Abstract
Juvenile dermatomyositis (JDM) is a multisystem vasculopathy that infrequently presents with acute complications (1). We report here the case of a 12-year-old girl with JDM who developed Thrombotic Thrombocytopenic purpura (TTP) and Purtscher's retinopathy. This is the second pediatric case of JDM with TTP and Purtscher's retinopathy in the literature. The diagnosis of JDM was based on her clinical presentation (fever, myalgia, proximal muscle weakness, characteristic skin rash and elevated muscle enzymes) (2). Despite improvement of rash, fever and weakness with corticosteroids and intravenous Immunoglobulins (IVIG), the patient developed retinopathy, thrombocytopenia, hemolytic anemia, renal failure, and pulmonary edema within 1 week of initial treatment. A clinical diagnosis of TTP and Purtscher's retinopathy was made and her ADAMTS13 activity was found to be low. Regardless of aggressive treatment with pulse steroid therapy, IVIG, plasmapheresis along with multiple infusions of Fresh Frozen plasma (FFP), her condition deteriorated. In view of her worsening condition, she received one dose of Rituximab and within 48 h, her hematological and retinal involvements improved. Rituximab was given at the same dose once weekly thereafter for 4 total doses. Her disease process was halted, and retinopathy improved significantly in 48 h and continued to gradually improve over 3 weeks of maintenance therapy with cyclosporine, methotrexate, and IVIG and then stabilized. This report documents the association of TTP and Purtscher's retinopathy with JDM, emphasizing that early recognition and prompt treatment with rituximab along with the current standard of care treatment i.e., Vincristine, corticosteroids and plasmapheresis could be of potential benefit in controlling disease activity.
Highlights
Juvenile dermatomyositis (JDM) is a rare autoimmune multi-system vasculopathy occurring in about 2–4 per Million children per year in the United States with peak onset between 5 and 14 years of age [1]
Several autoantibodies are associated with JDM including both myositis-specific and myositis associated [2]
Von Willebrand factor, an endothelial bound clotting factor, is found to be elevated during JDM activity due to the inflammation and ongoing autoimmune vascular injury [3]. It is unknown whether von Willebrand factor (vWF) has a role in triggering Thrombocytopenic purpura (TTP)
Summary
JDM is a rare autoimmune multi-system vasculopathy occurring in about 2–4 per Million children per year in the United States with peak onset between 5 and 14 years of age [1]. The patient was admitted to Pediatric Intensive Care Unit (PICU) Hemolytic anemia and renal failure continued to worsen (Table 1) Another IV pulse methylprednisolone (30 mg/kg/d) dose was given and 2 volumes plasmapheresis were exchanged. Days 4: Renal injury, hemolytic anemia, and thrombocytopenia persisted She received 2 cycles of plasmapheresis with FFP replacement, 2 cycles of hemodialysis, and two more methylprednisolone pulses. B cell ablating treatment with rituximab 375 mg/m2/dose was initiated She continued to have hemodialysis (3 times a week), plasmapheresis (daily) with FFP, IVIG and IV methylprednisolone pulses. She is being followed regularly by ophthalmology in her annual appointments
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
