Decision-Making in Diagnosis and Management of Extraintestinal Manifestations of Inflammatory Bowel Disease

Abstract

In the absence of certainty regarding the causes of both inflammatory bowel disease (IBD) and its extraintestinal manifestations (EIMs), there is necessarily ambiguity in both academic and clinical arenas concerning the diagnosis, classifications, and treatments of EIMs. While the “true” EIMs are considered extensions of the IBD gut pathogenesis with an immunologically mediated inflammatory consequence, other EIMs are considered to be complications of IBD itself or its treatment. A third group of IBD EIMs includes those disorders which seem to occur more often in IBD but for which an etiologic or pathophysiologic connection to IBD is highly theoretical. Patients with IBD and EIMs tend to have more severe, long-duration disease, and a reduced quality of life. EIMs presentation may or may not parallel IBD gut inflammatory activity. The clinical decision-making processes necessary for successfully managing simultaneously the gut component of IBD and its EIMs are presented. Based upon clinical experience and review of leading publications, the consensus of best practices, differential diagnoses for EIMs, and current management programs are presented with enumeration of specific decisions and considerations required for successful management of EIMs. EIMs of inflammatory bowel disease reflect the immunopathologic common ground and hence the systemic nature of the IBD. A defined decision-making process is offered which includes consultations and attention to the differential diagnosis to avoid not uncommon mistakes in diagnosis. Management of all EIMs requires assessment of both the clinical and pathologic status of the gut component of IBD combined with judicious selection of general and /or immunosuppression therapy for the EIMs.