Purpose of review: To provide the most recent evidence on anti-Jo-1 syndrome.
Recent findings: Several new evidences on anti-Jo-1 syndrome have recently emerged. It has been clearly established that, at disease onset, the classic clinical triad (arthritis, myositis and interstitial lung disease - ILD) is only rarely observed. Indeed, disease onset with an isolated arthritis is common. Patients presenting with an isolated manifestation are at high risk for the subsequent occurrence of initially lacking triad findings. Moreover, the ex-novo occurrence of accompanying features such as Raynaud's phenomenon, mechanic's hands and fever during follow-up is a strong risk factor for the occurrence of overt antisynthetase syndrome (ASSD) with further triad manifestations. Several contributions on ILD involvement and prognosis have been published, as well as the distinctive muscle MRI characteristics compared with healthy controls, and a novel definition of a rare skin manifestation (hiker's feet).
Summary: Recent evidence has shed a light on the need for a better understanding of the clinical course, imaging modalities and prognosis of anti-Jo-1 syndrome, providing some relevant elements to allow early diagnosis of this often unrecognized disease.