Recovery of Barium Swallow Radiographic Abnormalities in a Patient With Dermatomyositis and Severe Dysphagia After High-Dose Intravenous Immunoglobulins Florenzo Iannone, MD, PhD, Margherita Giannini, MD, and Giovanni Lapadula, MD A 53-year-old white woman complained of asthenia, muscle weakness, and dysphagia, related to initiation of swallowing without symptoms of esophageal reflux. She had Raynaud phe- nomenon heliotrope rash and Gottron papules with reduction of muscle strength assessed by manual muscle test. Serum crea- tine kinase levels were increased (1236 U/mL), PM-Scl antibody was present, and electromyography showed typical myositis changes. Muscle biopsy showed hypotrophic irregular myofi- bers and endomysial foci of inflammatory cells. Dermatomyosi- tis was diagnosed according to Bohan and Peter’s criteria. 1 The patient gave written informed consent. Therapy with prednisone 1 mg/kg per day rapidly ameliorated the manual muscle test, and creatine kinase levels dropped to normal (57 U/mL). Nevertheless, dysphagia worsened, and initiating swallowing either solids or liq- uids was difficult. There were no reflux symptoms. The Eating Assessment Tool (EAT-10) score, a self-administered measure of dysphagia, was very high (score, 26; reference value, ≤3). 2 Bar- ium swallow radiograph of hypopharynx and esophagus showed barium retention in the valleculas and piriform sinus due to poor hypopharynx emptying and esophageal dyskinesia (A). The patient undertook therapy with high-dose intravenous immu- noglobulins (IVIg 2 mg/kg in 1 day) every 30 days for 3 months, whereas prednisone was withdrawn within 2 months. A quick im- provement of dysphagia occurred, and after 3 months, EAT-10 test was normal (score, 0), and the barium swallow radiograph showed normal hypopharynx emptying and esophageal motility with no barium retention (B). Esophageal dyskinesia raised the question whether an overlap with scleroderma was present. The latter was not contemplated as typical scleroderma manifestations, such as skin thickness, organ involvement, and capillaroscopic changes, were lacking. Notwithstanding, development of subse- quent scleroderma cannot be ruled out. High-dose IVIg has been proven effective in some cases of glucocorticoid-resistant upper dysphagia in dermatomyositis, 3 and the assessment of clinical outcomes is based on symptoms or esophageal manometry. 4 In this case, barium swallow radiograph pictures provided an impres- sive direct perception of the recovery of hypopharynx function following IVIg treatment. REFERENCES 1. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975;292:344–347. 2. Belafsky PC, Mouadeb DA, Rees CJ, et al. Validity and reliability of the Eating Assessment Tool (EAT-10). Ann Otol Rhinol Laryngol. 2008;117:919–924. 3. Aggarwal R, Oddis CV. Therapeutic advances in myositis. Curr Opin Rheumatol. 2012;24:635–641. 4. Marie I, Menard JF, Hatron PY, et al. Intravenous immunoglobulins for steroid-refractory esophageal involvement related to polymyositis and dermatomyositis: a series of 73 patients. Arthritis Care Res (Hoboken). 2010;62:1748–1755. From the Interdisciplinary Department of Medicine–Rheumatology Unit, Medical School, University of Bari, Bari, Italy. This work has not received financial support. F.I. has received consulting fees, speaking fees or honoraria (<€10,000) from Pfizer, Merck, Abbott, and Bristol-Myers Squibb. The other authors declare no conflict of interest. Correspondence: Florenzo Iannone, MD, PhD, Associate Professor of Rheumatology, Interdisciplinary Department of Medicine–Rheumatology Unit, Policlinico, Piazza G. Cesare 11, 70124 Bari, Italy. E-mail: florenzo. iannone@uniba.it. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 1076-1608 DOI: 10.1097/RHU.0000000000000252 FIGURE. Barium swallow radiographs. A, At baseline, barium retention in the valleculas and piriform sinus (left panel) and esophageal dyskinesia (right panel) were detected. B, After IVIg therapy, hypopharynx emptying (left panel) and esophageal motility (right panel) were normal with no barium retention. IMAGES JCR: Journal of Clinical Rheumatology • Volume 21, Number 4, June 2015 www.jclinrheum.com 227 Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.