Pädiatrie und Therapeutik
Offener Zugang
ISSN: 2161-0665
ISSN: 2161-0665
Akhila Kavirayani, Jolanta Bernatoniene, Adam Finn and Athimalaipet V Ramanan
CVID is the most common clinically encountered primary antibody deficiency disorder and can be associated with autoimmune complications. We report 2 children with CVID who developed joint symptoms several years after initial diagnosis and were referred to a pediatric rheumatologist, who suspected temporomandibular joint arthritis clinically. Both patients underwent MRI imaging with contrast administration, which confirmed temporomandibular arthritis. They were successfully treated with intra-articular steroid joint injections. Juvenile idiopathic arthritis (JIA) can be one of the autoimmune associations with CVID, but temporomandibular arthritis as the primary manifestation is uncommon, difficult to detect clinically and has not been described previously in CVID. Although jaw pain and discomfort on chewing might indicate underlying temporomandibular joint arthritis, these symptoms might not necessarily be present. If not promptly identified and treated, arthritis of JIA can cause joint damage/disability and therefore needs specialist multidisciplinary management under a pediatric rheumatologist. JIA may not respond to steroid injections alone and might require escalation in therapy with systemic immunosuppressive agents. Little is known about the intricacies of systemic immunosuppressive therapy in patients with CVID who develop JIA.
Conclusion:In relation to CVID, juvenile idiopathic arthritis (JIA) can be an associated autoimmune manifestation. JIA presenting as temporomandibular joint arthritis as the first manifestation is unusual but can occur and might be potentially difficult to detect clinically. Pediatricians should be aware of the possibility of JIA in a child with CVID and refer to a pediatric rheumatologist for appropriate management. If not treated promptly, JIA can cause joint destruction and disability.