Familial concurrence of pauciarticular syndrome and systemic sclerosis

We observed a sibship of two children who had the syndrome of early-onset pauciarticular arthritis with anti-nuclear antibodies; their paternal grandmother died of progressive systemic sclerosis. Since the early-onset pauciarticular syndrome is very rare or non-existent in adults, the findings in this kindred pair raise the possibility that this disorder and adult systemic sclerosis may share some genetic or other etiological factors.