Sequential observation of generalized joint-resorptive phenomena in systemic mutilating rheumatoid arthritis

To examine the destructive course of systemic mutilating rheumatoid arthritis (RA) associated with the opera glass hand syndrome and severe generalized resorptive arthropathy, 49 (1.4%) patients were radiographically selected out of 3400 RA patients registered in this center. Of them, a retrospective chronological study was possible in 37 patients. Mean durations from the onset of RA to the opera glass hand syndrome and severe systemic resorptive arthritis were 16.3±6.5 and 16.9±7.0 years, respectively, and the progression rate of mutilating changes, especially of systemic joints, was significantly more rapid when changes started early after the onset of RA (r=−0.654,P<0.001). In five patients who were followed up during almost the whole mutilating process, an exponential increase of the number of joints involved, small and large joints alike, was observed in parallel with severe involvement of the cervical spine and elevated levels of ESR and CRP, within 4–5 years after the onset of the first mutilating change (rapid destructive pattern). From radiographical (massive, progressive bone erosion or cysts) and histopathological (extensive invasion of the synovia, bone and bone marrow by polygonal histiocytes, multi-nuclear giant-cells or osteoclasts) findings from these five cases, we suggest synovia—bone and/or synovia—bone marrow interaction are important factors for rapid bone resorption in mutilating RA.