibieron 3 o mas reemplazos articulares. Se realizaron 61 artroplastias totales de caderas (23 bilateral); la edad promedio a la cirugia fue de 24 años; el 85 por ciento de los pacientes era menor de 35 años. El tiempo promedio de seguimiento postoperatorio fue de 60 meses. Los resultados fueron a excelentes en 46 protesis (75 por ciento) y regular a pobre en 15 (25 por ciento). De estas 61 protesis se observo infeccion en 2 casos (3 por ciento), fallo mecanico debido a aflojamiento en 7 (11 por ciento) y osificacion heterotopica en 25 (41 por ciento). Siete protesis debieron ser revisadas quirurgicamente. Los 6 pacientes con ARJ que recibieron un total de 10 protesis de rodilla (4 bilaterales), permanecieron libres de complicaciones clinicas, alcanzando resultados bueno a excelente en el 80 por ciento de los casos. Los cuatro pacientes que fueron sometidos a reemplazos multiples por severa discapacidad funcional (grado IV ACR) mejoraron significativamente posterior a la cirugia funcional no clinica. Las atropatias totales de caderas y rodillas constituyen un recurso terapeutico a tener en cuenta en la rehabilitacion de pacientes con ADJ con severo compromiso funcional

Granuloma eosinófilo. Presentación de dos casos clínicos / Eosinophylic granuloma

La Histiocitois X es una rara entidad proliferativa benigna que incluye al :1)granuloma eosinófilo.2) Sindrome de Hand Schuller Christian y 3) Enfermedad de Lettere Siwe.El propósito de esta comunicación es presentar dos pacientes portadores de esta entidad.Caso 1: Mujer de 28 años que consultó por coxalgia y diabetes insípida de dos años de evolución.Presentó imágenes líticas en cabeza femoral y ramas isquiopubianas.La bipsia ósea reveló el diagnóstico de granuloma eosinófilo.Las lesiones respondieron a la terapia radiante.Dos años después desarrolló nuevas lesiones osteolíticas en cráneo,arcos cortales y columna.Catorce años después desarrolló aplastamiento vertebral con compresión radicular que requirió cirugía estabilizadora con evolución favorable.Caso 2: Mujer de 57 años de edad que consultó por cefalea,náuseas,vómitos y tumuración occipital.al año desarrolló una lesión tumoral expansiva en zona epitroclear izquierda.Presentaba imágenes líticas múltiples.Se diagnosticó granuloma por biopsia ósea.Ambas pacientes recibieron tratamiento con vinblastina presentando mejoría de la enfermedad sin progresión radiológica de la misma.

Granuloma eosinófilo. Presentación de dos casos clínicos / Eosinophylic granuloma

La Histiocitois X es una rara entidad proliferativa benigna que incluye al :1)granuloma eosinófilo.2) Sindrome de Hand Schuller Christian y 3) Enfermedad de Lettere Siwe.El propósito de esta comunicación es presentar dos pacientes portadores de esta entidad.Caso 1: Mujer de 28 años que consultó por coxalgia y diabetes insípida de dos años de evolución.Presentó imágenes líticas en cabeza femoral y ramas isquiopubianas.La bipsia ósea reveló el diagnóstico de granuloma eosinófilo.Las lesiones respondieron a la terapia radiante.Dos años después desarrolló nuevas lesiones osteolíticas en cráneo,arcos cortales y columna.Catorce años después desarrolló aplastamiento vertebral con compresión radicular que requirió cirugía estabilizadora con evolución favorable.Caso 2: Mujer de 57 años de edad que consultó por cefalea,náuseas,vómitos y tumuración occipital.al año desarrolló una lesión tumoral expansiva en zona epitroclear izquierda.Presentaba imágenes líticas múltiples.Se diagnosticó granuloma por biopsia ósea.Ambas pacientes recibieron tratamiento con vinblastina presentando mejoría de la enfermedad sin progresión radiológica de la misma.

Protrusio acetabuli in juvenile rheumatoid arthritis.

Acetabular protrusion (PA) as measured by a line crossing method was studied in 73 patients with juvenile rheumatoid arthritis (JRA) and its frequency found to be 12% (9/73), presenting bilaterally in 5 cases and unilaterally in 4. All patients had some other forms of radiological damage and the presence of PA was predominantly observed in the JRA group with greater age at onset (8 vs 4.2 years; p < 0.001) and lower frequency of extraarticular manifestations (22% vs 72%; p < 0.01). There was no correlation with type of JRA onset, course of disease, sex, disease duration, seropositivity for rheumatoid factor, and prior steroid intake.

Superolateral erosions of the humeral head in chronic inflammatory arthropathies.

Erosive lesions on the superolateral aspect of the humeral head were studied in 127 patients with chronic inflammatory arthropathies including rheumatoid arthritis (RA), juvenile rheumatoid arthritis (JRA), ankylosing spondylitis (AS), and psoriatic arthropathy (PA), as well as in a control group of 53 patients with non-inflammatory shoulder joint disease. Superolateral erosions were found in 39 out of 127 patients (31%), comprising 11/56 RA cases (20%), 22/50 JRA cases (44%), 4/9 cases of AS (44%), and 2/12 cases of PA (17%), but were absent in non-inflammatory disorders. Two morphologically distinct types of erosions were observed, an extensive one, present in all of the inflammatory conditions studied, and a circumscribed one occurring predominantly in JRA patients.

Atlantoaxial subluxation in systemic lupus erythematosus: further evidence of tendinous alterations.

We prospectively determined the frequency of atlantoaxial subluxation in a group of patients with systemic lupus erythematosus (SLE) and analyzed its relationship with tendinous laxity, Jaccoud's syndrome and other features of the disease. Five of 59 patients (8.5%) had atlantoaxial subluxation. No patient presented atlantoaxial subluxation in neutral lateral cervical radiographs but all 5 had anterior atlantoaxial subluxation in full flexion films; one patient also had lateral subluxation. The 5 patients with atlantoaxial subluxation were compared with the remaining 54. Mean SLE disease duration was longer in patients with atlantoaxial subluxation (12 years) than in those without (6.6 years) (p less than 0.01). Jaccoud's syndrome, patellar tendon elongation and articular hypermobility were significantly more frequent in patients with atlantoaxial subluxation. The presence or history of arthritis failed to distinguish patients with and without atlantoaxial subluxation, while chronic renal failure and increased serum parathyroid hormone levels were significantly associated to the presence of atlantoaxial subluxation. We suggest that atlantoaxial subluxation is further evidence of tendinous alterations seen in patients with SLE.

Evaluation of the measurement methods for protrusio acetabuli in normal children.

The normal values of the radiological lines most frequently used as references in the diagnosis of adult protrusio acetabuli were prospectively determined in 150 children (300 hips) without femoral pathology and aged between 2 and 15 years, mean age 8 years. The teardrop shape was modified according to the child's age, the "crossed" type predominating (48.7%). The mean centre edge (CE) angle fluctuated, with a median value of 31.2 degrees +/- 6.2 degrees, increasing with age and with slightly greater values in females; a large spread of CE angle values was observed within age groups. Acetabular ilioischial line values ranged from +7 to -5 mm, with a mean of +1.8 +/- 2 mm. This last method was the most suitable because it was not modified by changes in incidence of X-rays in radiological studies. With this method protrusio is diagnosed in children when the acetabular line is projected medially, provided that the ilioischial line is 1 or 3 mm or more for boys and girls, respectively. We conclude that the most trustworthy method available to evaluate PA in children should be "line crossing".

Tendinous laxity and Jaccoud's syndrome in patients with systemic lupus erythematosus. Possible role of secondary hyperparathyroidism.

Fourteen of 52 unselected patients with systemic lupus erythematosus (SLE) (27%) had ligamentous derangement demonstrated by either Jaccoud's syndrome and/or patellar tendon elongation. Three cases had only Jaccoud's syndrome, 4 isolated patellar tendinous laxity, while the remaining 7 presented both findings. Jaccoud's syndrome and/or tendinous laxity were not associated to an increased frequency of arthritis, corticosteroid therapy or a longer disease duration, but significantly associated with increased serum PTH levels secondary to chronic renal failure. Hyperparathyroidism secondary to chronic renal failure should, therefore, be considered a potential factor contributing to the development of Jaccoud's syndrome and/or tendinous laxity in patients with SLE.

Laxity and rupture of the patellar tendon in systemic lupus erythematosus. Association with secondary hyperparathyroidism.

We describe a patient with systemic lupus erythematosus, chronic renal failure and secondary hyperparathyroidism who developed laxity and elongation of the patellar tendon, followed by its rupture one year later. Hydroxyapatite and urate crystals were identified from the chalky material surrounding the patellar tendon observed at surgery. These findings suggest that tendon laxity and elongation could possibly be detected before its rupture and that secondary hyperparathyroidism could be considered as another causative factor of tendon damage in patients with systemic lupus erythematosus.

Radiologic review: the cervical spine in juvenile rheumatoid arthritis.

In our patients, zygapophyseal joint fusion was the most frequent and characteristic roentgenographic finding of cervical spine involvement in JRA. It was not associated with any subset of patients. Atlanto-dens interval greater than 4.5 mm was present in 20% of patients, but was not related to the development of neurologic manifestations. Mild enthesopathic-like changes around the upper cervical area were observed in some children. In patients with severe long standing disease and extensive zygapophyseal joint fusion, longitudinal ligament calcification was observed. Growth disturbances of the cervical spine were more frequently observed in patients with zygapophyseal joint fusion and earlier onset of disease.

Effect of antimalarial treatment on circulating immune complexes in rheumatoid arthritis.

The relationship between the effect of chloroquine treatment on circulating immune complexes in patients with rheumatoid arthritis (RA) was determined by the 125I Clq binding assay. Three groups were studied: (a) 20 patients treated daily for 6 months with chloroquine sulphate 250 mg plus prednisone 7.5 mg and nonsteroidal antiinflammatory drugs (NSAID); (b) 20 patients not taking antimalarials but treated with prednisone and NSAID; (c) 41 controls. Results suggest that chloroquine sulphate therapy induced a marked decrease of immune complexes in patients with RA, an effect not observed with treatment by prednisone and NSAID only.

Ectopic ossification following total hip replacement in juvenile chronic arthritis.

Of twenty-one patients with juvenile chronic arthritis (seventeen with juvenile rheumatoid arthritis and four with juvenile ankylosing spondylitis) who had total hip replacement before the age of thirty years, thirteen patients (62 per cent) showed different degrees of ectopic ossification: nine (53 per cent) of the seventeen with juvenile rheumatoid arthritis and all four of those with juvenile ankylosing spondylitis. Human leukocyte antigen B5 was found with an increased frequency in the patients with ectopic ossification. Functional capacity was not impaired regardless of the severity of the ectopic ossification. As in patients with adult-onset disease, ectopic ossification after total hip replacement occurred with a higher frequency in the patients with juvenile-onset ankylosing spondylitis than in those with juvenile-onset rheumatoid arthritis.