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Question |
12 yr old female presented with generalised joint contractures including inability to open mouth fully and generalised muscle wasting which has developed over the last 3 years,prior to which she was absolutely normal.Facial appearance has a puckered look with tight skin.Her fingers and toes show features of arachnodactyl.No h/o Raynauds phenomenon.No h/o dysphagia.No abnormal perinatal events.No h/o delayed mile stones.Bowel,micturition-normal.Investigations revealed atlanto axial subluxation.CXR NORMAL.USG abdomen normal.ESR 65MM.RA FACTOR -VE.ANA+VE.No Facilities at our centre to investigate futher.What could be the diagnosis and how to start treatment.
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Answer |
ONe should rule out scleroderma and juvenile dermatomyositis (JDMS) and also SLE. Atleast a dsDNA, CPK and MRI of the muscles would be useful. IF dsDNA positive, it is suggestive of SLE. If negative, think of JDMS especially if CPK high. Treatment would consist of steroids in SLE. For JDMS, along with steroids, one may require methotrexate.
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