Alvaro Sánchez

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.

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.

PS8:150 Mycobacterial infection in systemic lupus erythematosus: clinical significance and associated factors. data from the registry of patients with sle of the spanish society of rheumatology (relesser)

The aim of this work is to study the prevalence of mycobacterial infection (M.I.), the associated factors and their clinical significance in patients included in a large SLE cohort. Methods Retrospective descriptive study of RELESSER patients with a history of M.I. and analysis of the factors associated with the infection of this aetiology. Results In RELESSER 3,658 SLE patients were included. 90% women, mean age of 32.9 years. 93% Caucasians. The mean follow-up time (±S.D.) was 120.2 (±87.6) months. 705 (19.3%) patients had at least a serious infection, 1227 serious infections occurred. M.I. were diagnosed in 42 patients (1.2% of all RELESSER patients, 3.4% of all serious infections), 85.7% women. The incidence rate of mycobacterial infection was 1 per 1000 patients/year (95% CI: 0.7 to 1.4). M.I. presentation was pulmonary in 18 (42.9%) patients and extrapulmonary in 24 (57.1%)patients:joints in 8 (19.0%) patients, soft tissue in 6 (14.3%) and other sites in 10 (23.8%). The extrapulmonary form was associated with the use of immunosuppressants: 84.6% of the 13 patients treated with immunosuppressive drugs versus 44.4% of the 27 patients without (p=0.01). We did not observe this association with the use of corticosteroids. To study the factors associated with mycobacterial infection, we performed a bivariate analysis including the variables associated with severe infection identified in RELESSER (age, sex, ethnicity, use of corticosteroids, immunosuppressants, antimalarials, previous admission by SLE activity, use of rituximab, use of anti-TNF, Katz severity index, SDI damage index, SLEDAI activity index and Charlson comorbidity index). There is a statistically significant association with previous admission by SLE activity (RR: 2.9,95–95% CI: 1.3 to 6.2, p=0.007), renal impairment (RR:2.0, 95% CI: 1,1 to 3,7, p=0,04), the Katz score (RR: 2.1, 95% CI:1.1–4.0,p=0.04) and the Charlson index (RR: 2.5; 95% CI: 1.3 to 4.8, p=0.009). The accumulated damage (SDI>0) was closely associated with significance: RR: 2.0; 95% CI: 1.0 to 4.0, p=0.07. The use of immunosuppressants was associated with a significant increase in the risk of mycobacterial infection: RR: 4.3; 95% CI: 2.2 to 8.3, p=0.31. Two patients (4.8%) died (1respiratory and 1extrapulmonary). The mean survival after diagnosis in these cases was 21 days. Conclusion M.I. in RELESSER affects 1.15% of patients. Its incidence rate is 1 per 1000 patients/year (95% CI: 0.7 to 1.4). Extrapulmonary localization affects more than half of the patients and is associated with the use of immunosuppressants. Previous admission by SLE activity, renal involvement, severity of SLE, and increased number of associated comorbidities are factors associated with the existence of mycobacterial infection.