Vanishing fingers: systemic sclerosis-associated acro-osteolysis

A 37-year-old woman presented with digital pain and discoloration of 2 years duration.Physical examination revealed Raynaud's phenomenon, sclerodactyly and digital gangrene (Figure 1A, arrows).The test results for antinuclear antibody and anti-ribonucleoprotein antibody were positive.But, antineutrophil cytoplasmic antibodies, anti-double-stranded DNA, anti-Topo-I, anti-centromere and antiphospholipid antibodies were all negative.Monoclonal protein and cryoglobulin were not detected by immunoelectrophoresis.Notably, a hand X-ray demonstrated osteolysis of the distal phalanx (Figure 1B, arrows).Thus, she was diagnosed with acro-osteolysis due to systemic sclerosis (SSc).Vasodilators, antiplatelet drug and immunosuppressant were initiated, but her clinical condition did not recover.Then, digital necrosis was surgically resected.Progressive acro-osteolysis is strongly associated with severe digital ischemia and the incidence was reported to be about 9.8 per 100person-years in SSc patients.On the other hand, after 3 years of follow-up, the rate of progression increased remarkably, resulting in half of the patients developing severe osteolysis [1, 2].This case reminds readers to consider SSc as a cause of acro-osteolysis.Figure 1.Physical and X-ray findings of systemic sclerosis associated acro-osteolysis.