Retinopatía Purtscher-like asociada a dermatomiositis y linfohistiocitosis hemofagocítica / Purtscher-like retinopathy associated with dermatomyositis and hemophagocytic lymphohistiocytosis

CASO CLÍNICO: Niña de 11 años de edad con debilidad muscular progresiva debida a dermatomiositis y linfohistiocitosis hemofagocítica, en cuyo examen funduscópico se detectan múltiples exudados algodonosos, tortuosidad venosa y hemorragias en llama, lo que lleva al diagnóstico de retinopatía Purtscher-like. DISCUSIÓN: La retinopatía de Purtscher es una alteración microvascular de diagnóstico clínico y etiología probablemente embólica. Su tratamiento es controvertido, siendo los corticoides sistémicos como la elección más extendida

CASE REPORT: An 11 year-old girl with progressive muscle weakness due to dermatomyositis and hemophagocytic lymphohistiocytosis in which multiple cotton exudates, venous tortuosity and flame haemorrhages are detected in the funduscopic examination, leading to the diagnosis of Purtscher-like retinopathy. DISCUSSION: Purstcher's retinopathy is a microvascular disorder with clinical signs of probable thrombotic origin. Its treatment is controversial, with systemic corticosteroids being the most widespread choice

Purtscher-like retinopathy associated with dermatomyositis and hemophagocytic lymphohistiocytosis. / Retinopatía Purtscher-like asociada a dermatomiositis y linfohistiocitosis hemofagocítica.

CASE REPORT: An 11 year-old girl with progressive muscle weakness due to dermatomyositis and hemophagocytic lymphohistiocytosis in which multiple cotton exudates, venous tortuosity and flame haemorrhages are detected in the funduscopic examination, leading to the diagnosis of Purtscher-like retinopathy. DISCUSSION: Purstcher's retinopathy is a microvascular disorder with clinical signs of probable thrombotic origin. Its treatment is controversial, with systemic corticosteroids being the most widespread choice.

Cómo diagnosticamos y tratamos una enfermedad reumática: casos clínicos interactivos / How do we diagnose and treat a pediatric rheumatic disease: interactive cases report

En este seminario se trata el abordaje diagnóstico y terapéutico inicial de las enfermedades reumáticas pediátricas a través de la resolución interactiva de unos casos clínicos representativos de la artritis idiopática juvenil en sus distintas formas de expresión clínica, por ser esta la enfermedad más frecuente en esta especialidad. Se repasan los últimos avances en el diagnóstico y tratamiento de interés para el pediatra de Atención Primaria (AU)

This seminar approaches the initial diagnosis and treatment of juvenile idiopathic arthritis, the most frequent rheumatic disease in children. We present interactive cases report where the different clinical forms of juvenile idiopathic arthritis are distinguished. We review the recent advances in diagnosis and treatment of interest for the primary care pediatrician (AU)

Effectiveness of the treatment with intravenous pamidronate in calcinosis in juvenile dermatomyositis.

OBJECTIVE: Calcinosis is a frequent finding in up to 40% of children with juvenile dermatomyositis (JDM). Different treatments (aluminum hydroxide, diltiazem, probenecid, alendronate, etc.) have been used in an attempt to clear calcinosis and to avoid the onset of new calcium deposition, but none has been clearly effective. Pamidronate is a nitrogen-containing bisphosphonate with a potent inhibiting bone resorption effect that has been used to treat osteoporosis in children. We report three children with JDM who developed calcinosis and who received intravenous pamidronate with good results. METHODS: All three patients met the Bohan and Peter diagnostic criteria for JDM. Intravenous pamidronate was given at 1 mg/kg/day on three consecutive days every three months according to the protocol established by Glorieux et al. for osteoporosis treatment in osteogenesis imperfecta. RESULTS: The calcinosis which developed in all three patients improved. No important adverse events were observed. CONCLUSION: In all three cases, calcinosis significantly decreased, and even totally cleared in patient 1. Total clearance of pre-existing calcinosis in JDM with pamidronate therapy has not been previously described with any of the aforementioned treatments. The advantage of treatment with pamidronate compared to treatment with alendronate is that intravenous administration does not produce esophagitis, the most frequent adverse event when orally administering bisphosphonates. Our results strongly suggest that therapy with intravenous pamidronate in conjunction with good disease control with DMARD therapy is an apparently safe and effective treatment for calcinosis management in JDM.