Abstract
Abstract Antisynthetase syndrome is a rare inflammatory muscle disease related to dermatomyositis and polymyositis. Anti-Jo-1 antibodies are the most detected antibodies in antisynthetase syndrome. The main clinical features of antisynthetase syndrome are myositis, polyarthritis, interstitial lung disease, mechanic’s hands and Raynaud phenomenon. A 50-year-old man with a background of inflammatory muscle disease and interstitial lung disease, for which he is under the care of respiratory and rheumatology teams, was referred to the dermatology department by his general practitioner with refractory xerotic dry fissured skin on his fingers and feet. He reported itchy, sore and painful dry, cracked skin on both his hands, mainly on the fingertips. It did not affect the rest of his body. He trialled potent topical steroids and emollients, but his condition did not improve. He was on a reducing course of prednisolone 15 mg daily and on long-term oxygen therapy at home for interstitial lung disease. On clinical examination, he had an eczematous-looking eruption. The appearance of his hands was in keeping with ‘mechanic’s hands’. Together with the underlying diagnosis of interstitial lung disease, a diagnosis of antisynthetase syndrome was suspected. Blood tests revealed a fibrinogen of 5.39 g L−1 [reference interval (RI) 1.50–4.50], lupus creatine kinase of 1591 IU L−1 (RI 40–320), positive antibodies to Ro (SS-A) and positive antibodies to Jo-1. Jo-1 antibodies are found in dermatomyositis or polymyositis. Ro antibodies are associated with Sjogren syndrome (primary or secondary) or systemic lupus erythematosus, and serum may be antinuclear antibody negative. Therefore, we suspected a diagnosis of antisynthetase syndrome. A patch test showed a positive reaction to nickel, which was deemed to be in keeping with antisynthetase syndrome hand eruption, or ‘mechanic’s hands’. The patient was commenced on mycophenolate, a low dose of acitretin 10 mg and emollients and reported a significant improvement in symptoms. Repeat blood tests were scheduled for 3 months, including lipid profile and liver function test, for monitoring purposes. A follow-up was arranged for 12 weeks. He had a background of recurrent hospital attendance with pericardial effusions and chest symptoms and was tested for COVID-19 multiple times. This case demonstrates the importance of multidisciplinary coordination with rheumatology and respiratory colleagues in antisynthetase syndrome.
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