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Alessandra Meneghel, Giorgia Martini, Carolina Birolo, Alvise Tosoni, Andrea Pettenazzo, Francesco Zulian, Life-threatening systemic capillary leak syndrome in juvenile dermatomyositis, Rheumatology, Volume 56, Issue 10, October 2017, Pages 1822–1823, https://doi.org/10.1093/rheumatology/kex262
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Sir, JDM is a rare disease characterized by chronic inflammation of muscle and skin. Herein, we describe three cases of JDM complicated by systemic capillary leak syndrome (SCLS), a rare, life-threatening disorder characterized by severe hypotension, hypoalbuminaemia and haemoconcentration [1].
Case 1: a 7-year-old boy presented with a 3-week history of skin rash associated with progressive fatigue. Physical examination revealed symmetrical proximal muscle weakness, heliotrope rash and Gottron’s papules. On admission, laboratory tests showed elevation of all the muscle enzymes (Table 1). MRI showed spotty oedema of pelvic, gluteal and posterior thigh muscle groups consistent with inflammatory oedema. A diagnosis of JDM was made, and treatment with i.v. methylprednisolone pulses (30 mg/kg/day for 3 days) followed by prednisone (2 mg/kg/day) was promptly started. A week after admission, the patient presented with acute respiratory distress, dysphagia, sudden s.c. oedema of the neck and upper limbs, myalgia, oliguria and hypotension. Laboratory investigations showed worsening of all muscle enzymes, neutrophilic leucocytosis and a significant reduction of sodium and albumin (Table 1). The patient’s general condition rapidly deteriorated, and he needed admission into the paediatric intensive care unit (PICU), where albumin infusion, diuretics and total parenteral nutrition were administered. As SCLS was suspected, IVIG therapy (400 mg/kg/day for 5 days) was started. The patient was discharged 1 month after admission on oral prednisone (1 mg/kg/day), MTX (15 mg/m2/week) and monthly IVIG infusions (400 mg/kg/dose).
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