OBJECTIVE:To estimate the degree of familial aggregation of juvenile idiopathic arthritis (JIA), determine whether the aggregation of JIA and the aggregation of type 1 diabetes mellitus (type 1 DM) overlap, and identify multiplex JIA pedigrees.METHODS:Records of individuals with JIA or type 1 DM were probabilistically linked with records in the Utah Population Database (UPDB), a large computerized family history database. For each case of JIA or type 1 DM, 10 matched controls or 5 matched controls, respectively, were selected. All familial relationships among cases of JIA or type 1 DM were established. A familial risk score was calculated for each subject. For various levels of familial exposure to JIA or type 1 DM, one's risk (odds ratio [OR]) of developing JIA or type 1 DM was established (cases compared with controls). Recurrence risks for JIA were computed for relatives of JIA cases compared with relatives of controls. Extended JIA families were identified from a list of common ancestors.RESULTS:Records of a total of 443 patients were linked with the UPDB. Of these, 381 (86.0%) met criteria for JIA. An increased risk for JIA was observed among relatives of probands with JIA. The prevalence of type 1 DM among JIA cases was higher than the US prevalence of type 1 DM (P < 0.003). The recurrence risk for JIA was significantly elevated among first-degree relatives of cases with JIA (OR 30.4). The overall prevalence of JIA was 28/100,000. Four extended JIA pedigrees were identified.CONCLUSION:There is familial aggregation of JIA in the Intermountain West region of the US. We have demonstrated that multiplex JIA pedigrees can be identified using a genealogic database.

Compressive optic neuropathy with acute or chronic vision loss has been associated with various skull base tumors, aneurysms, Graves disease, trauma, and, less commonly, fibrous dysplasia and osteopetrosis. The authors present a case of acute visual deterioration in a 25-year-old woman who had massive calvarial hypertrophy with optic canal stenosis secondary to renal osteodystrophy (uremic leontiasis ossea [ULO]; bighead disease). Significant Visual field restoration was achieved with high-dose corticosteroids followed by optic nerve decompression. This is the first case report of cranial neuropathy associated with ULO.

An orbital abscess is an ophthalmic surgical emergency that is typically caused by the spread of bacteria from adjacent structures, such as the sinuses, eyelids, or teeth. Although acute dacryocystitis is commonly associated with preseptal cellulitis, it rarely causes orbital infection. Infection of the lacrimal sac will typically localize in the preseptal space because the lacrimal sac lies anterior to the orbital septum. To the authors' knowledge, this is the first report of an intraconal abscess secondary to acute dacryocystitis. The key points in the surgical management of this entity are discussed.