Wide spectrum of disease severity and extrarenal manifestations (intracranial aneurysms, thoracic aortic dissection, retinal artery and vein occlusions, pericardial effusion, pancreatic cysts and intrapapillary mucinous neoplasm, seminal vesicle cysts, arachnoid cyst, dural diverticulum, colonic and duodenal diverticula).