CASE BASED REVIEW Rituximab-induced regression of CREST-related calcinosis Dayane Raquel de Paula & Fabiane Barbero Klem & Pedro Gabriel Lorencetti & Carolina Muller & Valderilio Feijó Azevedo Received: 21 October 2012 / Accepted: 7 November 2012 # Clinical Rheumatology 2012 Abstract About a quarter of sclerodermic patients present calcinosis. However, patients with limited form of the dis- ease are more likely to have calcinosis than patients with diffuse form. We report a case of a 54-year-old female patient with limited cutaneous scleroderma using rituximab (RTX) to treat lung fibrosis and arthritis. Into RTX treat- ment, she also had a complete resolution of calcinosis in her hands. The patient reported improvement in dyspnea and synovitis after two courses of RTX (four weekly infusions 375 mg/m 2 each). After 7 months of the first infusion, the calcinosis in her fingers had a complete remission, especial- ly the right thumb. Based on current evidences, we discuss the use of rituximab as a promising therapy to treat not only lung disease but also calcinosis in patients with scleroderma. Keywords Calcinosis . Rituximab . Scleroderma . Treatment Introduction About a quarter of sclerodermic patients present calcinosis. Patients with limited form of the disease, who has anticentro- mere antibody positive [1], are more likely to have calcinosis than patients with diffuse form. This prevalence is due to a deposition of hydroxyapatite in areas frequently exposed to microtrauma (fingers, elbow, and forearm) [2], resulting in pain (ischemia), inflammation, and ulceration of the affected area and a secondary infection site. A standard treatment for calcinosis has not been established yet. However, some drugs have been proven potential benefit: warfarin, bisphospho- nates, diltiazem, aluminum hydroxide, minocyclin, and pro- benecid [2–4] and anti-TNF alfa. Alternative treatments comprise carbon dioxide laser, shock wave lithotripsy, and extracorporeal surgery [2, 3]. Recently, rituximab (RTX) has been considered to treat lung fibrosis and also calcinosis in scleroderma patients [5–8]. We report a case of a patient with limited scleroderma using RTX to treat lung fibrosis who had a complete resolution of calcinosis in their hands. Report A 54-year-old female patient presented with signs of limited cutaneous scleroderma. The symptoms had started 15 years ago with Raynaud's phenomenon in hands and feet, facial telangiectasia, and skin thickening on neck and face. Nine years ago, a skin biopsy had revealed scleroderma, although, the anti-Scl70 was undetectable on that occasion. ANA was positive 1:160 homogeneous nucleolar pattern. Five years ago, she had had an episode of pericarditis and after the event, computed tomography (CT) of the chest and echocardiography were performed, showing any changes and the estimated pul- monary artery pressure on echocardiography was 35 mmHg. One year later, CT lung scans showed progression to lung D. R. de Paula : F. B. Klem : P. G. Lorencetti Faculty of Medicine, Federal University of Paraná, Curitiba, Brazil C. Muller Scleroderma Outpatient Clinic, Hospital de Clinicas, Federal University of Paraná, Curitiba, Brazil V. F. Azevedo Hospital de Clinicas, Federal University of Paraná, Rua Lamenha Lins 1110- ap11a Rebouças, Curitiba, Paraná, Brazil 80250-020 V. F. Azevedo (*) Edumed-Health Research and Education, Rua Lamenha Lins 1110- ap11a Rebouças, Curitiba, Paraná, Brazil 80250-020 e-mail: valderilio@hotmail.com Clin Rheumatol DOI 10.1007/s10067-012-2124-z