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Mia Rodziewicz, Patrick Kiely, The successful use of subcutaneous abatacept in refractory anti- human transcriptional intermediary factor 1-gamma dermatomyositis skin and oesphagopharyngeal disease, Rheumatology, Volume 57, Issue 10, October 2018, Pages 1866–1867, https://doi.org/10.1093/rheumatology/key146
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Sir, Successful management of dermatomyositis (DM) requires suppression of immune-driven inflammatory disease in the skin as well as striated, oesophageal and cardiac muscle. Control of all manifestations of this disease can be difficult, and in a recent series only 38% of DM patients achieved clinical skin remission over 3 years despite active treatment [1]. We report the efficacy of subcutaneous abatacept in a patient with resistant skin and oesophagopharyngeal disease.
A 70-year-old Peruvian woman presented in 2013 with an erythematous rash, joint pains, dysphagia and proximal muscle weakness. She had extensive cutaneous DM features—a periorbital heliotrope rash, widespread erythematous scaling of the scalp, arms and upper back in a shawl distribution, and V-sign on the anterior chest. On the hands there were Gottron’s papules, periungual erythema and ragged nailfolds. Video fluoroscopy confirmed oesophagopharyngeal dysmotility. Peak serum creatinine kinase (CK) was 2070 U/L (normal range 5–200), troponin I <17 ng/l, 25-OH vitamin D 53 nmol/l and thyroid function normal. ANA was positive 1/160–640 and ENA screen negative. Anti-human transcriptional intermediary factor 1 (TIF1)-γ antibody was weakly positive and anti-Jo-1, -PL7, -PL12, -OJ, -EJ, -MDA-5, -SAE, -Mi-2, -NXP-2, -SRP, -Ku and -PmScl were negative. EMG revealed proximal myopathic motor units and MRI of thighs showed oedema of both rectus femori and the left gracilis muscles. Biopsy showed interstitial and perivascular foci of lymphocytes and macrophages, and no necrosis. This phenotype of DM with severe cutaneous and oesophageal disease, no lung involvement and TIF1-γ antibodies prompted a thorough malignancy screen with normal CT chest, abdomen and pelvis, CEA, CA125, CA 19-9, αFP and fluorodeoxyglucose-PET scan.
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