TITLE:
Effectiveness of Rituximab Therapy on Severe Calcinosis in 4 Children with Juvenile Dermatomyositis
AUTHORS:
Mohammad Alhemairi, Mohammed Muzaffer
KEYWORDS:
Calcinosis, Rituximab, Juvenile Dermatomyositis
JOURNAL NAME:
Open Journal of Rheumatology and Autoimmune Diseases,
Vol.7 No.1,
January
6,
2017
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 review 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. Results: 4 patients (2 male, 2 female), interval
between onset of symptoms and diagnosis was 6 - 12 months, course of JDM was
polycyclic or relapsing, duration of follow up was 5 - 7 years. Calcinosis was
severe causing ulceration, recurrent 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 conventional 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 activity and frequency of admission especially due to
complications of calcinosis. One patient had complete clearance of calcinosis
for the last 5 years. Others
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
effect of rituximab in treatment of calcinosis in 4 patients with JDM-associated
severe calcinosis when it was used with other conventional DMARDs.