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Abstract
The clinical manifestations of antisynthetase syndrome are severe interstitial pneumonitis, mild polyarthritis, and myositis. This disease is accompanied by anti-Jo-1 antibodies and anti-Ro/SSA antibodies and occasionally by the concurrence of anti-Jo-1 and anti-Ro/SSA antibodies, which leads to a more severe form of interstitial lung disease. In this case, the patient was transferred to our hospital because of pulmonary fibrosis with myositis and diagnosed with antisynthetase syndrome and the concurrence of anti-Jo-1 with anti-Ro/SSA antibodies. He was refractory to glucocorticoids, and developed leucopenia and thrombocytopenia. He was treated with rituximab infusions, but the interstitial pneumonitis progressed very rapidly and he died.
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Fig. 1.
Pulmonary function test showed restrictive pattern and reduced diffusion capacity (forced vital capacity [FVC], 46%: forced expiratory volume in 1 sec [FEV1], 53%: CO diffusing capacity [DLCO], 44%).
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