ABSTRACT
Algumas doenças reumáticas, como dermatomiosite juvenil (DMJ), lúpus eritematoso sistêmico juvenil (LESJ) e esclerose sistêmica (ES), podem apresentar depósitos de cálcio nos tecidos subcutâneo e muscular, lesões conhecidas como calcinoses. Extensas coleções líquidas de cálcio referidas como milk of calcium são formas raras de calcinoses presentes na DMJ. Descrevemos um paciente de 15 anos de idade, com diagnóstico de síndrome de sobreposição ou overlap (esclerodermatomiosite e LESJ), cuja ressonância magnética (RM) evidenciou coleções líquidas perimusculares em membros inferiores e que, durante procedimento cirúrgico, foi observada a presença de coleção líquida esbranquiçada sugestiva de milk of calcium.
Rheumatic diseases such as juvenile dermatomyositis (JDM), juvenile sistemic lupus erythematosus (JSLE) and sistemic sclerosis may have calcium deposits in the subcutaneous and muscle tissues known as calcinosis. Extensive calcium-laden fluid collections referred as "milk of calcium" are rare forms of calcinosis in JDM. We describe a 15-year old patient with overlap syndrome (sclerodermatomyositis and JSLE), whose magnetic resonance imaging (MRI) showed perimusculares fluid collections in the lower limbs. During surgery, we observed the presence of whitish fluid collection suggestive of "milk of calcium".
Subject(s)
Adolescent , Humans , Male , Calcinosis/complications , Dermatomyositis/complications , Lupus Erythematosus, Systemic/complications , Muscular Diseases/complicationsABSTRACT
A calcinose é uma complicação comum e incapacitante da dermatomiosite. Ela constitui uma característica marcante da doença, ocorrendo principalmente em pacientes pediátricos. Pouco se sabe a respeito de sua fisiopatologia e não existe um tratamento reconhecido universalmente. Este trabalho relata dois casos de dermatomiosite juvenil e calcinose grave com melhora clínica com o uso do alendronato e do diltiazen em um paciente e da imunoglobulina endovenosa em outro.
Calcinosis is a common and debilitating complication of dermatomyositis. It is a hallmark of the disease, occurring mainly in pediatric patients. Little is known about its pathophysiology, and there is no universally recognized treatment. This is a report of two children with juvenile dermatomyositis and severe calcinosis who showed improvement with the use of alendronate and diltiazen in one patient, and intravenous immunoglobulin in the other.
ABSTRACT
CONTEXT: Patients with refractory juvenile idiopathic arthritis can benefit from aggressive therapy. CASE REPORT: We followed the clinical course of 4 patients (2 male, 2 female) aged 9.1-17.8 years (mean of 14.5 years) with polyarticular onset of juvenile rheumatoid arthritis and one 16-year-old boy with juvenile spondyloarthropathy associated with inflammatory bowel disease. All the juvenile rheumatoid arthritis patients fulfilled the diagnostic criteria established by the American College of Rheumatology. All patients had unremitting arthritis despite maximum therapy. All patients began receiving treatment using intravenous cyclophosphamide at 500-750 mg/m² and intravenous methylprednisolone at 30 mg/kg, for 3 days monthly (1 g maximum). The patients received between 3 and 11 monthly treatments, and/or 3-5 treatments every two months for 12 months, according to the severity of the disease and/or response to the therapy. All but one patient were evaluated retrospectively at the start (time 0) and 6 months (time 1), and 12 months (time 2) after the beginning of the treatment. A rapid and clinically significant suppression of systemic and articular manifestations was seen in all patients. Our results showed the favorable effect of this treatment on the clinical and some laboratory manifestations of juvenile idiopathic arthritis