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Introduction Overlap syndromes with juvenile systemic sclerosis are infrequent and diagnostic challenge. The objective is to describe the clinical and pathological correlation of two patients with juvenile systemic scleroderma and overlap syndrome.Material and Methods Report of CasesResults Patient 1. A 16-year-old adolescent who presented Raynaud’s phenomenon, polyarthralgias, myalgias, and gradual loss of muscle strength in the upper and lower limbs. Examination showed diffuse skin induration, malar telangiectasias, Rodnan score 21/51, calcinosis in the right thigh. Proximal muscle strength in limbs 3/5. Laboratory tests: ANA 1/320 homogeneous, antiSCL70 (+), anti-Ro 52 (+), anti PM SCL 100 and 70 negative; total CPK: 10,330.2 U/L. Electromyography: myositis. Echocardiogram and TEM of the chest: normal. Capillaroscopy late pattern. Muscle biopsy: multifocal lymphocytic inflammatory infiltrates within the endomysium and periendomysial vessels., consisting of CD8, CD3, and CD 20 positive T lymphocytes. Histopathological findings of Juvenile Polymyositis. Her diagnostic was Juvenile Diffuse Cutaneous Systemic Sclerosis with Polymyositis. She started prednisone 60 mg/d and methotrexate 25mg subcutaneously weekly. The patient improved without cardiopulmonary or renal complications.Patient 2. A 16-year-old adolescent who began with Raynaud’s phenomenon at the age of 12. Two years later, she was diagnosed of Systemic Lupus Erythematosus: malar erythema, photosensitivity, polyarthritis, antibodies ANA, anti-Sm and anti-RO 52 were positive. Antimalarials and prednisone were started. One year later, her physical examination showed diffuse skin induration, malar erythema, edema of the lower legs. Laboratory: antibodies ANA 1/160 homogeneous, antibodies anti-RNP/Sm and anti-DNA (ds) were positive, antibodies anti-SCL 70, anti-Centromere were negative. Proteinuria 9.02 g/24 h. Chest TEM: interstitial lung disease. Respiratory function tests: mild restriction. Renal biopsy revealed glomerular ischemic changes, diffuse podocyte pedicellar effacement, arterioles with medial hyperplasia and fibrosis. Immunofluorescent showed immunoglobulins and complement deposition in endothelium and mesangium. Histopathological findings included scleroderma-associated nephropathy and podocytopathy lupic. She started prednisone 50 mg/d, mycophenolate mofetil 2 g/d and rituximab 1 g intravenously, with clinical improvement in the kidneys, lungs, and skin.Conclusions The clinical and histopathological correlation of Juvenile Systemic Scleroderma with other autoimmune diseases is important to define the etiology of the clinical manifestations, the prognosis, the use of high doses of corticosteroids and prevent complications.