Lasma for 1 week and intravenous cyclophosphamide treatment (750 mg/m2/month) were started.Results The treatment resulted in slow improvement in his muscle weakness within 4 weeks along with markedly decreased muscle enzymes and normalization of renal function. Prednisolone was changed to 1 mg/day of oral prednisolone, which was later decreased to 0.5 mg/day followed by transition to long-term oral methotrexate 10 mg / m2/week (after 6 monthly pulses of cyclophosphamide). Conclusions The described clinical case Methyl 3-amino-2,4-dichloro-5-fluorobenzoate demonstrates the varied nature of renal pathology at juvenile dermatomyositis. The kidney damage appeared to have been very severe. But under the well-timed and adequate therapy it underwent to a back development in a great degree.Author details 1 Department of Pediatric Rheumatology, Orenburg Regional Children's Hospital, Orenburg, Russia. 2Department of Pediatric Nephrology, Orenburg Regional Children's Hospital, Orenburg, Russia. Published: 14 Septemberdoi:10.1186/1546-0096-9-S1-P49 Cite this article as: Frolenko et al.: Thrombotic microangiopathy in juvenile dermatomyositis. Pediatric Rheumatology 2011 9(Suppl 1):P49.
Vogler et al. Pediatric Rheumatology 2012, 10(Suppl 1):A73 http://www.ped-rheum.com/content/10/S1/APOSTER PRESENTATIONOpen AccessPulmonary thromboembolism in children with rheumatic diseasesLarry B Vogler4*, Sheila Angeles-Han3, Sampath Prahalad2, Egla C Rabinovich1 From 2011 Pediatric Rheumatology Symposium sponsored by the American College of Rheumatology Miami, FL, USA. 2-5 JunePurpose To demonstrate the clinical features and predisposing factors of pulmonary thrombotic events in children with rheumatic diseases. Methods Boc-D-Lys-OH Chart review, observational. Results Thrombotic events have been associated with antiphospholipid antibodies in autoimmune diseases, including systemic lupus erythematosus (SLE). However, pulmonary thromboembolism (PTE) from deep vein thromboses (DVT) or in situ pulmonary arterial thrombosis is uncommon in rheumatic diseases, especially in children. TheTablePt/Gender Dx Age at Dx (yr) Age at PTE Symptoms DVT Lupus AC Anticardio AB D-dimer (ng/ml) Albumin (g/dl) AT III ( ) Fibrinogen (ng/dl) 1/F SLE thrombocytopenia 12.6 15.2 leg pain + + 647 (nl <220) 4.7 (nl 3.7-5.5) 105 (nl 77-132) 718 (nl 180-394) 2/F SLE nephritis (IV) 14.1 14.8 chest pain dyspnea 8770 2.0 278 234 3/M SLE nephritis (V) 9.0 16.8 chest pain dyspnea 1600 0.7 7/2 n/a 4/F SLE nephritis (IV) 12.0 12.6 chest pain dyspnea >10,000 1.7 154 298 5/M SSc PAH 12.8 16 chest pain dyspnea n/a 4.1 n/a n/a + +/+ >10,000 3.6 114 421 6/M PAN CVA 0.3 6/4 leg paindiagnosis and treatment of PTE may be delayed due to PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/13485127 a paucity of symptoms or to symptoms attributed to more common manifestations such as pleuritis or pneumonia. We report findings in 6 children with PTE secondary to SLE (4), Systemic Sclerosis (SSc) (1) and Polyarteritis Nodosa (PAN) (1).Conclusion Although antiphospholipid antibodies are common in SLE, pulmonary arterial thrombosis is rare. These 4 cases of SLE represent only 1.7 of 234 pediatric lupus patients seen at Emory University over 18 years. Pulmonary thromboemboli may mimic pleuritis with effusion or pneumonia. Besides antiphospholipid antibodies, which PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/14445666 were present in only 2 of these patients, other[PAH pulmonary arterial hypertension, CVA: cerebral vascular accident, AT III: anti-thrombin III, N/A: not available] All patients were treated with heparin and improved. No patient had any other genetic risk factors predisposi.