2016, Number 3
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An Med Asoc Med Hosp ABC 2016; 61 (3)
Juvenile systemic lupus erythematosus: clinical and immunological patterns of disease expression in a cohort of 150 patients of the Hospital Infantil de México «Federico Gómez»
Rodríguez GAN, Osorio SSB, Maldonado VMR, Faugier FE
Language: Spanish
References: 17
Page: 182-187
PDF size: 207.85 Kb.
ABSTRACT
Background: Juvenile systemic lupus erythematosus is a chronic multisystem autoimmune disease of unpredictable course and prognosis. It manifests with a wide spectrum of clinical and immunological abnormalities.
Objective: To define the pattern of disease expression and gain a better understanding of juvenile systemic lupus erythematosus in Mexico.
Methods: We retrospectively analyzed the demographic, clinical and laboratory features of patients with juvenile systemic lupus erythematosus diagnosed before age 18 in the Children’s Hospital of Mexico.
Results: A cohort of 150 patients with a mean age at diagnosis of 11.3 ± 3.13 years (mean ± 1 standard deviation) (range, 2-17 years) and a mean period of follow-up of 3.34 ± 2.14 years. One hundred thirty-one (87.3%) patients were female. The most common manifestations were mucocutaneous (74.7%), hematological (56%) and musculoskeletal (39.3%) abnormalities. Upon diagnosis, renal damage was found in 56 patients (37.3%), of which 28 (18.3%) were diagnosed with lupus nephritis. Antinuclear and double-stranded anti-DNA antibodies were positive in most patients, 96.7% and 77.3%, respectively; antiphospholipid antibody positivity was observed in 40.7%. During follow-up, the immunosuppressant that we employed the most was cyclophosphamide, in 100 patients (66.6 %), followed by azathioprine,in 81 patients (54%), and a combination of both, in 56.7%.
Conclusions: This study suggests that in our patients, the expression pattern of the observed disease is similar to that found in patients with juvenile systemic lupus erythematosus from other series.
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