title: Juvenile diffuse systemic sclerosis/systemic lupus erythematosus overlap syndrome--a case report.
abstract: We report a rare case of diffuse systemic sclerosis (SSc) evolving into diffuse SSc/systemic lupus erythematosus (SLE) overlap syndrome. A 15-year-old boy was diagnosed as diffuse SSc with initial presentations of Raynaud's phenomenon and skin tightening. He underwent Chinese herbal treatment and clinical symptoms deteriorated in the following 3 years. On admission to our ward, serositis with pleural effusion, severe pulmonary fibrosis with moderate pulmonary hypertension, swallowing difficulty, and polyarthritis were observed. Autoantibody profiles revealed concurrence of anti-double-stranded DNA, anti-Smith, anti-topoisomerase I, and anti-ribonucleoprotein antibodies. The patient fulfills the criteria for both diffuse SSc and SLE. After drainage for pleural effusion accompanied by oral prednisolone and sildenafil, there were improvement of respiratory distress, swallowing difficulty, and pulmonary hypertension. In conclusion, connective tissue diseases may overlap with each other during the disease course. Serial follow-up for clinical symptoms as well as serological changes is recommended.
authors.affiliation: Division of Hematology and Oncology, Department of Pediatrics, Taichung Veterans General Hospital, No. 160, Sec. 3, Chung-Kang Rd., Taichung, 40705, Taiwan. linhengkuei@gmail.com
authors.forename: Heng-Kuei
authors.initials: HK
authors.lastname: Lin
authors.forename: Jiaan-Der
authors.initials: JD
authors.lastname: Wang
authors.forename: Lin-Shien
authors.initials: LS
authors.lastname: Fu
mesh_terms: D000293:Adolescent; D000959:Antihypertensive Agents / Q000627:therapeutic use; D001168:Arthritis / Q000209:etiology; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood; D003680:Deglutition Disorders / Q000209:etiology; D018450:Disease Progression; D004322:Drainage; D004365:Drugs, Chinese Herbal / Q000627:therapeutic use; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D008297:Male; D010879:Piperazines / Q000627:therapeutic use; D010996:Pleural Effusion / Q000209:etiology; D011239:Prednisolone / Q000627:therapeutic use; D011658:Pulmonary Fibrosis / Q000209:etiology; D011687:Purines / Q000627:therapeutic use; D011928:Raynaud Disease / Q000209:etiology; D045743:Scleroderma, Diffuse / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D012700:Serositis / Q000209:etiology; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D001323:Autoantibodies; D015415:Biomarkers; D004365:Drugs, Chinese Herbal; D005938:Glucocorticoids; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D011239:Prednisolone; D000068677:Sildenafil Citrate