Abstract

A 57 year old man with nail-patella syndrome (NPS) and associated renal disease is described who developed an inflammatory polyarthropathy and polyarteritis-like vasculitis. Vasculitis and serum complement abnormalities have not previously been reported in NPS. NPS is a rare autosomal dominant connective tissue disorder affecting both mesenchymal and ectodermal tissue. The condition is reviewed with particular reference to its renal pathology, including the distinctive electron microscopic (EM) finding of collagen deposition in the glomerular basement membrane (GBM). The possibility of the underlying collagen abnormality acting as a trigger for immune-inflammatory changes is discussed.