Open Journal of Rheumatology and Autoimmune Diseases, 2017, 7, 16-29 http://www.scirp.org/journal/ojra ISSN Online: 2164-005X ISSN Print: 2163-9914 DOI: 10.4236/ojra.2017.71002 January 6, 2017 Effectiveness of Rituximab Therapy on Severe Calcinosis in 4 Children with Juvenile Dermatomyositis Mohammad Alhemairi¹, Mohammed Muzaffer² 1 Department of General Pediatrics, Rabigh General Hospital, Rabigh, Kingdom of Saudi Arabia 2 Department of Pediatrics and Pediatric Rheumatology, King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia Abstract Background: Calcinosis is an important sequela of JDM which may cause significant morbidity and mortality. There is no standard curative treatment for calcinosis but different agents were used with variable efficacy. We report the favorable outcome of rituximab on severe calcinosis in 4 JDM patients and present their clinical data. Patients and Methods: A retrospective chart re- view of 4 children with JDM and severe calcinosis who received rituximab for relapsing or polycyclic JDM course. Diagnosis and follow up of calcinosis was clinically and by X-ray. Review data included: age of patients at onset of JDM symptoms and diagnosis, clinical and laboratory criteria at diagnosis, disease course and duration of follow up. Data about calcinosis onset, sites, severity and its progression were also included. Further data about rituximab therapy included: dosage, side effects, other treatment used before, during or after this drug and outcome and duration of follow up of calcinosis after therapy. Re- sults: 4 patients (2 male, 2 female), interval between onset of symptoms and diagnosis was 6 - 12 months, course of JDM was polycyclic or relapsing, dura- tion of follow up was 5 - 7 years. Calcinosis was severe causing ulceration, re- current skin infections and joint limitation. It was not improving despite treatment with different DMARDs and/or bisphosphonates, colchicine and warfarin. Reason to start rituximab was inadequate disease control with con- ventional DMARDs. All patients received steroids and more than one DMARD before starting rituximab and were continued thereafter, follow up after rituximab was 3 to 5 years. All patients had improvement in disease ac- tivity and frequency of admission especially due to complications of calcino- sis. One patient had complete clearance of calcinosis for the last 5 years. Oth- ers had significant improvement in calcinosis with no new lesions, decreased sites and density and decreased calcinosis related contractures. There were no serious side effects to rituximab. Conclusion: Our study showed the favorable How to cite this paper: Alhemairi, M. and Muzaffer, M. (2017) Effectiveness of Ri- tuximab Therapy on Severe Calcinosis in 4 Children with Juvenile Dermatomyositis. Open Journal of Rheumatology and Au- toimmune Diseases, 7, 16-29. https://doi.org/10.4236/ojra.2017.71002 Received: November 17, 2016 Accepted: January 3, 2017 Published: January 6, 2017 Copyright © 2017 by authors and Scientific Research Publishing Inc. This work is licensed under the Creative Commons Attribution International License (CC BY 4.0). http://creativecommons.org/licenses/by/4.0/ Open Access